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Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children that primarily involves the skeletal muscles and skin. However, it can manifest with a diverse range of clinical features across multiple organ systems. JDM vasculopathy causing optic neuritis and medullary bone infarcts in children are rare manifestations of JDM and have been seldom reported in literature. Similarly, cardiovascular manifestations in the form of refractory myocarditis leading to congestive cardiac failure during the early course of the disease have also been rare findings. Early diagnosis and aggressive management of such complications using biological disease-modifying synthetic antirheumatic drugs has significantly reduced the mortality and morbidity associated with JDM. Herein, we report a mini case series of three patients with JDM who presented with these rare systemic manifestations and achieved favorable outcomes while being managed using the Childhood Arthritis and Research Alliance-Treatment Protocols.

Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children that primarily involves the skeletal muscles and skin. However, it can manifest with a diverse range of clinical features across multiple organ systems. JDM vasculopathy causing optic neuritis and medullary bone infarcts in children are rare manifestations of JDM and have been seldom reported in literature. Similarly, cardiovascular manifestations in the form of refractory myocarditis leading to congestive cardiac failure during the early course of the disease have also been rare findings. Early diagnosis and aggressive management of such complications using biological disease-modifying synthetic antirheumatic drugs has significantly reduced the mortality and morbidity associated with JDM. Herein, we report a mini case series of three patients with JDM who presented with these rare systemic manifestations and achieved favorable outcomes while being managed using the Childhood Arthritis and Research Alliance-Treatment Protocols. [ABSTRACT FROM AUTHOR]

Purpose: Bronchiolitis obliterans with pneumonic organization, or organizing pneumonia (OP), is an inflammatory disorder of the lungs, which can be triggered following pulmonary attacks of infectious or non-infectious origin. The non-infectious origins of OP include various entities including connective tissue diseases, exposure to toxic substances, medications, autoimmune diseases, and thoracic radiotherapy. The objective of this article is to summarize the literature on post-radiotherapy organized pneumonia, its etiologies, its clinical and radiological characteristics, as well as its treatment., Materials and Methods: A systematic review was performed in Medline database using the search engine PubMed. Keywords for the search included cryptogenic organizing pneumonia, bronchiolitis obliterans organizing pneumonia (BOOP), idiopathic organizing pneumonia and radiation, radiotherapy, breast cancer. The selected articles had to study the link between bronchiolitis obliterans with pneumonic organization and radiotherapy., Results: A total of 96 articles were identified. Of these 96 articles, 49 fulfilled the defined selection criteria. Fourteen epidemiological studies were found in the literature. These epidemiological studies have published incidences of post-radiotherapy organizing pneumonia of less than 2.9% for patients treated for breast cancer. The predictive risk factors for bronchiolitis obliterans with pneumonic organization syndrome were age, smoking and the volume of irradiated lung. In a post-radiation context, bronchiolitis obliterans with pneumonic organization could be diagnosed several months, or even up to a year, after the end of irradiation. Treatment was based on the prescription of long-term corticosteroid therapy. Bronchiolitis obliterans with pneumonic organization should not be confused with post-radiation pulmonary fibrosis, which is inflammatory, dose-dependent, non-immunological, and localized in the irradiation area., Conclusion: Organized pneumonia secondary to radiotherapy is a syndrome affecting approximately 1.4 to 3% of patients treated with radiotherapy for breast cancer. The main risk factors found are age, smoking and the volume of lung irradiated. Post-radiotherapy organized pneumonia needs to be known to all radiotherapists to improve patient care., (Copyright © 2024. Published by Elsevier Masson SAS.)

Background This study analyzed the incidence, characteristics, and mortality risk associated with cryptogenic organizing pneumonia (COP) among hospitalizations for systemic lupus erythematosus (SLE) with lung involvement. Methods Adult hospitalizations from the 2016-2020 nationwide inpatient sample were analyzed using relevant International Classification of Diseases (ICD)-10 codes for SLE with lung involvement (M32.13) and COP (J84.116). We compared baseline characteristics of individuals with SLE and COP to those of other lung involvements using Chi-square tests for categorical variables and the Wilcoxon rank sum test for continuous variables. A Cox proportional hazards model was used to assess the risk of developing COP in the pooled cohort of SLE patients. The impact of COP on SLE mortality was assessed using multivariate logistic regression adjusting for illness severity, baseline risk of mortality at admission, and patient- and hospital-level covariates. Results Of 40,356 admissions for SLE, 3,175 (7.9%) were due to lung involvement, with COP identified in 570 cases (17.9%). Compared with other lung involvement in SLE, individuals with COP were significantly older (mean age: 65 vs. 44.3 years; p<0.001), mostly female (515; 90.4% vs. 2,305 males; 88.5%; p=0.572), had a greater baseline risk of mortality [diagnosis-related groups (DRG) major or extreme likelihood of dying: 360; 63.1% vs. 1,133; 43.5%; p<0.001], and had a higher prevalence of peripheral vascular disease (25; 4.4% vs. 39; 1.5%; p<0.001), and lower prevalence of lymphocytopenia (45; 7.9% vs. 359; 13.8%; p=0.001), and hypothyroidism (44; 7.8% vs. 357; 13.7%; p=0.001). Predictors of COP included female sex [adjusted hazard ratio (AHR): 1.46; 95% confidence interval (CI): 1.12-2.96; p=0.022]; hospitalizations occurring in the third quarter of the year (AHR: 1.37; 95% CI: 1.05-2.23; p=0.038); hospital stays of six days or longer (AHR: 1.71; 95% CI: 1.06-2.77; p=0.029); undergoing five or more procedures during the same hospitalization (AHR: 1.56; 95% CI: 1.26-3.56; p=0.041); coexisting lymphocytopenia (AHR: 1.92; 95% CI: 1.16-3.19; p=0.011); need for mechanical ventilation (AHR: 1.60; 95% CI: 1.48-3.93; p=0.049), presence of another autoimmune disorder (AHR: 1.37; 95% CI: 1.15-4.29; p=0.040), and being hospitalized at private, investor-owned hospitals (AHR: 2.62; 95% CI: 1.03-6.64; p=0.043). Mortality in SLE with lung involvement was correlated with age ≥ 60 years [hazard ratio (HR) (95% CI) 1.16 (1.05-1.56); p=0.012], coexisting lupus nephritis [HR (95% CI), 2.44 (2.04-3.49); p=0.031], cancer [HR (95% CI), 3.49 (2.19-5.79); p<0.001], liver disease [HR (95% CI), 9.82 (4.79-12.57); p<0.001]; immune deficiency [HR (95% CI), 2.22 (2.02-3.11); p=0.031], hypothyroidism [HR (95% CI), 4.67 (1.47-7.75); p=0.009], and high blood pressure [HR (95% CI), 3.15 (2.83-4.51); p<0.001]. In the multivariable analysis, COP remained significantly associated with an increased risk of mortality [AHR (95% CI), 1.43 (1.16-2.74); p=0.031]. The incidence of COP did not significantly impact hospitalization costs ($US 94,772 ± 14,759 vs. 95,982 ± 32,625; p=0.954) or length of stay (mean length of hospital stay: 8.3 vs.6.8 days; p=0.147). Conclusion Cryptogenic organizing pneumonia was associated with 1% of all hospitalizations for SLE and 18% of cases involving lung complications in SLE. The presence of COP significantly increased the risk of mortality in SLE patients with lung involvement., Competing Interests: Human subjects: All authors have confirmed that this study did not involve human participants or tissue. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Uwumiro et al.)

Introduction: Organizing pneumonia (OP) is an idiopathic interstitial pneumonia characterized radiologically by the patchy peripheral areas of ground-glass opacities and consolidation. It is commonly associated with a variety of conditions such as connective tissue diseases (CTD), drugs, infections, malignancy, radiation exposure, post-transplant, and other interstitial pneumonia. There are no specific clinical manifestations unless there is an underlying etiology. We present a series of such cases. Aims and Objectives: The aim of the study was to identify the clinical characteristics and etiological spectrum of patients manifesting radiologically with OP pattern. Materials and Methods: This was a retrospective analysis of clinico-radiological profile and etiological diagnosis of 23 patients, who had a radiological diagnosis of OP during the period of January 2017–September 2019. Results: Our patients presented with nonspecific symptoms of cough, fever, breathlessness, and occasionally with hemoptysis. The various etiologies identified were CTD (n = 4), infection (n = 2), drugs (n = 4), radiation (n = 1), chronic aspiration syndrome (n = 1), malignancy (n = 2), hypersensitivity pneumonitis (n = 1), and chronic heart failure (n = 2), and in majority (n = 7), no underlying etiology was evident and were labeled as cryptogenic organizing pneumonia. Conclusion: OP is an underdiagnosed entity and is associated with numerous diseases varying from pulmonary tuberculosis to malignancy. Identification of the underlying disease process is of paramount importance as it enables the treating physician to implement necessary therapeutic interventions.

Atypical presentations of cryptococcal infection have been described as manifestations of immune reconstitution inflammatory syndrome (IRIS) in patients with human immunodeficiency virus (HIV) infection following the introduction of combination antiretroviral therapy (cART). We describe a patient presenting with cryptococcal bronchiolitis obliterans organizing pneumonia (BOOP) as a cryptococcal-IRIS (C-IRIS), eight weeks after cART initiation. To our knowledge, this is the first patient with HIV disease reported to have cryptococcal IRIS-related BOOP.

Atypical presentations of cryptococcal infection have been described as manifestations of immune reconstitution inflammatory syndrome (IRIS) in patients with human immunodeficiency virus (HIV) infection following the introduction of combination antiretroviral therapy (cART). We describe a patient presenting with cryptococcal bronchiolitis obliterans organizing pneumonia (BOOP) as a cryptococcal-IRIS (C-IRIS), eight weeks after cART initiation. To our knowledge, this is the first patient with HIV disease reported to have cryptococcal IRIS-related BOOP. [ABSTRACT FROM AUTHOR]

Introduction: Organizing pneumonia (OP) is an idiopathic interstitial pneumonia characterized radiologically by the patchy peripheral areas of ground-glass opacities and consolidation. It is commonly associated with a variety of conditions such as connective tissue diseases (CTD), drugs, infections, malignancy, radiation exposure, post-transplant, and other interstitial pneumonia. There are no specific clinical manifestations unless there is an underlying etiology. We present a series of such cases. Aims and Objectives: The aim of the study was to identify the clinical characteristics and etiological spectrum of patients manifesting radiologically with OP pattern. Materials and Methods: This was a retrospective analysis of clinico-radiological profile and etiological diagnosis of 23 patients, who had a radiological diagnosis of OP during the period of January 2017-September 2019. Results: Our patients presented with nonspecific symptoms of cough, fever, breathlessness, and occasionally with hemoptysis. The various etiologies identified were CTD (n = 4), infection (n = 2), drugs (n = 4), radiation (n = 1), chronic aspiration syndrome (n = 1), malignancy (n = 2), hypersensitivity pneumonitis (n = 1), and chronic heart failure (n = 2), and in majority (n = 7), no underlying etiology was evident and were labeled as cryptogenic organizing pneumonia. Conclusion: OP is an underdiagnosed entity and is associated with numerous diseases varying from pulmonary tuberculosis to malignancy. Identification of the underlying disease process is of paramount importance as it enables the treating physician to implement necessary therapeutic interventions. [ABSTRACT FROM AUTHOR]

Objective: Bronchiolitis obliterans organizing pneumonia (BOOP) is a clinico-patho-radiological diagnosis which rarely presents as a pulmonary manifestation of lupus. In this concise report, organizing pneumonia was found as the sole pulmonary manifestation of SLE in different age groups. Method: All three patients diagnosed with SLE according to SLICC 2012 classification criteria, were admitted in rheumatology ward of NIMS hospital, Hyderabad, India from May to November, 2018. Their diagnosis of BOOP was either biopsy proven or imaging guided. Review of literature was done with MeSH terms (SLE, BOOP) in PubMed and approximately 10 articles were reviewed including latest of 2019 published in Scientific Reports. Result: There were three patients – one juvenile lupus and two adults. Two patients were male and one female. All three patients had SLE with high disease activity. They all had organising pneumonia as pulmonary manifestation with other organ involvement. Juvenile patient had a fatal outcome while the others had a good recovery with steroid and immunosuppressive. Conclusion: BOOP is a rare pulmonary manifestation in lupus. It can be diagnosed early with more precision using computerised tomography of lung without waiting for biopsy report. This will result in a better prognosis by rapid initiation of corticosteroid and immunosuppressive treatment. [ABSTRACT FROM AUTHOR]

Bronchiolitis obliterans organizing pneumonia is an interstitial lung disease rarely occurring after radiotherapy probably due to an activation of autoimmune processes. Most cases have been described after postoperative radiotherapy for breast cancer. Corticosteroids represent the main treatment, prognosis is generally favorable. We described a case of bronchiolitis obliterans organizing pneumonia after stereotactic ablative radiation therapy for a recurrent lung cancer. Antibiotics and steroids were administered to solve the clinical picture. After three years, a new lesion at the right lung was found and treated with stereotactic ablative radiation therapy and concomitant long course of steroids with no recurrence of bronchiolitis obliterans organizing pneumonia. Bronchiolitis obliterans organizing pneumonia is a rare event after radiotherapy with undefined risk factors. In our case, steroids played an important role in management and, maybe, in preventing bronchiolitis obliterans organizing pneumonia recurrence after second course of stereotactic ablative radiation therapy. [ABSTRACT FROM AUTHOR]

Several drugs have been implicated in the induction of systemic lupus erythematosus (SLE), but there are only some observations of carbamazepine induced SLE since the first case described in 1966, this drug has also been implicated in the induction of other disorders and rarely pulmonary toxicity; but the occurrence of two rare side effects of this drug: the induced SLE and pulmonary nodules consistent with the bronchiolitis obliterans organizing pneumonia in same patient is really unusual and -to our knowledge- the second observation reported in the literature.

Résumé Les complications pulmonaires pouvant être induites par la radiothérapie se composent essentiellement de la pneumopathie radique (aiguë ou subaiguë), de la fibrose pulmonaire, et de la bronchiolite oblitérante avec pneumopathie organisée (abrégée en « pneumopathie organisée » chez les francophones, « BOOP » chez les anglo-saxons). Cet article est une synthèse de la littérature portant sur ces pathologies, faisant une mise au point sur la démarche diagnostique, sur les connaissances physiopathologiques actuelles, et sur les moyens non dosimétriques de prévention et de traitement. Puisqu’aucun signe clinique ou paraclinique n’est pathognomonique de ces phénomènes, le diagnostic doit être porté par élimination. Les examens paracliniques absolument nécessaires sont la scanographie thoracique en coupes fines et les épreuves fonctionnelles respiratoires avec mesure de la diffusion libre du CO. Par ailleurs, aucun traitement n’a vraiment montré d’efficacité dans la prévention des complications pulmonaires radio-induites et dans le traitement de la fibrose. Les molécules les plus avancées et les plus prometteuses concernant la prévention des complications pulmonaires de la radiothérapie sont l’amifostine, les inhibiteurs de l’enzyme de conversion de l’angiotensine et la pentoxifylline. Des inhibiteurs de la synthèse du collagène sont en cours de développement préclinique, afin de limiter la fibrose radio-induite. Dans le cas du traitement de la pneumopathie radique, les corticoïdes tiennent une place centrale, sans qu’il n’existe un protocole standardisé. Des alternatives (immunosuppresseurs) existent, sur la foi de résultats issus de cas cliniques. Ces données mettent en lumière le rôle primordial de la protection dosimétrique des poumons dans la prévention de la toxicité pulmonaire radio-induite. Radiation-induced lung injuries mainly include the (acute or sub-acute) radiation pneumonitis, the lung fibrosis and the bronchiolitis obliterans organizing pneumonia (BOOP). The present review aims at describing the diagnostic process, the current physiopathological knowledge, and the available (non dosimetric) preventive and curative treatments. Radiation-induced lung injury is a diagnosis of exclusion, since clinical, radiological, or biological pathognomonic evidences do not exist. Investigations should necessarily include a thoracic high resolution CT-scan and lung function tests with a diffusing capacity of the lung for carbon monoxide. No treatment ever really showed efficacy to prevent acute radiation-induced lung injury, or to treat radiation-induced lung fibrosis. The most promising drugs in order to prevent radiation-induced lung injury are amifostine, angiotensin-converting-enzyme inhibitors and pentoxifylline. Inhibitors of collagen synthesis are currently tested at a pre-clinical stage to limit the radiation-induced lung fibrosis. Regarding available treatments of radiation-induced pneumonitis, corticoids can be considered the cornerstone. However, no standardized program or guidelines concerning the initial dose and the gradual tapering have been scientifically established. Alternative treatments can be prescribed, based on clinical cases reporting on the efficacy of immunosuppressive drugs. Such data highlight the major role of the lung dosimetric protection in order to efficiently prevent radiation-induced lung injury. [ABSTRACT FROM AUTHOR]

Radiation-induced organizing pneumonia (RIOP) is an inflammatory lung disease that is occasionally observed after irradiation to the breast. It is a type of secondary organizing pneumonia that is characterized by infiltrates outside the irradiated volume that are sometimes migratory. Corticosteroids work acutely, but relapse of pneumonia is often experienced. Management of RIOP should simply be symptom-oriented, and the use of corticosteroids should be limited to severe symptoms from the perspective not only of cost-effectiveness but also of cancer treatment. Once steroid therapy is started, it takes a long time to stop it due to frequent relapses. We review RIOP from the perspective of its diagnosis, epidemiology, molecular pathogenesis, and patient management. [ABSTRACT FROM AUTHOR]

Purpose/Aim of the Study: Adenosine signaling was studied in bronchiolitis obliterans organizing pneumonia (BOOP) resulting from unilateral lung ischemia.Materials and Methods: Ischemia was achieved by either left main pulmonary artery or complete hilar ligation. Sprague–Dawley (SD) rats, Dahl salt sensitive (SS) rats and SS mutant rat strains containing a mutation in the A2Badenosine receptor gene (Adora2b) were studied. Adenosine concentrations were measured in bronchoalveolar lavage (BAL) by HPLC. A2A(A2AAR) and A2Badenosine receptor (A2BAR) mRNA and protein were quantified.Results: Twenty-four hours after unilateral PA ligation, BAL adenosine concentrations from ischemic lungs were increased relative to contralateral lungs in SD rats. A2BAR mRNA and protein concentrations were increased after PA ligation while miR27a, a negatively regulating microRNA, was decreased in ischemic lungs. A2AAR mRNA and protein concentrations remained unchanged following ischemia. A2BAR protein was increased in PA ligated lungs of SS rats after 7 days, and 4 h after complete hilar ligation in SD rats. SS-Adora2b mutants showed a greater extent of BOOP relative to SS rats, and greater inflammatory changes.Conclusion: Increased A2BAR and adenosine following unilateral lung ischemia as well as more BOOP in A2BAR mutant rats implicate a protective role for A2BAR signaling in countering ischemic lung injury. [ABSTRACT FROM PUBLISHER]

Cryptogenic organizing pneumonia is a diffuse interstitial lung disease that starts in the alveolar wall and subsequently expands to the alveolar ducts and respiratory bronchioles. Randomized controlled trials are lacking to guide the treatment of cryptogenic organizing pneumonia, so treatment decisions and practice guidelines are often based upon observations from case series or expert clinical opinions. The backbone of treatment involves immunosuppression via corticosteroids. In refractory cases, cytotoxic therapy is considered. The evidence that supports the use of these regimens are limited. The goal of this scoping review is to conduct a systematic search of the literature to determine what regimens have been utilized to treat steroid refractory organizing pneumonia and to characterize the evidence supporting their use. [ABSTRACT FROM AUTHOR]

Cryptogenic organizing pneumonia (COP) is a distinct histopathologic entity characterized by Masson bodies which are intraalveolar buds of granulation tissue; consisting of connective tissue and myofibroblasts. This pathologic pattern when present with the characteristic imaging and clinical features of COP defines the diagnosis of the disease. A specific etiology is not found. Treatment with corticosteroids results in a rapid clinical and imaging improvement but unfortunately relapses are common upon decreasing the dose or stopping the treatment. This case report describes a typical case of COP with the characteristic improvement with corticosteroid therapy and relapse upon treatment taperKEY

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LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP). Combined homozygosity mapping and exome sequencing identified a homozygous LRBA mutation in this patient (p.Asp248Glufs*2). Such clinical and immunological findings have not been described to date and illustrate the broad and variable clinical phenotype of human LRBA deficiency. [ABSTRACT FROM AUTHOR]

Uma paciente 48 anos apresentou-se, assintomática, mas com achado radiológico de condensações alveolares, algumas constituindo nódulos em lobo superior direito, lobo médio e língula com predomínio perihilar. Foi considerado o diagnóstico diferencial de neoplasia, sarcoidose, doenças granulomatosas e tuberculose, entre outras doenças. O diagnóstico de bronquiolite obliterante com pneumonia em organização foi estabelecido através de videotoracoscopia e foi iniciado o tratamento com esteróides. Houve boa evolução com resolução radiológica.48 years-old woman, asymptomatic, presented with radiographic findings of patchy airspace consolidation with nodular opacities in the upper right lobe, middle lobe and lingula. The differential diagnoses of neoplasms, sarcoidosis, granulomatous diseases and tuberculosis were considered. Diagnosis of Bronchiolitis obliterans organizing pneumonia was made by means of videothoracoscopy whereupon treatment with corticosteroids was begun. The patient had a positive evolution with resolution of the radiographic finding.

Bronchiolitis obliterans organizing pneumonia (BOOP) is defined by buds of granulation tissue within lung distal airspaces. The diagnosis requires the histopathologic evidence of organizing pneumonia along with a suggestive clinical and radiographic pattern. This disorder is characterized by a good response to corticosteroids and an excellent prognosis. It can occur in association with a broad spectrum of clinical conditions or can be isolated, in this last case named cryptogenic organizing pneumonia. We searched for BOOP in patients with autoimmune rheumatic diseases (ARD) in the literature, and we found 32 well-documented cases. We reported here demographic features, manifestations, treatment and outcome of patients with BOOP associated with ARD. Notably, BOOP can be the presenting feature in some patients with ARD; thus, a close follow-up of patients with BOOP is recommended. [ABSTRACT FROM AUTHOR]

Background. Radiation therapy for breast cancer has been implicated in the development of bronchiolitis obliterans organizing pneumonia (BOOP).This inflammatory lung disorder was first noted in 1983, and there have been numerous reports ofBOOPoccurringinwomenwhohavehadradiationtherapyfor breast cancer since 1995.This studywas undertaken to perform a systematic reviewofpostradiotherapyBOOPtodetermine the occurrence, presentation, treatment, and outcome. Materials and Methods. A systematic literature review was conducted according to the guidelines provided by the Preferred ReportingItemsforSystematicReviewsandMeta-Analysesreport. Results. The literature search yielded 10 Japanese epidemiological reports with 129 women, 4 case series reports with 36 women, and 24 case reports with 34 women. Common symptoms included fever, cough, and shortness of breath. Most patients received corticosteroid therapy, and duration of treatment ranged from 6 months to 1 year, although some patients received steroids for longer than 1 year because of relapse, which occurred in approximately one half of patients. No deaths have been reported. Conclusion. BOOP is a rare but significant complication from radiation therapy for breast cancer. Chest radiographic studies for women who report new respiratory symptoms during the postradiation period can be beneficial for early diagnosis and for guiding appropriate management. [ABSTRACT FROM AUTHOR]

Las manifestaciones extraintestinales respiratorias en la enfermedad inflamatoria intestinal (EII) son excepcionales. Presentamos un caso de bronquiolitis obliterante con neumonía organizada (BONO) en paciente con enfermedad de Crohn, en remisión clínica sin tratamientos farmacológicos.Extraintestinal respiratory manifestations in inflammatory bowel disease (IBD) are rare. We present a case of bronchiolitis obliterans organizing pneumonia (BOOP) in a patient with Crohn's disease, with clinical remission with no drug therapy.

A male patient presented with respiratory distress for the last 2 months followed by gradual onset drowsiness and disorientation for the last 4-5 days. On investigation, he was found to be suffering from multiple myeloma (MM) with features of hypercalcemia, lytic bone lesions, renal compromise and anemia complicated by meningoencephalitis. He was also found to be inflicted with bronchiolitis obliterans organizing pneumonia (BOOP) as diagnosed by chest computerized tomography and transbronchial biopsy. Whether this association is sporadic or has some pathophysiological basis remains to be discovered, though recurrent pneumonias due to immunosuppression in MM may be a possible etiopathological factor. The patient was stabilized and discharged on request, but he presented again after 2 months with features of hypercalcemia in spite of getting a dose of zoledronate during the previous admission. This time he was given prednisolone and melphalan and was discharged in a stable condition after being counseled for follow-up visits. [ABSTRACT FROM AUTHOR]

Abstract: Background and objective: Bronchiolitis obliterans organizing pneumonia (BOOP) is a distinct clinicopathological entity histologically characterized by intra-alveolar granulation tissue and absence of extensive fibrotic lesions. Effective macrolide treatment of BOOP has been reported anecdotally. This study aimed to investigate whether alveolar macrophages (AMs) produce aberrant proinflammatory cytokines in BOOP and whether this can be inhibited by clarithromycin (CAM) or azithromycin (AZM). Methods: AMs collected by bronchoalveolar lavage (BAL) from 6 BOOP patients and 8 non-ILD controls were cultured for 24h in the presence or absence of CAM, AZM, lipopolysaccharide (LPS), or dexamethasone (DEX). Tumor necrosis factor alpha (TNF-α), soluble TNF receptor 1 (sTNFR1), sTNFR2, interleukin 1beta (IL-1β), IL-6, IL-8, IL-10, interferon gamma inducible protein 10 (IP-10) and CC chemokine ligand 18 (CCL18) were measured in the culture supernatant by ELISA. Results: The spontaneous and LPS-stimulated production of all investigated cytokines by AMs was significantly increased in BOOP compared to controls. CAM and AZM induced a dose-dependent suppression of spontaneous TNF-α, sTNFR2, IL-6, IL-8 and CCL18 production (p <0.05). CAM also inhibited the IL-1β production. CAM and AZM significantly and dose-dependently attenuated the LPS-stimulated production of sTNFR1, sTNFR2, IL-8 and CCL18 (p <0.05). CAM also inhibited the LPS-stimulated TNF-α, IL-1β, IL-6 and IL-10 production. Conclusions: AMs from BOOP patients produce abundant proinflammatory cytokines which may be pivotal in the disease pathogenesis. Macrolides inhibit this cytokine production, CAM more efficiently than AZM. [Copyright &y& Elsevier]

Summary: Background: Cryptogenic organizing pneumonia (COP) is a rare disease, and its diagnosis requires histological confirmation. The objective of our study was to describe the findings of the thoracic high-resolution computed tomography (HR-CT) and bronchoalveolar lavage (BAL) in patients with confirmed COP and evaluate the complementary diagnostic use of BAL and thoracic HR-CT. Methods: Patients recorded in the registry of interstitial pulmonary diseases between 1991 and 2008 were located and the COP patients selected. Results: We identified 21 patients with histological confirmation of COP. The median age was 58.0 ± 15.9 years, and 61.9% of patients were female. The most frequent thoracic HR-CT profile was patchy infiltrate (71.4%), followed by parenchymatous consolidation (42.9%). The most frequent BAL profile was mixed alveolitis (62%) with lymphocyte predominance, a CD4/CD8 index of 0.4 and foamy macrophages. The effectiveness of transbronchial biopsy was 66.6%. The diagnostic utility of Poletti’s BAL criteria gives us a specificity of 88.8%, although the sensitivity obtained was low. The specificity of certain HR-CT profiles is 99%. In addition, we observed a complementary use of the HR-CT and the BAL. Conclusions: The imaging findings and BAL could be useful for patients with appropriate clinical presentation and for those whose transbronchial biopsy is negative or for whom a confirmatory biopsy cannot be performed. [ABSTRACT FROM AUTHOR]

The most frequent manifestations of Cryptococcus neoformans (CN) disease are systemic infections in immunocompromised patients and localized pulmonary disease in immunocompetent individuals. Such pulmonary cryptococcosis can range from asymptomatic infection to frank pneumonia that can be severe. Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare severe form of pneumonitis caused by a variety of infectious and toxic agents or connective tissue diseases. BOOP due to Cryptococcus neoformans has very rarely been reported; there have been only five such case reports, mostly in immunocompromised patients. We report herein on a case of CN-associated BOOP in an immunocompetent individual and discuss the diagnosis and treatment of this entity. [ABSTRACT FROM AUTHOR]

Cryptogenic organizing pneumonia (COP, formerly bronchiolitis obliterans organizing pneumonia) is rare in children. We describe an 11-year-old girl with Epstein–Barr virus (EBV) reactivation/presumed post-transplant lymphoproliferative disease (PTLD) 15 months after undergoing a deceased donor kidney transplantation. Treatment with reduced immunosuppression, ganciclovir, and cytomegalovirus immunoglobulin was complicated by severe graft rejection, prompting therapy with methylprednisolone, anti-thymocyte globulin and four weekly doses of rituximab (total 1500 mg/m2). Tacrolimus- and prednisone-based anti-rejection prophylaxis was complemented with low-dose sirolimus. When the lactate dehydrogenase and uric acid levels rose 10 weeks after the first rituximab infusion and bilateral pulmonary nodules were detected by computerized tomography, recurrence of PTLD was suspected. Open lung biopsy of the clinically asymptomatic patient identified the nodules as COP, characterized by abundant CD3+ T-cells, few B-cells, and the absence of EBV, cytomegalovirus, or adenovirus antigens. With normalization of the peripheral B-cell count, EB viremia reappeared and persisted, despite minimal immunosuppression. Four years later, the patient was diagnosed with classical Hodgkin lymphoma-type PTLD with multiple pulmonary and abdominal nodes. This first report of rituximab-associated, pediatric COP highlights the risk of pulmonary complications after treatment with B-cell depleting agents in solid organ transplant recipients, and the importance of a histopathologic diagnosis and vigilant follow-up of such lesions. [ABSTRACT FROM AUTHOR]

Several drugs have been implicated in the induction of systemic lupus erythematosus (SLE), but there are only some observations of carbamazepine induced SLE since the first case described in 1966, this drug has also been implicated in the induction of other disorders and rarely pulmonary toxicity; but the occurrence of two rare side effects of this drug: the induced SLE and pulmonary nodules consistent with the bronchiolitis obliterans organizing pneumonia in same patient is really unusual and -to our knowledge- the second observation reported in the literature. [ABSTRACT FROM AUTHOR]

Bronchiolitis obliterans organizing pneumonia (BOOP) is a diffuse infiltrative pulmonary disorder that has an insidious onset. The patient often presents with flulike symptoms and radiological findings showing bilateral patchy infiltrates. Histological features show fibromyxoid connective tissue plugs that are evenly spaced in small distal airways and alveolar spaces. Treatment for BOOP is corticosteroid therapy. Critical care nurses must be cognizant of BOOP as a differential diagnosis for patients for whom antibiotic therapy has not resolved symptoms. [ABSTRACT FROM AUTHOR]

BACKGROUND: Bronchiolitis obliterans organizing pneumonia (BOOP) is a distinct pattern of reaction of the lung to injury. It may be idiopathic or secondary to a variety of injuries. The term cryptogenic organizing pneumonia (COP) is used for patients with idiopathic BOOP. In this study we describe clinical and radiologic features of patients with BOOP. METHODS: The medical records of 33 patients with diagnosis of BOOP on surgical lung biopsy over a 10-year time period were reviewed retrospectively. We obtained data on clinical and radiologic manifestations, etiology, and outcome of these patients. RESULTS: Dyspnea was the most common symptom, followed by dry cough and fever. Crackles was the most common physical finding. Mean age at diagnosis of BOOP was 59 years, and 42% were females. The main radiologic manifestation was bilateral patchy consolidation. Most patients had favorable prognosis; however, 17% did not respond to treatment. Female sex was more common in COP than in secondary BOOP (P = .004). Patients with COP had longer symptom duration before the diagnosis than secondary BOOP (P = .01). Patients with secondary BOOP reported fever more frequently, compared to COP (P = .005). Pleural effusion was present in 60% of patients with secondary BOOP, whereas none of the patients with COP had effusion (P = .004). CONCLUSIONS: COP and secondary BOOP have diverse clinical and radiologic manifestations. Patients with secondary BOOP are more symptomatic. Both COP and secondary BOOP patients have good prognosis, and most respond to treatment with corticosteroids or by discontinuing the injurious drug. [ABSTRACT FROM AUTHOR]

Summary: Cryptogenic organizing pneumonia (COP), also known as bronchiolitis obliterans organizing pneumonia (BOOP) is emerging to be a frequently recognized pulmonary illness. The management and follow-up of the disease progress remains arbitrary. We present a case of COP where integrated computerized tomography-positron emission tomography (CT-PET) along with blood inflammatory markers, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were used to assess the progress of the disease process. Fluorodeoxyglucose (FDG) activity on CT-PET correlated well with blood inflammatory markers during remission and relapse. To the best of our knowledge, this is the first case report to show such correlation. CT-PET may provide valuable information about the disease activity distinguishing active inflammation from residual fibrosis and thus help to tailor the steroid therapy. [Copyright &y& Elsevier]

Background: Although organizing pneumonia (OP) is a common pathological finding, studies including a substantial number of patients with idiopathic forms from a unique center and a long follow-up are rare. Objectives: To determine patients with cryptogenic forms of organizing pneumonia (COP), in order to characterize their clinical course, to identify predictive factors for relapse and to assess their effect on outcome. Methods: For a 19-year period, all histopathological reports from a community teaching hospital were reviewed, and OP was found in 210 lung specimens belonging to 197 patients. Results: Thirty-three (17%) patients presented cryptogenic forms and 32 of them (97%) responded to steroid therapy. At follow-up, 14 patients presented no relapses (no-relapse group, NR) and 18 (56%) presented relapses (relapsing group, RG) that resolved with ulterior treatment. Multifocal opacities on chest X-ray (RG 83% vs. NR 36%, p = 0.02) appeared to be a predictor for relapse. Patients with relapses showed a shorter time span to chest X-ray normalization (RG 8 ± 8 weeks vs. NR 13 ± 9 weeks, p = 0.09) that became significant in patients with 3 or more relapses (multiple-relapse group, MR, 4 ± 2 weeks vs. NR 13 ± 9 weeks, p < 0.04). Although the initial prednisone dose was similar in patients with relapsing forms, its maintenance was shorter than in patients without relapses, showing a trend to significance (RG 4 ± 3 weeks, NR 7 ± 6 weeks, p = 0.09). Lower levels of lactate dehydrogenase and γ-glutamyltransferase, although always within the normal range, were found in patients with relapsing forms. Conclusion: COP is a specific but infrequent form of OP with a good response to steroid therapy. Relapses are frequent and typical characteristics of COP which resolved with ulterior treatment. Multifocal opacities on chest X-ray and a shorter maintenance of the initial steroid dose may increase the risk of relapse. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The article focuses on the study which aims to determine the association of pulmonary disease with inflammatory bowel disease (IBD). To test, the medical records of IBD patients referring to their respiratory abnormalities and the case series documenting instances of respiratory pathology in patients with known IBD were reviewed. The studies, which used respiratory symptoms, high-resolution CT and pulmonary function testing, found that respiratory system disorders are common among IBD patients.