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A man in his 60s was hospitalised for dyspnoea with minimal exertion and at rest. His medical history includes hypertension, non-ischaemic cardiomyopathy, left bundle branch block and congestive heart failure (HF). He had received a cardiac resynchronisation therapy implantable cardioverter-defibrillator (CRT-D) 2 years prior to the current admission, with initial improvement in symptoms, after which he was lost for follow-up. He is currently on a medical regimen that includes a β-blocker, a loop diuretic and an angiotensin receptor blocker. Physical exam reveals elevated jugular venous pressure, crackles at both bases and a third heart sound. The chest X-ray (CXR) is …

A young man in his 20s presented to the emergency department with a 2-week history of dyspnoea and fever. His medical history was remarkable for recurrent episodes of fever with arthralgia in childhood f. Clinical examination revealed pedal oedema, bounding pulses and a holodiastolic murmur. Additionally, chest auscultation revealed bibasilar crackles. The ECG showed sinus tachycardia with features of left ventricular volume overload. Two-dimensional transthoracic echocardiography was performed and …

­An 83-year-old woman with a history of hypertension, diabetes and paroxysmal atrial fibrillation anticoagulated with acenocoumarol was brought to the emergency department due to dyspnoea. At admission, the patient reported a 1-week history of malaise, shortness of breath and non-productive cough. She denied fever but reported pain on the left flank. On examination, auscultation showed arrhythmic tones and crackles in the left lower lung field. Laboratory findings showed leucocytosis of 15.32×103/μL, and the C reactive protein was 177 mg/L. The activated partial thromboplastin time was 54.8 s, and the international normalised ratio was 7.09. A chest X-ray showed left lower lobe consolidation with pleural effusion. Point-of-care ultrasound was performed using a low-frequency curved transducer (2–5 MHz). The probe was placed in the left posterior axillary showing a pulmonary consolidation, but also a hypoechoic lesion in the spleen was found (figure 1).Figure 1Ultrasound image of the spleen in longitudinal section demonstrating a large, hypoechoic, wedge-shaped lesion.QuestionWhat is the most likely diagnosis?Splenic abscessSubcapsular splenic haematomaSplenic infarctionSplenic hydatid cystFor answer see page 2

Background:Interstitial lung disease (ILD) represents the most frequent pulmonary manifestation of primary Sjogren’s syndrome (pSS), with a prevalence ranging between 6-70% in different retrospectives studies. Non-specific interstitial pneumonia (NSIP) is recognized as the most common ILD disorder, followed by organizing pneumonia (OP), usual interstitial pneumonia (UIP) and lymphocytic interstitial pneumonia (LIP), specifically associated with pSS but less frequent.Objectives:To investigate the prevalence of fibrosing patterns in a monocentric cohort of pSS patients evaluated for lung involvement in a cross-sectional study.Methods:In a cross-sectional study all patients fulfilling ACR/EULAR classification criteria for pSS and with a known diagnosis of ILD were enrolled; the other patients were carefully investigated for signs or symptoms suggestive for ILD (including the search for velcro crackles with a digital device); when suspect, patients underwent to high resolution computed tomography (HRCT). An expert radiologist re-evaluated all HRCT for classifying the ILD pattern as: UIP, fibrotic NSIP, fibrotic OP, NSIP, OP, LIP, indeterminate.Results:One hundred and eighty-five pSS patients were enrolled; among them 34 showed ILD (18.4%) with the following features: M/F 3/31, median age 57 (range 24-80), median FVC 90% (39-127%), median DLCO 49% (20-84%). Patients were classified in two groups according to radiologic classification: the group 1 (18 pts 52,9%) included UIP (13 patients, 38.2%), fibrotic NSIP (4, 11.8%), fibrotic OP (1 2.9%); the group 2 (16 pts, 47.1%) included NSIP (6, 17.6%), OP (4, 11.8%), indeterminate (4, 11.8%), LIP (2, 5.9%). No significant differences were observed between the two groups with the exception of anti-SSB positivity more frequently detected in non-fibrosing pattern (p0,043).Conclusion:Despite previous observations, our data suggest a high prevalence of fibrosing ILD pattern in pSS patients. We participate at a multidisciplinary team with expert pulmonologists and radiologists and some patients of our cohort firstly referred to pulmonologist for appearance of ILD before the diagnosis of pSS, contributing to the possible selection of more severe lung disease. However, these data suggest first of all that pSS should always be considered in differential diagnosis of fibrosing ILD; moreover, since fibrosing ILD is thought to have a worse response to immunosuppressive drugs, the role of new possible therapeutic strategies such as anti-fibrotic could represent an important field of interest.Disclosure of Interests:None declared

Background:Rheumatoid arthritis (RA) affects 0.4-1.3% of general population (1). It can affect lungs in different ways, with interstitial lung disease (ILD) as the most severe. Clinically evident ILD has been reported in 10-42% of patients, with a great impact in prognosis (2).Objectives:Toidentify the prevalence of lung involvement in early rheumatoid arthritis patients (ERA) without previous known lung disease and describe the association between high resolution computed tomography (HRCT), lung functional tests (LFT) and clinical findings.Methods:Cross sectional multicentric study. We included ERA patients (1 year or less since diagnose) consecutively. Patients with previous RA related lung disease or biologic/targeted synthetic Dmard treatment were excluded. HRCT, immunological tests (rheumatoid factor, anti-CCP, ANA), LFT and clinical evaluation were performed.Results:We included 74 patients, 63 (85,1%) woman, mean (SD) of 47 (17,7) years. Thirty-seven patients (50%) were current or former smokers. Abnormal findings in HRCT were found in 62 patients (88,6%): ILD in 6 (8,6%), airway involvement in 40 (70%) and emphysema in 7 (10%). Ten patients (13,5%) had abnormal auscultation (2 sibilances, 2 roncus, and 6 crackles). Six patients (8,1%) had digital clubbing. Regarding immunological tests, 54/61 (88,5%) patients were positive for Anti CCP, and 53/61 (86,9%) were positive for FR. We compared features of patients with findings related to RA in HRCT (interstitial and/or airway) with those without them. We found no differences in the mean (SD) of DAS-28 [4,74 (1,38) vs 4,32 (1,39); p= 0,27]. The prevalence of anti- CCP was not higher in patients with abnormal HRCT [38/44 (86,3%) vs 16/17 (94,1%); p=0,39]. Patients with abnormal HRCT were older [median (IQR) 50,5 years (44,5-59,5) vs 43 years (32-51); p=0,008) and showed higher VSG values [mean (SD) 39,09 (24,03) vs 27,38 (17,6); p= 0,043]. Abnormal physical examination or dyspnea (class 2 mMRC or higher) was significantly associated with HRCT abnormalities [26 (50%) vs 3 (13,6%); p=0.003) and the presence of ILD on HRCT was significantly associated with crackles on the auscultation [4/68(6,25%) vs 2/6 (33,33%); p 0,023].Conclusion:This study shows a high prevalence of lung involvement in ERA patients of less 1 year from diagnosis. Also, we showed a significant association between HRCT and physical examination findings. This data highlights the importance of the clinical examination in Rheumatoid Arthritis patients. More studies with bigger samples and longitudinal follow up are needed to confirm and complete our results.References:[1]Rooney BK, Silman AJ. Epidemiology of the rheumatic diseases. Curr Opin Rheumatol [Internet]. 1999 Mar [cited 2016 Jul 19];11(2):91–7. Available from:http://www.ncbi.nlm.nih.gov/pubmed/10319210.[2]Antin-Ozerkis D, Evans J, Rubinowitz A, Homer RJ, Matthay RA. Pulmonary Manifestations of Rheumatoid Arthritis. Clin Chest Med [Internet]. 2010;31(3):451–78. Available from:http://dx.doi.org/10.1016/j.ccm.2010.04.003.Disclosure of Interests:None declared

Background:Interstitial lung disease (ILD) is one of the more frequent and potentially severe extra-articular manifestation of rheumatoid arthritis (RA). ILD significantly decreases the survival and quality of life of patients and influences the treatment approach to the patient.Despite its clinical relevance, the prevalence, incidence and survival of RA-ILD is unknown and supposed on the base of retrospective data or registry-based studies.Objectives:For the first time, the Lung Involvement in Rheumatoid Arthritis (LIRA) study aims to investigate epidemiology, features and prognosis of RA-ILD patients in a prospective international multicentre study.Methods:All RA patients referring to the involved centres will be evaluated every six months with a digital stethoscope and a software able to identify velcro crackles with a diagnostic accuracy of 83.9% (VECTOR). In fact, velcro crackles are virtually identified in all stages of fibrosing alveolitis like RA-ILD, and their search is as a simple and reliable method to screening patients to be undergone to high resolution computed tomography (HRCT).For each patient, clinical and serological data are recorded at baseline and every six months; when velcro crackles or other conditions suspicious for ILD, such as cough or dyspnoea, are detected, a HRCT is requested to confirm ILD. Patients with ILD periodically perform pulmonary function tests to monitor lung function evolution.Results:At now, 205 RA patients have been enrolled (female/male 161/44, mean age 64.8±12.9 years, mean disease duration 14.2±8.9 years), anti-citrullinated peptides antibodies (ACPA) and rheumatoid factor (RF) were positive in 77.1% and 78.1%, respectively. The prevalence of ILD was 21% (43 patients). In other 13 patients the HRCT is ongoing; therefore, we could suppose up to a prevalence of 27.3%. Patients with ILD were symptomatic in 53.5% of cases (23 patients), they are more frequently males and were older than patients without ILD (mean age 73.2±7.4 and 62.7±13.2; pConclusion:The prevalence and the incidence of RA-ILD is still not well defined. Preliminary data of our study confirm a prevalence of ILD higher than 20%, patients are asymptomatic in almost the half of cases and more frequently males and elderly. Our study can help to define the clinical history of these patients, the possible association with clinical and serological features and the supposed role of some drugs.References:[1]Manfredi A, et al. Diagnostic accuracy of a velcro sound detector (VECTOR) for interstitial lung disease in rheumatoid arthritis patients: the InSPIRAtE validation study (INterStitial pneumonia in rheumatoid ArThritis with an electronic device). BMC Pulm Med. 2019;19:111.[2]Bendstrup E, et al. Interstitial Lung Disease in Rheumatoid Arthritis Remains a Challenge for Clinicians. J Clin Med. 2019:8Disclosure of Interests:Marco Sebastiani: None declared, Caterina Vacchi: None declared, Giulia Cassone: None declared, Fabiola Atzeni: None declared, Martina Biggioggero: None declared, Antonio Carriero: None declared, Gian Luca Erre: None declared, Anna Laura Fedele: None declared, Federica Furini: None declared, Paola Tomietto: None declared, Vincenzo Venerito: None declared, Belén Atienza-Mateo: None declared, Giovanni Della Casa: None declared, Stefania Cerri: None declared, Gilda Sandri: None declared, Adalgisa Palermo: None declared, Elena Galli: None declared, Fabrizio Pancaldi: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Carlo Salvarani: None declared, Andreina Manfredi: None declared

ObjectiveTo explore the clinical pathways, including signs and symptoms, and symptom progression patterns preceding idiopathic pulmonary fibrosis (IPF) diagnosis.Design and settingA historical cohort study was conducted using primary care patient records from the Optimum Patient Care Research Database.ParticipantsPatients included were at least 30 years, had IPF diagnosis, identified via clinical-coding and free-text records and had a consultation with a chest specialist prior to IPF diagnosis.Outcome measuresThe signs and symptoms in the year prior to IPF diagnosis from clinical codes and free-text in primary care electronic records included: cough, dyspnoea, dry cough, weight loss, fatigue/malaise, loss of appetite, crackles and clubbed fingers. The time course of presentations of clinical features and investigations in the years prior to IPF diagnosis were mapped.ResultsWithin 462 patients identified, the majority (77.9%) had a respiratory consultation within 365 days prior to the chest specialist visit preceding the IPF diagnosis recorded in their primary care records. The most common symptoms recorded in the 1 year prior to IPF diagnosis were dyspnoea (48.7%) and cough (40.9%); other signs and symptoms were rarely recorded (ConclusionProlonged cough and/or progressive dyspnoea, especially if accompanied with weight loss, should signal for a referral to specialist assessment at the earliest opportunity.

BackgroundIn contrast with the technical progress of the stethoscope, lung sound terminology has remained confused, weakening the usefulness of auscultation. We examined how observer preferences regarding terminology and auscultatory skill influenced the choice of terms used to describe lung sounds.MethodsThirty-one staff physicians (SP), 65 residents (R) and 47 medical students (MS) spontaneously described the audio recordings of 5 lung sounds classified acoustically as: (1) normal breath sound; (2) wheezes; (3) crackles; (4) stridor and (5) pleural friction rub. A rating was considered correct if a correct term or synonym was used to describe it (term use ascribed to preference). The use of any incorrect terms was ascribed to deficient auscultatory skill.ResultsRates of correct sound identification were: (i) normal breath sound: SP=21.4%; R=11.6%; MS=17.1%; (ii) wheezes: SP=82.8%; R=85.2%; MS=86.4%; (iii) crackles: SP=63%; R=68.5%; MS=70.7%; (iv) stridor: SP=92.8%; R=90%; MS=72.1% and (v) pleural friction rub: SP=35.7%; R=6.2%; MS=3.2%. The 3 groups used 66 descriptive terms: 17 were ascribed to preferences regarding terminology, and 49 to deficient auscultatory skill. Three-group agreement on use of a term occurred on 107 occasions: 70 involved correct terms (65.4%) and 37 (34.6%) incorrect ones. Rate of use of recommended terms, rather than accepted synonyms, was 100% for the wheezes and the stridor, 55% for the normal breath sound, 22% for the crackles and 14% for the pleural friction rub.ConclusionsThe observers’ ability to describe lung sounds was high for the wheezes and the stridor, fair for the crackles and poor for the normal breath sound and the pleural friction rub. Lack of auscultatory skill largely surpassed observer preference as a factor determining the choice of terminology. Wide dissemination of educational programs on lung auscultation (eg, self-learning via computer-assisted learning tools) is urgently needed to promote use of standardised lung sound terminology.

An 87-year-old man was admitted to Jikei University Hospital presenting with fever and fatigue. Physical examinations revealed inspiratory coarse crackles on the left lung field. A chest CT detected a tumour-like consolidation around the thoracic aorta (figure 1A). Biochemical examinations found a high number of white blood cells and elevated C-reactive protein level, and a diagnosis of pneumonia was made. Penicillin antibiotics (ampicillin/sulbactam) were administered. Two days later, methicillin-sensitive Staphylococcus aureus (MSSA) was detected in two sets of blood cultures. An echocardiogram showed no vegetation and a urine culture was negative. A sputum culture was unobtainable, but pneumonia was the only focus of infection for the MSSA. Penicillin antibiotics were continued, but the patient suddenly developed cardiopulmonary arrest …

Neuroendocrine cell hyperplasia of infancy (NEHI) has recently been described as an obstructive airway disease that affects infants aged 1-24 months, and presents typically with tachypnoea, crackles and hypoxia. The pathogenesis of the disease is unknown. We describe the clinical course of nine infants with radiologically and histologically confirmed NEHI. Host or environmental factors were not associated with the disease development. All infants with lung function tests demonstrated findings consistent with severe irreversible peripheral airway obstruction, assessed with whole body plethysmography (6/6) or the rapid thoracoabdominal compression technique (5/5). While the symptoms abated in all infants, six infants developed a non-atopic asthma during the follow-up. Systemic or inhaled corticosteroid treatment did not affect the duration of the symptoms. NEHI may mimic severe asthma and thus this entity should be taken into account when evaluating infants with chronic respiratory symptoms.

Background Diagnosing early stages of heart failure with mild symptoms is difficult. B-type natriuretic peptide (BNP) has promising biochemical test characteristics, but its diagnostic yield on top of readily available diagnostic knowledge has not been sufficiently quantified in early stages of heart failure. Objectives To quantify the added diagnostic value of BNP for the diagnosis of heart failure in a population relevant to GPs and validate the findings in an independent primary care patient population. Design Individual patient data meta-analysis followed by external validation. The additional diagnostic yield of BNP above standard clinical information was compared with ECG and chest x-ray results. Patients and methods Derivation was performed on two existing datasets from Hillingdon (n=127) and Rotterdam (n=149) while the UK Natriuretic Peptide Study (n=306) served as validation dataset. Included were patients with suspected heart failure referred to a rapid-access diagnostic outpatient clinic. Case definition was according to the ESC guideline. Logistic regression was used to assess discrimination (with the c-statistic) and calibration. Results Of the 276 patients in the derivation set, 30.8% had heart failure. The clinical model (encompassing age, gender, known coronary artery disease, diabetes, orthopnoea, elevated jugular venous pressure, crackles, pitting oedema and S3 gallop) had a c-statistic of 0.79. Adding, respectively, chest x-ray results, ECG results or BNP to the clinical model increased the c-statistic to 0.84, 0.85 and 0.92. Neither ECG nor chest x-ray added significantly to the ‘clinical plus BNP’ model. All models had adequate calibration. The ‘clinical plus BNP’ diagnostic model performed well in an independent cohort with comparable inclusion criteria (c-statistic=0.91 and adequate calibration). Using separate cut-off values for ‘ruling in’ (typically implying referral for echocardiography) and for ‘ruling out’ heart failure—creating a grey zone—resulted in insufficient proportions of patients with a correct diagnosis. Conclusion BNP has considerable diagnostic value in addition to signs and symptoms in patients suspected of heart failure in primary care. However, using BNP alone with the currently recommended cut-off levels is not sufficient to make a reliable diagnosis of heart failure.

A 54-year-old female presented with chronic dry cough and dyspnoea over 3 months and was referred to our outpatient clinic. She had no history of smoking, allergy or respiratory disorders. Chest X-ray demonstrated bilateral upper lung predominant consolidation (figure 1A). Chest CT revealed extensive upper lobe predominant subpleural consolidation with air bronchograms as well as extensive ground glass opacities (GGOs) with intralobular septal thickening (figure 1B–D). Mediastinal lymphadenopathy in stations 4R and 10R with no calcification was observed. There were no crackles on auscultation with oxygen saturation of 95% on room air despite a wide range of abnormal shadows on chest X-ray and CT. Based on the available clinical and radiographic findings, the differential diagnosis encompassed pulmonary sarcoidosis, malignant lymphoma, bronchoalveolar carcinoma, lymphangitis carcinomatosa and amyloidosis. Figure 1 Chest X-rays and CT scans. Chest …

A 34-year-old Caucasian man with chronic renal failure on peritoneal dialysis presented with a 10-day history of productive cough, worsening dyspnoea and haemoptysis. He was a non-smoker, with no previous respiratory illnesses. There was no history of foreign travel or significant domestic or environmental exposures. He was feverish and tachypnoeic with scattered crackles throughout both lungs and mildly hypoxic (oxygen saturation 92% on air). He had no peripheral oedema. His primary renal disease was focal segmental glomerulosclerosis with frequently relapsing nephrotic syndrome since childhood. Medication included long-term immunosuppressive therapy with tacrolimus and corticosteroids (5 mg prednisolone), and he had received cyclophosphamide and levamisole during childhood. He had started fluoxetine hydrochloride 20 mg daily for depression 6 months earlier. Renal transplantation was being considered. A chest radiograph showed bilateral patchy consolidation. Pneumonia was diagnosed clinically, and he was treated with oral antibiotics and prednisolone dose doubled. Initially better, he was readmitted 2 weeks later with worsening breathlessness. He had raised …

A 24-year-old woman came to the emergency room of our hospital, 6 hours after a sting to the pulp of her middle finger of her left upper limb by an Indian red scorpion. On examination, she had tachycardia, tachypnoea, hypotension and low oxygen saturation at room temperature. On auscultation, there was poor air entry in all areas of the right lung along with crackles. The left lung field was normal on auscultation. Chest X-ray showed unilateral haziness of right lung field. Two-dimensional echocardiography showed clinical findings of myocarditis. Arterial blood gas showed metabolic acidosis with severe hypoxaemia, suggestive of type 1 respiratory failure. After intubation and initiating ventillatory support, the patient was given intravenous analgesics, antihistaminic and infiltrated of site of bite with 2% xylocaine. The patient was started on inotropes, alpha receptor blocker, intravenous steroids, bronchodilators and diuretics support. The patient clinically improved over the course of treatment and was subsequently discharged.

To present diagnosis and treatment modalities of children with interstitial lung disease associated with frequent or rare surfactant protein C gene (SFTPC) mutation.Twenty-two children with chronic lung disease associated with SFTPC mutation in a heterozygous form.Mutations located in the BRICHOS domain ('BRICHOS domain' group) were identified in six children, whereas 16 children carried mutations located outside the BRICHOS domain ('non-BRICHOS domain' group). The median age of onset was 3 (0-24) months. Four patients had neonatal respiratory distress, and symptom onset was associated with acute bronchiolitis in nine patients. Cough, tachypnoea and failure to thrive were initially noticed in all the children. Physical examination at presentation revealed tachypnoea (n=22), clubbing (n=1) and crackles (n=5). Low oxygen saturation (95%) was observed in 18 patients. The predominant findings on initial high-resolution CT (HRCT) scans were basal-predominant ground-glass opacity (n=21) and cystic spaces (n=3). Bronchoalveolar lavage fluid (BALF) cell counts showed 379+/-56x10(3) cells/ml with increased neutrophil percentage (18+/-4%) independent of the mutation status. The median follow-up was 3.2 (1-18.3) years. Eighteen patients were treated by monthly methylprednisolone pulses associated with oral prednisolone (n=16), hydroxychloroquine (n=11) and/or azithromycin (n=4). Fifteen patients benefited from enteral nutrition.Initial diagnosis is based on clinical presentation, radiological features and BALF analysis, but the definitive diagnosis requires genetic analysis. Although progressive improvement was seen in most patients, the development of new therapeutic strategies with minimal side effects is needed.

A 68-year-old woman came to our hospital because of a disturbance in consciousness the previous day and fever 3 days prior. She had no abdominal pain, nausea, vomiting or diarrhoea prior to admission. She had diabetes and haemoglobin A1c of 7.0%. Vital signs at the time of her visit were as follows: body temperature 40.5°C, blood pressure 137/63 mm Hg, heart rate 120 bpm and a respiratory rate of 50/min. She had jaundice. Chest examination revealed pan-inspiratory crackles and she had blood sputum. There was no knocking pain around the liver, nor Murphy’s sign. The blood test revealed a white cell count of 3100/μL (normal range 4000–9000/μL). Her haemoglobin was 6.9 g/dL (normal range 13.5–17.6 g/dL). Her mean corpuscular volume (MCV) could not be measured because of significant haemolysis; however, …

Background: Mutations in the ABCA3 gene can result in fatal surfactant deficiency in term newborns and chronic interstitial lung disease in older children. Previous studies on ABCA3 mutations focused primarily on the genetic abnormalities and reported limited clinical information about the resultant disease. Objective: To systematically analyze the clinical presentation, pulmonary function, diagnostic imaging, pathologic features, and outcomes of children with ABCA3 mutations. Methods: The records of nine children with ABCA3 mutations evaluated at Texas Children9s Hospital between 1992 and 2005 were reviewed and their current clinical status updated. Previous diagnostic imaging studies and lung biopsy specimens were reexamined. Results of DNA analyses were confirmed. Results: Age at symptom onset ranged from birth to four years. Cough, crackles, failure to thrive, and clubbing were frequent findings. Mean lung function was low but tended to remain static. Computed tomography scans commonly revealed ground-glass opacification, septal thickening, parenchymal cysts, and pectus excavatum. Histopathologic patterns included pulmonary alveolar proteinosis, desquamative interstitial pneumonitis, and nonspecific interstitial pneumonitis and varied with age. Dense abnormalities of lamellar bodies, characteristic of ABCA3 mutations, were seen by electron microscopy in all adequate specimens. Outcomes varied with the age at which the severity of lung disease warranted open lung biopsy, and some patients have had prolonged survival without lung transplantation. Conclusions: The presentation and course of interstitial lung disease due to ABCA3 mutations are variable, and open lung biopsy and genetic testing are warranted early in the evaluation of children with a consistent clinical picture.

Case 1 A 63 year old white woman was admitted to hospital in February 2000 owing to rapidly increasing dyspnoea on exertion (NYHA III), chest pain, and fever. The presence of sclerodactyly with loss of distal parts of the fingers, and megacapillaries at capillaroscopy, allowed the diagnosis of limited cutaneous systemic sclerosis (fig 1). Physical examination also disclosed crackles in the lower lobes of the lungs, systolic murmur with loud pulmonary second sound, and feet oedema. Figure 1 Case 1. (A) Chest x ray findings show bibasilar reticular opacities, cardiomegaly with accentuation of the right ventricle, and enlargement of the pulmonary artery, which are consistent with interstitial lung disease and pulmonary hypertension. (B) High resolution CT of the lungs discloses features of interstitial lung disease with “ground glass” opacities and widening of the central blood vessels. (C) Doppler echocardiography shows blood regurgitation through the tricuspid valve, which allows estimation of PASP. (D) Perfusion scintigraphy of the lungs carried out in February 2000 (D/1) shows redistribution of the blood flow into the upper parts of the lungs and irregular areas of diminished blood flow in the peripheral parts of the lungs consistent with PAH and pulmonary thromboembolism. For comparison, perfusion scintigraphy of the lungs carried out in June 2000 (D/2) shows a significant increase in blood flow in the lower parts of both lungs, which paralleled a decrease in PASP as measured with Doppler echocardiography. Laboratory tests included high erythrocyte sedimentation rate (145 mm/1st h), C reactive protein (2530 mg/l, normal

Objective: To investigate the validity and reliability of computerised acoustic analysis in the detection of abnormal respiratory noises in infants. Methods: Blinded, prospective comparison of acoustic analysis with stethoscope examination. Validity and reliability of acoustic analysis were assessed by calculating the degree of observer agreement using the κ statistic with 95% confidence intervals (CI). Results: 102 infants under 18 months were recruited. Convergent validity for agreement between stethoscope examination and acoustic analysis was poor for wheeze (κ = 0.07 (95% CI, −0.13 to 0.26)) and rattles (κ = 0.11 (−0.05 to 0.27)) and fair for crackles (κ = 0.36 (0.18 to 0.54)). Both the stethoscope and acoustic analysis distinguished well between sounds (discriminant validity). Agreement between observers for the presence of wheeze was poor for both stethoscope examination and acoustic analysis. Agreement for rattles was moderate for the stethoscope but poor for acoustic analysis. Agreement for crackles was moderate using both techniques. Within-observer reliability for all sounds using acoustic analysis was moderate to good. Conclusions: The stethoscope is unreliable for assessing respiratory sounds in infants. This has important implications for its use as a diagnostic tool for lung disorders in infants, and confirms that it cannot be used as a gold standard. Because of the unreliability of the stethoscope, the validity of acoustic analysis could not be demonstrated, although it could discriminate between sounds well and showed good within-observer reliability. For acoustic analysis, targeted training and the development of computerised pattern recognition systems may improve reliability so that it can be used in clinical practice.

An 80-year-old woman was admitted with a history of cough and gradually worsening shortness of breath of 3 months duration. Respiratory system examination revealed tracheal shift to the left, together with impaired percussion, diminished breath sounds and crackles at the left base. Her medical history included chronic renal failure for which she received haemodialysis three times a week. Her current medications included Sevelamer tablets 800 mg three times a day. The patient was on this particular medication for a period of 10 months prior to admission. There was no history of any …

BACKGROUNDFibrosing alveolitis (FA) is a common and serious complication of rheumatoid arthritis (RA). Before the availability of high resolution computed tomographic (HRCT) scanning, it was difficult to diagnose accurately without recourse to biopsy. Prospective studies have reported a prevalence of interstitial lung disease (ILD) of 19–44%. The term ILD used by these authors encompasses a variety of appearances on HRCT scans. This prospective study used HRCT scanning to determine the true prevalence of FA in hospital outpatients with RA, and to study associated clinical characteristics.METHODSOne hundred and fifty consecutive patients with RA were selected from a hospital outpatient department, irrespective of the presence or absence of chest disease. All underwent a detailed clinical assessment, chest HRCT scanning, and conventional chest radiography within 4 weeks of full pulmonary function tests.RESULTSSeventy percent of patients were current or reformed cigarette smokers. Twenty eight (19%) had FA, most frequently of reticular pattern, and 12 of this group (43%) also had emphysematous bullae. None of the previously suggested risk factors for developing FA were confirmed. Fifty four percent of patients with HRCT evidence of FA had bilateral basal chest crackles, 82% had a reduced carbon monoxide transfer factor (Tlco), 14% had restrictive pulmonary function tests, and 14% had bilateral chest radiographic signs of FA.CONCLUSIONSHRCT evidence of FA was present in 19% of hospital outpatients with RA. Abnormalities on chest examination or on full pulmonary function tests, even without restrictive changes or chest radiographic abnormalities, should prompt physicians to request a chest HRCT scan when investigating dyspnoea in patients with RA.

A 30-year-old African–American man, a truck driver, with a known history of gastro-oesophageal reflux disease, presented with shortness of breath, dry cough and malaise. He was recently hospitalised for community-acquired pneumonia and treated with moxifloxacin, initially intravenously and later orally, after discharge. He felt subjectively better for a few days and then started having more symptoms, which prompted him to return to the emergency department. On examination, he was febrile, tachycardic and tachypnoeic. On auscultation, he had coarse crackles on the right side of his lung fields. Laboratory data showed a white cell count of 18.5, with neutrophil predominance. Chest X-ray revealed infiltrates bilaterally but most prominently on the right (figure 1). He was started on broad-spectrum antibiotics for presumed atypical or nosocomial infection. Within the next 2 days his condition deteriorated and …

BACKGROUND--Crackles are a prominent clinical feature of asbestosis and may be an early sign of the condition. Auscultation, however, is subjective and interexaminer disagreement is a problem. Computerised lung sound analysis can visualise, store, and analyse lung sounds and disagreement on the presence of crackles is minimal. High resolution computed tomography (HRCT) is superior to chest radiography in detecting early signs of asbestosis. The aim of this study was to compare clinical auscultation, time expanded wave form analysis (TEW), chest radiography, and HRCT in detecting signs of asbestosis in asbestos workers. METHODS--Fifty three asbestos workers (51 men and two women) were investigated. Chest radiography and HRCT were assessed by two independent readers for detection of interstitial opacities. HRCT was performed in the supine position with additional sections at the bases in the prone position. Auscultation for persistent fine inspiratory crackles was performed by two independent examiners unacquainted with the diagnosis. TEW analysis was obtained from a 33 second recording of lung sounds over the lung bases. TEW and auscultation were performed in a control group of 13 subjects who had a normal chest radiograph. There were 10 current smokers and three previous smokers. In asbestos workers the extent of pulmonary opacities on the chest radiograph was scored according to the International Labour Office (ILO) scale. Patients were divided into two groups: 21 patients in whom the chest radiograph was > 1/0 (group 1) and 32 patients in whom the chest radiograph was scored < or = 1/0 (group 2) on the ILO scale. RESULTS--In patients with an ILO score of < or = 1/0 repetitive mid to late inspiratory crackles were detected by auscultation in seven (22%) patients and by TEW in 14 (44%). HRCT detected definite interstitial opacities in 11 (34%) and gravity dependent subpleural lines in two (6%) patients. All but two patients with evidence of interstitial disease or gravity dependent subpleural lines on HRCT had crackles detected by TEW. In patients with an ILO score of > 1/0 auscultation and TEW revealed mid to late inspiratory crackles in all patients, whereas HRCT revealed gravity dependent subpleural lines in one patient and signs of definite interstitial fibrosis in the rest. In normal subjects crackles different from those detected in asbestosis were detected by TEW in three subjects but only in one subject by auscultation. These were early, fine inspiratory crackles. CONCLUSION--Mid to late inspiratory crackles in asbestos workers are detected by TEW more frequently than by auscultation. Signs of early asbestosis not apparent on the plain radiograph are detected by TEW and HRCT with similar frequency. off

In a randomised single blind trial the Chinese herbs Shuang Huang Lian were evaluated for the treatment of acute bronchiolitis. Children with acute bronchiolitis and serological evidence of recent respiratory syncytial virus infection were studied in a tertiary hospital in Harbin, China. The 96 children were randomised into three treatment groups: herbs, herbs with antibiotics, and antibiotics alone. The herbs were prepared by the medical school pharmacy and administered daily by intravenous infusion for seven days. The main outcomes, assessed blindly, were symptomatic improvement in cough, fever, wheezing, chest signs, and duration of stay in hospital. The mean duration of symptoms from the start of treatment was 6.2 (confidence interval 5.6 to 6.9) days in the two groups treated with herbs compared with 8.6 (confidence interval 7.5 to 9.8) days in the group treated with antibiotics alone. The mean reductions in duration of clinical manifestations for treatment with antibiotics alone compared with herbs were: from 3.1 to 1.5 days for fever, 9.1 to 6.1 days for cough, 6.5 to 4.1 days for wheezing, and 7.2 to 4.9 days for chest crackles. No adverse effect of Shuang Huang Lian herbal treatment was detected. In conclusion, this study confirms Chinese experience with Shuang Huang Lian that it is safe and effective, and warrants further study.

A 62 year old woman was referred to hospital by her general practitioner because of a few days history of progressive shortness of breath. She had associated fever but no cough or sputum production. Her medical history included rheumatoid arthritis, for which she had been taking methotrexate for the past 12 years. On examination, she was visibly dyspnoeic, with low oxygen saturations. Her respiratory rate was 22 breaths/min, oxygen saturation was 90% on air, heart rate was 100 beats/min, blood pressure was 115/72 mm Hg, and her temperature was 37.4oC. Chronic hand changes, consistent with rheumatoid arthritis, were noted. Bilateral widespread crackles were heard on auscultation of the chest. Blood tests showed a white blood cell count of 8.8×109/L (reference range 4-10) and a C reactive protein of 1219 nmol/L (

Background Lung ultrasound (LUS) was reported to have a good diagnostic accuracy in the assessment of rheumatoid arthritis-related interstitial lung disease (RA-ILD), as compared to high resolution CT (HRCT), currently considered the diagnostic gold standard. However, partial verification, leading to biased estimates of the accuracy of LUS, is the rule in these studies, as the probability of undergoing HRCT is higher in patients with ILD. Objectives To assess the influence of verification bias in the assessment of the diagnostic accuracy of LUS in the detection of RA-ILD Methods We analyzed the data of a previously reported study comparing the diagnostic accuracy of LUS in comparison with usual clinical practice to detect RA-ILD [1]. LUS was performed in 152 RA patients, and its accuracy in the detection of ILD was compared to 3 clinical algorithms (Alg1: bibasilar crackles and dyspnea; Alg2: as Alg1 + reduced pulmonary function tests (PFTs); Alg3: as Alg1 + positive Chest X-Ray). By design, partial verification was significant: only 71/152 patients were verified with HRCT, 49/152 had PFTs, and 72/152 had Chest X-Ray. Estimated of sensitivity and specificity based on observed data (missing completely at random model MCAR), were compared with the estimates obtained with the Begg-Greenes method (missing at random assumption, MAR) [2], and with multivariate imputation (multivariate imputation by chained equations, MICE) [3]. Results Accuracy estimates based on complete data only (MCAR) differ significantly from the MAR estimates and from MICE results, and tend to inflate sensitivity estimates. Figure 1 reports the point sensitivity and specificity estimates under different assumptions for LUS and the 3 clinical algorithms, and the “ignorance region” as defined by Kosinski and Barnhart [4]. LLUS had the highest diagnostic accuracy. However, LUS estimates of sensitivity and specificity varied from 63.4% to 88.2%, and from 68.5% to 81.3% respectively. Figure 2 reports the influence of estimation model on the likelihood ratios of RA-ILD based on LUS results. LR+ varied from 2.80 to 4.33, and LR- from 0.17 to 0.45. Conclusions Verification bias can severely affect estimates of diagnostic accuracy of LUS in RA-ILD, and should be always taken into account in the interpretation of results. References Antivalle M, et al. Lung Ultrasound Screening for Interstitial Lung Disease in Rheumatoid Arthritis. Comparison with Usual Detection Algorithms in Clinical Practice. Arthritis Rheum 2014;66(suppl): S1301 Begg CB, R.A. Greenes, Assessment of diagnostic tests when disease verification is subject to selection bias. Biometrics 1983;39:207–215. van Buuren S. Multiple imputation of discrete and continuous data by fully conditional specification. Stat Methods Med Res. 2007;16:219-42. Kosinski AS, Barnhart HX, Accounting for nonignorable verification bias in assessment of diagnostic tests, Biometrics 2003; 59: 163–171. Disclosure of Interest None declared

A 44-year-old man presented to hospital with haemoptysis and progressive dyspnoea. Examination revealed room air oxygen saturation of 91% with diffuse chest crackles, gynaecomastia and hepatosplenomegaly. Scrotal examination was unremarkable. Radiological investigations revealed numerous bilateral pulmonary nodules of variable size (cannonball appearance) (figure 1), gynaecomastia (figure 2A), hepatosplenomegaly and a large retroperitoneal mass (figure 2B). Scrotal ultrasound showed no abnormalities. Figure 1 Plain posteroanterior chest radiograph (A) and axial CT scan in the lung window (B) showing numerous bilateral …

Resident: I would like to present the case of one of my new patients. She is a 63-year-old Caucasian woman with long-term sarcoidosis of the lungs and multiorgan involvement including liver and spleen. Despite pneumological surveillance and prednisolone treatment, her respiratory situation worsened, and was started on home oxygen treatment 5 years ago when fibrosis of the lungs was diagnosed. She also has severe osteoporosis due to long-term steroid use and immobility. She is now presenting with worsening dyspnoea of a duration of 4 months despite using 8 litres of oxygen. I admitted a prematurely aged woman in a poor general condition. She presented with dyspnoea, right upper quadrant abdominal pain and back pain. A physical examination revealed stigmata of hypoxaemia, light crackles on both lungs, clinical signs for steroid use and an enlarged liver painful on palpation. She is a non-smoker and she has no history of cardiac diseases or cardiac involvement by sarcoidosis. An echocardiogram was normal and right heart catheterisation 2 months ago showed no pulmonary hypertension. There was no fever or relevant inflammation parameters. Laboratory tests revealed a mild hyperbilirubinaemia of 1.8 mg/dl, slightly reduced albumin of 32 g/litre, normal transaminases and an elevated γ-glutamyl transpeptidase (GGT) (981 U/litre) and alkaline phosphatase (AP) (559 U/litre). Her International Normalised Ratio (INR) was within the normal range. An ultrasound scan showed an enlarged liver and spleen and some ascites. Consultant: Concerning her respiratory situation, we have to think of a worsening of lung fibrosis due to sarcoidosis, since there is no trace of cardiac or infectious conditions so …

Background and aims Echocardiograms are a major resource for diagnosing congenital and acquired heart disease. Paediatricians with special expertise in Paediatric Cardiology are now at the forefront of the paediatric cardiac care service. The aim of this audit was to report on the quality of referrals – investigating the indications for referral, documentation of symptoms and signs. Methods The medical notes of 46 children who attended outpatient echocardiogram clinic were retrospectively reviewed (April to June 2012). We looked at indications for referral, documentation of symptoms and signs in the initial referral letters. Results 41 out of 46 children attended the clinics, of which 39 sets of notes were available to be reviewed. 22 were new and 17 were follow-ups. The main indications for referral were presence of murmur, family history, congenital heart disease, syndromes and Kawasaki Disease. 16 children were referred because of murmur, 12 of whom were asymptomatic. Half did not comment on grade, position or radiation of the murmur. One-third did not have documentation of femoral pulses, signs of heart failure (crackles on auscultation, hepatomegaly, oedema). Conclusions Indications for referral to echocardiogram clinic were appropriate. However, the quality of documentation of basic examination findings was poor. This audit starts a discussion regarding setting the standard for quality of referrals to echocardiogram clinic, and highlights deficiencies in documentation. Recommendations include a standardised pro forma and an emphasis during general practice study days.

Objectives To analyze the effects of cyclophosphamide (CYC) pulse therapy on parameters of lung function in patients (pts) with systemic sclerosis (SSc). Methods Thirty pts with SSc (25 women and 5 men, aged 30-70 years, mean disease duration 1,6 (group II). Monthly CYC pulses in a dose of 500 mg/m 2 body surface were given for the period of 6 months, followed by 3-monthly frequency maintenance pulses treatment for the next 18 months. The efficacy was evaluated by comparing FVC% and DLco% at the baseline, 1 month after the sixth pulse, and after 24 months from the start of therapy. Results At baseline, there were no significant group differences for the age of onset of SSc, digital ulcers or scars, arthritis, dyspnea and fatigue, presens of basilar Velcro-like crackles on lung auscultation, presens of fibrosis on chest radiography, gastroesophagel reflux, renal involvement and assessed value of the right ventricular systolic pressure. Pts from group II had an earlier onset of Raynaud9s phenomenon (RP), longstanding RP, diffuse cutaneous SSc, systemic inflamation defined by increase in CRP, anticentromere antibody positivity, higher values of FVC% and lower values of DLco% than those with group I (46.15±17.82 years; 5.82±5.99 years; 13 pts; 12.25±26.02; 7 pts; 105.05±15.37%; 58.21±7.94% versus 51.96±9.6 years; 2.96±3.25 years; 6 pts; 10.70±8.53;2 pts; 85±12.79% and 69.28±9.91% respectively; p Conclusions Our results suggest that CYC was able to prevent deterioration of FVC after 6 and 24 months in all pts with SSc and lung involvement. Surprisingly enought, the fact is that only in patients with FVC%/DLco%>1.6, having a risk factor for future development of SSc associated pulmonary arterial hypertension, CYC was able to prevent deterioration of DLco after 6 months of therapy, but not such results were observed after 24 months of therapy. The maintenance of treatment with CYC may be necessary only for patients with FVC%/DLco% Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3010

Background Methotrexate pneumonitis (MTX-P) is most frequently seen within the first year of treatment 1 and is best avoided in established cases of Interstitial Lung disease (ILD). BSR recommends that if pre-treatment Chest X-Ray (CXR) is abnormal then consider High Resolution Computed Tomography (HRCT) and Pulmonary Function tests (PFTs) to ascertain TLCO, but TLCO may be more sensitive in some cases. Recent studies have suggested that incidence of MTX-P is not as common as previously thought 2,3 . Clinical presentation is usually of male smokers with pre-existing lung disease presenting with respiratory symptoms and signs and raised ESR 4,5 . Objectives To study if spiromtery alone, rather than full PFTs would be sufficient in patients without risk factors when commencing Methotrexate Methods In August 2013 we carried out an audit of 58 patients who were on Methotrexate for at least greater than 6 months for any Autoimmune Inflammatory Rheumatic Disease (AIIRD). Results 43 patients were between 31-70 years of age, range being 15-80 years. 22 were males and 36 females. 36 patients had RA, 7 had Psoriatic arthritis and the rest had some other AIIRD. Patients audited had started MTX from 1996 up to 2013 (show table). 49 patients were on MTX for more than 12 months. Baseline CXR was done in 46 patients. 12 patients each were either current or ex-smokers. 29 patients were not checked for crackles in chest at the time of MTX commencement. Among the rest, only one had crackles. Two patients complained of breathlessness on MTX commencement. Baseline PFTs were done in 39 patients but only 34 had their transfer co-efficient (KCO) checked. One patient had restrictive picture on PFTs and ten had an obstructive picture. KCO was less than 70% in 5 patients who were all current or ex-smokers. HRCT was performed in 4 of these. One patient, who was a smoker and had obstructive PFTs, had fibrosis with alveolitis related to RA and MTX was stopped after initial few doses. MTX was started in the other three. Repeat PFTs were required in three patients for respiratory symptoms and as KCO was >70%, MTX was continued in all three. Two patients, who had not had a baseline PFT, had one, ten years after being on MTX and it was continued in both of them. Conclusions None of the patients developed MTX-P and only one had ILD related to RA. Our results suggest that if we just do a baseline spirometry when commencing MTX, except in patients with risk factors, then we can save on National Health Service budget, as the cost of full PFTs is £250/– and of spirometry alone is £ 60/– 2 , but we ideally need larger numbers to study to make final conclusions References Imokawa S, Colby TV, Leslie KO, Helmers RA. Methotrexate Pneumonitis: review of the literature and histopathological findings in nine patients. Eur Respir J 2000;15:373–81 Sathi N et al. How common is methotrexate pneumonitis? A large prospective study investigates. Clin Rheumatol. 2012 Jan;31(1):79-83 Salliot C et al. Long-term safety of methotrexate monotherapy in patients with rheumatoid arthritis: a systematic literature research. Ann Rheum Dis. 2009 Jul;68(7):1100-4 Searles G et al. Methotrexate pneumonitis in rheumatoid arthritis: potential risk factors. Four case reports and a review of the literature. J Rheumatol. 1987 Dec;14(6):1164-71 Hargreaves MR et al. Acute pneumonitis associated with low dose methotrexate treatment for rheumatoid arthritis: report of five cases and review of published reports. Thorax. 1992 Aug;47(8):628-33. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1294

Background Interstitial lung disease (ILD) in rheumatoid arthritis (RA) is frequent. Joint disease precedes the interstitial lung involvement by several years in most cases, although ILD may be the first RA manifestation. Objectives The aim of this study was to evaluate the prevalence and clinical presentation of ILD in patients with early RA, and to identify factors associated with its occurrence. Methods 48 patients (34 women and 14 men) with early RA, fulfilling 2010 ACR-EULAR classification criteria were enrolled to the study. The mean age was 59.6 years (±15.7). The mean disease duration was 4.7 months. Detailed medical and smoking history was taken. A physical examination, laboratory analysis, PFT and HRCT of the lung were performed in all patients. Pulmonary manifestations were defined as the presence of respiratory symptoms and abnormalities revealed on physical examination of the lung. The diagnosis of ILD was based on high resolution computed tomography (HRCT) of the lung and defined as the presence of diffuse or patchy reticular or reticulo-nodular lesions, honeycombing and/or ground-glass opacities, localized mainly in the peripheral or subpleural parts of the lower lobes. Non-specific interstitial lung lesions were defined as the presence of parenchymal or subpleural bands, or pleural thickening. Results Based on the definitions above, ILD was diagnosed in 11 patients (23%) and non-specific interstitial lung lesions in 20 patients (41.6%). Among patients with ILD, 6 had reticular lesions, 3 had ground-glass opacities and remaining 2 had ground-glass opacities associated with reticular lesions. Respiratory symptoms were reported in 25 patients (52%) and included: cough (11), dyspnea (4), decreased physical endurance (3), fatigue (9) and fever (14). The physical examination revealed abnormalities in 3 patients (6.3%) and included: bilateral basal crackles (1) and wheezes and/or rhonchi (2). Pulmonary manifestations occurred in 27 patients (56.3%). 6 of them (22%) had ILD and 11 (40.7%) non-specific interstitial lung lesions. Among patients with ILD, 6 (54.5%) had the following pulmonary manifestations: cough (3), fatigue (2), fever (3) and bilateral basal crackles (1). 11 patients with non-specific interstitial lung lesions (55%) reported the following respiratory symptoms: cough (6), dyspnea (11), decreased physical endurance (3), fatigue (8) and fever (7). PFT showed restrictive process in 1 patient (2.1%) with non-specific interstitial lung lesions on HRCT of the lung. The obstructive lung disease occurred in 1 patient (1.8%) with normal chest HRCT. There were significant differences in time from the first RA symptoms to the first rheumatological assessment (9 vs 3.6 months, p- 0.008), time from the first symptoms to RA diagnosis (9.1 vs 4.6 months, p- 0.02) and time from the first symptoms to treatment (8.9 vs 4.3, p- 0.02) between patients with and without ILD. There were no significant differences between these two groups in terms of age, gender, RA activity (ESR, CRP, SJC, TJC, DAS28, SDAI, CDAI), presence and level of RF and anti-citrulinated peptide, presence of pulmonary manifestations and results of PFT. Conclusions Our study disclosed a great percentage of interstitial lung lesions in patients with early RA. Delay in RA diagnosis and treatment appears to be associated with increased risk of ILD in patients with RA. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4221

A 50-year-old man presented with dyspnoea while sitting, standing and walking but resolved completely in supine position. On cardiorespiratory examinations, fine crackles were noted over bibasal area. Chest X-ray showed bilateral reticulonodular shadows, restrictive pattern on spirometry, elevated alveolar arterial O2 gradient on arterial blood gas. High-resolution CT of the thorax revealed pattern as ‘confident’ or ‘certain’ radiographic diagnosis of idiopathic pulmonary fibrosis (IPF). Bubble-contrast echocardiography in recumbent, sitting and upright positions revealed no intracardiac (right to left shunt) or intrapulmonary shunts. This case highlights the necessity of awareness of this syndrome in cases of interstitial lung diseases (ILDs) also. Although 188 cases have been described thus far of platypnoea-orthodeoxia syndrome (P-OS) of various aetiologies, to the best of our knowledge, it is the first ever case of P-OS in ILD/IPF. Both lung bases were predominantly affected in this patient, platypnoea and orthodeoxia were attributed to areas of low/zero ventilation/perfusion (V/Q) ratio (zone 1 phenomena) as no other obvious explanation was found.

Radical new ideas are needed to ensure that deaths from respiratory diseases in children continue to fall The invitation to comment on a paper that reports changes which occurred during a period that coincided, within a year or two, with my career as a consultant paediatrician presents me with an irresistible temptation to reminisce. At medical school in the late 1950s I learned that pneumonia, one time captain of the men of death, had responded dramatically first to sulphonamides and then to penicillin, but that some deaths were still “inevitable”—a phrase much used at that time to excuse our inability to manage conditions that we did not fully understand. Asthma was common in children but was considered to be an unusual cause of death, although it was responsible for the first death I encountered as a paediatric senior house officer. In the minds of my teachers asthma was readily distinguished from bronchitis and, to this day, I can replicate a table listing the differences between asthma and wheezy bronchitis, most of which has—to my surprise—turned out to be accurate.1 In those far off days most children with cystic fibrosis (CF) died in the pre-school years; Pseudomonas species were not the problem they are now and most children died while still colonised with Staphylococcus aureus or Haemophilus influenzae . Bronchiolitis was considered to be rather an esoteric diagnosis for which there was no diagnostic test – it would be a few years before its association with the respiratory syncytial virus (RSV) was appreciated.2 It was thought safer to diagnose pneumonia if crackles were prominent and to treat the child with antibiotics, while those with predominant wheeze were labelled “bronchitis” and given ephedrine. In the mid 1960s I extracted data from the Registrar General’s annual reports when, as a junior, …

A 27-year-old woman was referred from her general practitioner (GP) 6 weeks postpartum with weight loss, dyspnoea and bilateral leg swelling. She had been treated by the GP for a chest infection the week before. On admission she was tachycardic, hypotensive and hypoxic with bilateral leg oedema and bibasal crackles. A chest radiograph showed diffuse multinodular opacification. A CT pulmonary angiogram showed dilated pulmonary artery. Echocardiogram confirmed right ventricular strain and dilation. Sputum microscopy was positive for acid-fast bacilli. The patient developed confusion and low-grade fever and the CT head scan showed meningeal involvement. One year of antituberculous therapy was started and she made a clinical recovery after 10 days and was discharged with regular follow-up. An echocardiogram showed resolution of the right heart strain 4 months later.

A 70-year-old man, a case of diabetes mellitus with nephropathy, presented with malaise, poor appetite and progressive shortness of breath for 1 week. Physical examination showed crackles at bilateral lung fields and no oedema of limbs. His blood urea nitrogen (BUN) and serum creatine levels were 245 and 33 mg/dL, respectively. Anaemia (haemoglobin 6.4 g/dL) and hypoalbuminaemia (albumin 2.6 g/dL) were also noticed. The ECG showed a sinus rhythm without significant ST-T wave changes. Chest radiography showed bilateral …

An adult male labourer, a smoker and alcoholic was admitted to our hospital with a short history of fever, myalgia, breathlessness and oliguria. On examination he was icteric and hypotensive. Calf muscle tenderness was present. A provisional diagnosis of leptospirosis was made and he was started on treatment with crystalline penicillin. Blood pressure (BP) did not improve with fluids. Inotropes were started. The patient was taken for Slow Low Efficiency Daily Dialysis (SLEDD) during which he developed chest pain. ECG showed an anterolateral myocardial infarction (MI). He also complained of breathlessness and haemoptysis. Antiplatelets were withheld in view of thrombocytopaenia and haemoptysis; heparin could not be given because of the deranged coagulation parameters. The patient was managed symptomatically with nitrates. After the BP improved SLEDD was restarted. On day 3 of admission the patient became tachypnoeic and hypoxic, bilateral coarse crackles were present on auscultation. He was intubated and mechanically ventilated. Suctioning of endotracheal tube revealed fresh blood, and chest CT revealed alveolar haemorrhage. In spite of aggressive resuscitative measures, mechanical ventilation and antibiotics, the patient expired on the 12th day following admission.

Background and Aims Chickenpox is an acute, benign, highly contagious disease characterized by generalized vesicular exanthema with self-limited evolution. Pneumonia is the most serious complication of varicella, occurring more frequently in adults (>20%) than in children. An outbreak of the disease started in late autumn 2011 in Romania and continues in present. Methods and results: The authors present the case of a 4 years old boy admitted into the Infectious Diseases Hospital with chickenpox. On the 4-th day of the disease high fever, dyspnea with tachypnea, intercostal retractions, pleuritic pain and cough appeared and the patient was transferred to the Children Hospital. Clinical examination showed abolished left basal vesicular breath sound with wet crackles in the middle and superior lung area; chest X-ray finded inferior left lobe pneumonia and mild pleural effusion. Tracheal aspirate culture was negative. Leucokytosis with neutrophilia and increased ESR and C-reactive protein was founded. Broad spectrum antibiotherapy was started with favourable evolution after 3 weeks. Conclusions Among the most serious complications of varicella is pneumonia; it is less common in children than in adults but it may lead to death. However, the epidemic status in Romania in 2011–2012 was associated with an increased number of viral pneumonias; in our case the radiological aspect was highly suggestive for a secondary bacterial infection even with negative aspirate culture (explained by prior antibiotherapy). The history for chickenpox vaccine was negative in our patient. In Romania, the chickenpox immunization is not included into the National Programe of Immunisations at this moment.

From 1 October 2009 to 1 December 2010, we conducted a prospective hospital-based study at KFSHRC to evaluate the role of Bocavirus (HBoV) infections in hospitalized children with chronic medical or immunocompromising conditions. Clinical and epidemiological data were recorded and respiratory samples including nasopharyngeal aspirate or nasopharyngeal swabs were obtained from all children less than 14 years old with acute respiratory tract infections. HBoV was screened in all respiratory samples by real time PCR, in addition to 13 common respiratory viruses. During the study, HBoV was detected in respiratory samples from 25 (2%) of 1016 symptomatic patient. HBoV co-existence with other respiratory pathogens occurred in 72% (18/25) of respiratory samples from symptomatic patients. HBoV infections were detected in every month except June and July with peaks in the month of September, October, November, and December. The main diagnosis in 13 patients (52%) with HBoV was radiologically confirmed pneumonia. For the other 12 patients with HBoV infections the main diagnosis were gastroenteritis(4 cases), chest exacerbation (3 cases), upper respiratory tract infections (2 cases), persistent fever (1 case), seizure (1 case), otitis media (1 case). The main clinical signs and symptoms of HBoV positive patients included fever, cough tachypnea, dyspnea, crackles, wheezing, abdominal pain, vomiting and diarrhea. The present study suggest that HBoV may be a fairly common cause of pneumonia in high-risk children hospitalized with acute respiratory infections and associated with morbidity. However, further study is needed to clarify if HBoV plays a pathogenic role in community acquired pneumonia in high-risk children.

A 73 year old woman, Mrs K, presented to her general practitioner with a one month history of increasing breathlessness and fatigue. She had no other symptoms and no medical history. Mrs K was a non-smoker and was not taking any drugs. On examination, Mrs K’s respiratory rate was 14 breaths per minute and her heart rate was 64 beats per minute. Her blood pressure was 125/73 mm Hg and she was clinically euvolaemic. She was found to have a new systolic murmur, loudest at the apex, with bibasal fine crackles. Her elecrocardiogram showed sinus rhythm. Her C reactive protein level was raised at 12 mg/L (

A 60 year old woman presented to the chest clinic with four years of worsening dyspnoea. On waking she felt unrefreshed and had a headache. She felt sleepy throughout the day. Her medical history included kyphoscoliosis, spina bifida, hypertension, and osteoporosis. The drugs she was taking included amiloride, furosemide, nitrazepam, and calcium-vitamin D supplements. She was born in the United Kingdom, was a lifelong non-smoker, and a retired teacher. She owned no pets and reported no exposure to asbestos. On physical examination, her respiratory rate was 28 breaths/min and oxygen saturation was 86% on ambient air. Temperature and haemodynamics were normal. General inspection showed marked kyphosis and rapid shallow breathing. She had a global reduction in breath sounds but no crackles, wheeze, or rub. The rest of the examination was unremarkable, with no evidence of right heart failure. Blood tests showed a raised haemoglobin of 157 g/L (reference range 115-165), but other haematological, biochemical, and inflammatory markers were normal. Arterial blood sampling on ambient air showed pH 7.36, arterial carbon dioxide tension 9.14 kPa, arterial oxygen tension 6.4 kPa, base excess 7.8, and bicarbonate 33 mmol/L. Her body mass index was within the normal range. Figure 1⇓ shows the chest radiograph. ### 1 What is the primary abnormality on the chest radiograph? #### Short answer The primary abnormality of the chest radiograph is severe kyphoscoliosis with profoundly reduced lung volumes. #### Long answer This radiograph shows kyphoscoliosis with predominantly left lateral mediastinal displacement. Lung volumes are profoundly reduced, and this is more marked on the left. The left hemidiaphragm is raised with the gastric bubble …

Aims To assess if clinical factors predict admission for bronchiolitis. Methods We conducted a prospective cohort study, recruiting babies less than 12 months old who presented to the Emergency Department (ED) with bronchiolitis (diagnosed clinically by ED doctors). Infants9 clinical signs and symptom were recorded on presentation. These were analysed to determine those factors which predicted admission, both singly and by using multiple logistic regression. Results 86 infants were recruited to the study. The mean age (±SE) was 22±1.5 weeks and 40% of infants were female. Five risk factors (shown in Tables - age, gender, heart rate, respiratory rate and oxygen saturation) predict admission in 88.4% of our cohort. In the table (table 1), odds ratios for continuous variables reflect impact per unit change. Factors which did not predict admission included wheeze, crackles, absence of wheeze and crackles, increased work of breathing, poor feeding and apnoea. Conclusion This supports but develops further the previous observations of Walsh et al 1 that clinical factors can be used to predict admission in bronchiolitis; however our study identifies 5 key factors rather than the 12 that were previously identified. Factors were identified which did not predict admission although some of these may reflect the sample size. These factors can be used to develop algorithms to guide general practitioners to determine which babies need referral to secondary care, ED doctors to determine who requires hospital admission and help reduce unnecessary attendances at the ED. Further work needs to be done to develop appropriate tools and to assess their utility prospectively.

Aims To highlight a rare respiratory condition, its investigation and management. Methods A 12 year old girl referred by GP to her local DGH with a history of worsening exertional and nocturnal dyspnoea, and weight loss subsequent to a flu like illness one year previously. Past medical history was unremarkable. Her exercise tolerance was severely restricted due to cough and shortness of breath. She had shown no response to either bronchodilators or inhaled steroids. She had been treated with 3 courses of oral antibiotics for presumed chest infections without improvement. Results She was dyspnoeic at rest, hypoxic in room air (85%), which improved to 93% in 1 litre of oxygen. She was thin but not clubbed. She had marked pectus excavatum. She had markedly reduced air entry and coarse crackles bilaterally. Her chest X-ray showed widespread interstitial shadowing. She had normal routine bloods, blood gas, and ECG. Her FEV1 was 30% predicted with a restrictive flow-volume loop. Her differential diagnoses included pneumocystis pneumonia, drug reaction, interstitial lung disease, and alveolar proteinosis. She was referred to her regional respiratory centre. Further investigations of note were: ▶Pneumocyctis PCR: Negative ▶Bronchoscopy: Normal airway anatomy. Creamy BAL fluid. ▶Immune function: Normal ▶CT Chest: widespread alveolar and interstitial change, with ‘crazy paving’ ▶Echo: Normal ▶Quantiferon & AFB staining: Negative ▶BAL showed abnormal cytology, with amorphous proteinaceous debris. Strongly PAS stain positive, diagnostic for Pulmonary Alveolar Proteinosis (PAP). She also grew haemophilus resistant to amoxicillin, commenced on co-amoxiclav. Conclusion PAP can present in the neonatal period, due to surfactant protein B deficiency, and is usually fatal. Later onset disease can be related to disturbance of granulocyte-macrophage colony stimulating factor (GM-CSF), and is diagnosed on BAL without the need for lung biopsy. CT appearances are suggestive. She was referred to the Royal Brompton for bilateral whole lung lavage. Ongoing care issues include correction of hypoxaemia, improving nutritional status, repeat lavage treatment and consideration of inhaled GMCSF. This condition has a variable course. Some patients will have spontaneous remission, some will have their disease controlled with multiple lavage treatments and some have disease with progression to respiratory failure.

Aims To assess whether auscultation findings at presentation with bronchiolitis vary with age or viral aetiology. Methods We conducted an opportunistic prospective cohort study of infants who presented to the Emergency Department (ED) with bronchiolitis (diagnosed by ED doctors). Chest auscultation findings in all children were assessed and recorded prospectively at presentation. Viral PCR was carried out on nasopharyngeal aspirates when requested by clinician. Chest auscultation findings were correlated with age and viral aetiology. Our outcome measures were variation of auscultatory signs with age and viral aetiology Results 86 children were recruited to the study. The mean age (±SE) was 22±1.5 weeks and 40% were female. Infants who presented with wheeze were significantly older ((mean ±SE) 26.6±1.9 weeks) than babies who presented without wheeze (17.3±2.1 weeks) (ANOVA, p=0.002). Those who presented without normal auscultation findings were younger than those with wheeze or crackles or both (15.1±2.6 weeks vs 24.4±1.7 weeks) (ANOVA, p=0.006). There was no difference in auscultation findings depending on the virus responsible for bronchiolitis. In 68% of cases (n=34) bronchiolitis was caused by RSV, 32% (n=16) by rhinovirus, 10% (n=5) by adenovirus. Conclusion Chest auscultation findings associated with bronchiolitis vary according to age, with infants older than 24 weeks likely to present with wheeze and those younger than 16 weeks more likely to present without auscultatory chest signs. This is a crucial finding as it has been previously reported that crackles are a consistent feature of bronchiolitis1. Differences in chest signs may reflect differences in pathomechanisms of bronchiolitis and may explain why there are variations in response to medical treatment reported in the literature. Clinicians should be especially vigilant with young infants (

A 42-year-old woman with a history of hypothyroidism and Addison’s disease presented with acute onset of breathlessness, chest pain and dry cough. The chest pain was pleuritic in nature and radiated into the back. On physical examination, she was hypotensive with blood pressure of 87/54 mm Hg. Her pulse rate was 95/min with normal jugular venous pressure. Respiratory system examination revealed mild tachypnoea (RR 20) without any crackles or wheezes. A chest radiograph was unremarkable. Oxygen saturations were 98% breathing 24% oxygen and arterial blood gas analysis showed pH 7.36, pO2 13.5 kPa, pCO2 4.4 kPa and HCO3 20.3 mmol/l. The white cell count was 12.3×109/l and C reactive protein 44 mg/l (normal range 0–8). Biochemical profile showed hyponatraemia and hyperkalaemia, consistent with adrenal insufficiency. An echocardiogram was unremarkable. …

Objectives The hazard of inhaling non-asbestiform elongated mineral particles is still inconclusive. The objective was to assess the risk of lung fibrosis in workers processing ores containing quartz and non-asbestiform minerals. Methods A retrospective cohort study was designed. A study cohort of 344 stone workers and their family in Hualien was established in 2010. By health examination and questionnaire, we obtained lifetime occupational history, duration and frequency of exposure to 45 common used ores, smoking and medical history. Survival analysis was applied. The follow-up started from the year of beginning their work at stone and ended in the survey of 2010. At the end of follow-up, the development of pulmonary fibrosis profusion 1/1 or greater with lung crackle was regarded as an event, and subjects without the event were treated as right censored. Results After excluding 12 subjects with history of tuberculosis and occupational history of metal casting and welding, 328 subjects were enrolled for analysis. The mean follow-up year was 25.0 and 8137 person-years were accrued. 17 subjects had fibrosis profusion 1/1 or greater combined with crackles. After adjusting the pack-years of smoking and age, processing nephrite (RR=3.06, p=0.03) and chalcedony (RR=3.42, p=0.03) had higher risk compared with reference group (non-exposure family). Log-rank test found that processing nephrite (p=0.01) and chalcedony (p=0.02) had increased risk. The hazard ratio adjusted for age, sex and pack-year was 3.0 (p=0.04) for processing nephrite and 4.26 (p=0.01) for processing chalcedony. Conclusions Processing ores containing quartz and non-asbestiform minerals might cause lung fibrosis.

Our patient was a 62-year-old retired accountant of previous good health. He was not taking any medications and had no family history of neurological disease. He was involved in a minor road traffic accident in which his car was shunted from behind. No immediate injuries were sustained; however, he presented to his general practitioner 4 days later with lumbar spine discomfort. He was prescribed a codeine–paracetamol combination pill and diclofenac. Immediately after taking the first dose of these medications he developed severe dizziness and marked vomiting, which culminated in mild haematemesis, indicative of a Mallory–Weiss tear. Subsequently he discovered that he was dyspnoeic lying flat. He presented to his local emergency department with symptoms including marked orthopnoea, and dyspnoea on water immersion past his costal margin and on bending forward. Physical examination showed gross paradoxical abdominal motion and mild breathlessness when recumbent. Respiratory system, some left basal crackles; cardiovascular system, normal; abdomen, normal; neurology, normal. Arterial blood gas measurements showed a Pao2 of 11.1 kPa and a Paco2 of 5.1 kPa. A chest radiograph showed an elevated left hemidiaphragm with some atelectasis above it. A CT pulmonary angiogram excluded any diaphragmatic rupture or pulmonary embolus as an underlying cause of orthopnoea. His sniff nasal inspiratory pressure (SNIP) was measured at 22 cm H2O. Detailed testing showed no response to bilateral anterior magnetic phrenic nerve stimulation or to right unilateral phrenic nerve stimulation, with a greatly reduced response to left-sided stimulation (twitch transdiaphragmatic pressure 1.3 cm H2O; normal >8 cm H2O). Pulmonary function tests (performed in …

A 13 week old boy presented to accident and emergency with fever, breathing difficulties, and reduced feeding. On examination, his respiratory rate was 60 breaths/min, pulse was 180 beats/min, oxygen saturation was 91% in air, and temperature was 37.8oC. He had intercostal and subcostal recession and a tracheal tug. Auscultation of the chest showed widespread wheeze and crackles and heart sounds were normal. A chest radiograph showed an obscured right heart border and generalised hyperinflation. Blood tests showed a white cell count of 22.0×109/l, and a nasopharyngeal aspirate was positive for respiratory syncytial virus on polymerase chain reaction testing. The boy was born at term by normal delivery with a birth weight of 4100 g. The antenatal course was uneventful. He was kept in hospital for two days after delivery because he was “mucousy.” His parents described him as a noisy and “snuffly” breather since birth, with frequent coughing and “choking” episodes. Because of this he had been seen by his general practitioner and in casualty several times, but he had not been admitted to hospital before. He had been breast fed and was thriving well. A few hours after admission he suddenly deteriorated after a feed. His pulse rose to 230 beats/min and respiratory rate to 80 breaths/min. Capillary gas showed pH 7.03, partial pressure of carbon dioxide 12.18 kPa, bicarbonate 17 mmol/l, and base excess −7.4. He was admitted to paediatric intensive care and was intubated and ventilated. He remained intubated for 10 days and two attempts to extubate failed because he developed stridor. Once extubated successfully, we performed further investigations to check for any underlying abnormality. The following radiological image was obtained (fig 1⇓).

Pneumorrhachis (air within the spinal canal) is rare and usually associated with other air leaks. Diagnosis is usually by high resolution CT scan. A 9-year-old boy had a 10-day history of persistent cough despite treatment with inhaled bronchodilators, systemic steroids and antibiotics before coming to hospital. Physical examination demonstrated good general condition, cervical subcutaneous emphysema and reduced breath sounds with widespread crackles and …

A 44 year old man was brought to the emergency department with a two day history of fever, skin rash, and a rapidly progressive shortness of breath. On examination, notable observations included an extensive rash, cyanosis, a fast respiratory rate (40 breaths/min), bilateral crackles on lung auscultation, and a temperature that reached a maximum of 40.4°C. Arterial blood gas levels showed severe hypoxemia (PaO2, 48 mmHg; fraction of inspired oxygen [FiO2], 21%). Chest radiography showed bilateral consolidation of the air spaces, mainly in the lower lung fields, which confirmed the clinically suspected diagnosis of community acquired pneumonia (fig 1).⇓ Fig 1 Skin rash in a patient with community acquired pneumonia ### Short answers ### Long answers #### 1 Diagnosis of the skin rash The term “erythema multiforme” is used to describe a special category of maculopapular rash that encompasses a wide range of different morphologic types (including plaques, vesicles, and bullae), which can occur concurrently. Rashes of this nature occur as a reaction to an infection or to medication (box 1);1 …