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Objective: The organizing pneumonia (OP) pattern is the second most common finding in anti-synthase antibody syndrome (ASS), and nonspecific interstitial pneumonia (NSIP) is the most common finding. This study analysed the OP score changes by semiquantitative and quantitative analysis methods and the correlation between the high-resolution computed tomography (HRCT) indexes and the pulmonary function test parameters (PFTs) in ASS patients., Methods: Data from ASS-OP patients admitted to the respiratory department of Ping Jin Hospital from October 2014 to June 2020 were retrospectively reviewed and analysed., Results: Fourteen ASS-OP patients were recruited for this study. (1) In method-1, the consolidation (CO) score and the mean lung attenuation (MA) of poorly aerated and fibrosis lung fields (MA fibrosis ) (r=0.56, P=0.04), the ground-glass opacity (GGO) score and the MA of non-aerated lung fields (MA nonaerated ) (r=-0.64, P=0.01), and the CO plus the GGO (CG) score and the MA nonaerated (r=-0.59, P=0.03) of the lung fields had liner correlations. In method-2, the GGO score to the MA nonaerated (r=-0.58, P=0.03), and the CG (r=-0.68, P=0.01) score to the MA nonaerated had liner correlations. The FVC% (r=0.68, P=0.01) and FEV 1 % (r=0.64, P=0.01) to the MA fibrosis had good linear correlations. (2) Compared to the values before treatment, the CO pattern score, volume and weight percentages of the extracted whole lung volume with attenuation values of the nonaerated area (V nonated %, W nonaerated %), the volume of poorly aerated and fibrosis lung tissue (V fibrosis %, W fibrosis %), the weight percentages of normal aerated lung (W normal %), and the MA fibrosis exhibited significant differences during the 3-6 month follow-up period., Conclusion: The GGO and CO scored by the semiquantitative or quantitative analysis methods was similar. The HRCT quantitative analysis parameters showed a good correlation with the PFTs in ASS-OP patients, can provide an accurate OP pattern interpretation, and may be used as a monitoring and therapeutic indicator for ASS-OP patients., (Copyright © 2021 Elsevier España, S.L.U. All rights reserved.)

Childhood interstitial lung diseases, which some authors refer to as diffuse diseases of the lung, constitute a group of entities that are characterized by remodeling of the interstitium and distal airspaces that cause disturbances of gas exchange in the lungs. While some entities have few symptoms and naturally evolve favorably, others are potentially lethal. Its etiology is very varied, including forms of genetic cause, infectious origin, associated with systemic diseases, drugs and some remain of unknown origin. At present, the development of genetic testing allows diagnosing a group of pathologies, avoiding sometimes a lung biopsy. Its treatment includes different immunosuppressive and immunomodulatory drugs, mainly corticosteroids and hydroxychloroquine, which aim to reduce inflammation, stabilize the disease and prevent the phenomena of remodeling and fibrosis. This consensus is focused on children under 2 years of age, because most of the new entities recently described are manifested at this age., Competing Interests: The authors report no conflicts of interest in this work., (Sociedad Argentina de Pediatría.)

Maximum androgen blockade is the standard endocrine treatment for advanced prostate cancer. Interstitial lung disease in different degrees of severity, with low mortality and excellent response to treatment may appear with its use. We report a 77 years old patient with advanced prostate cancer who developed severe and progressive respiratory failure associated to bilateral pulmonary infiltrates, attributed to the direct effect of maximum androgen blockade. Despite the therapeutic efforts, the patient died. Lung pathology revealed Usual Interstitial Pneumonia.

Unlabelled: Primary Sjögren syndrome (PSS) is a chronic inflammatory autoimmune disease. Interstitial lung disease (ILD) can be an extraglandular complication., Objective: To evaluate the clinical characteristics of patients diagnosed with PSS with ILD., Methods: Multicentre cohort study with 25 patients diagnosed with PSS and ILD. Data of PSS, prognostic factors, pulmonary involvement variables, complementary tests that suggest a worse diagnosis and treatment given were collected. EULAR index was measured for Sjögren's syndrome., Results: We identified 25 patients. In 15/25 the diagnosis of ILD was done before the diagnosis of PSS. The histopathological patterns found were: 12 NSIP, 5 UIP, 4 OP, 2 LIP. PFRs showed restrictive pattern. The majority of the patients received glucocorticoid therapy, antimalarial or immunosuppressive treatment., Conclusions: Patients affected with PSS must be screened to catch a precocious diagnosis of ILD. The majority of the patients were diagnosed of ILD before being diagnosed of PSS. Multicenter cohorts are increasingly demanded and a multidisciplinary management is needed., (Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Background: Assessment of patients with suspected interstitial lung disease (ILD) includes surgical lung biopsy (SLB) when clinical and radiological data are inconclusive. However, cryobiopsy is acquiring an important role in the ILD diagnostic process. The objective of this study was to evaluate the diagnostic yield, safety and economic costs of the systematic use of cryobiopsy in the assessment of patients with suspected ILD., Methods: This was a retrospective observational study of patients who had undergone transbronchial cryobiopsy for evaluation of ILD from January 2011 to January 2014. The procedures were performed with a video bronchoscope using a cryoprobe for the collection of lung parenchyma specimens, which were analyzed by pathologists. Diagnostic yield, complications and economic costs of this technique were analyzed., Results: Criobiopsy specimens from a total of 33 patients were included. A specific diagnosis was obtained in 26, producing a diagnostic yield of 79%. In 5 patients, SLB was required for a histopathological confirmation of disease, but the procedure could not be performed in 4, due to severe comorbidities. The most frequent complications were pneumothorax (12%) and gradei (9%) or gradeii (21%) bleeding. There were no life-threatening complications. The systematic use of cryobiopsy saved up to €59,846., Conclusion: Cryobiopsy is a safe and potentially useful technique in the diagnostic assessment of patients with ILD. Furthermore, the systematic use of cryobiopsy has an important economic impact., (Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.)

Introduction: Our objective was to evaluate whether the number and volume of surgical lung biopsies (SLB) influence the diagnosis of diffuse interstitial lung disease (ILD)., Methods: Retrospective study of SLB for suspected ILD in patients from the Mayo Clinic from January 2002 to January 2010. Data were collected in the institution and analyzed., Results: 311 patients were studied. Mean number of biopsies was 2.05 (SD 0.6); 1 biopsy in 50 (16%), 2 in 198 (63.7%), 3 in 59 (19%) and 4 in 4 (1.3%). Histopathologic diagnosis was: definitive (specific): 232 (74.6%), descriptive (non-specific): 76 (24.4%), no diagnosis: 3 (1%). After excluding patients without diagnosis (n=3), there were 50 patients with only 1 biopsy, 196 with 2 and 62 with 3 or 4; the definitive diagnostic yield was similar in all 3 groups (37/50; 74%, 150/196; 77%, and 45/62; 73%) (Chi-square, p value 0.8). The propensity score analysis between patients with 1 SLB and patients with more than 1 SLB also showed no difference in diagnostic yield. Regarding the volume of biopsies, mean total volume was 34.4 cm(3) (SD 46): 41.2 cm(3) (3 cases) in patients with no diagnosis; 33.6 cm(3) (232 cases, SD 47) in patients with specific diagnosis; and 36.6 cm(3) (76 cases, SD 44) in patients with descriptive diagnosis. Biopsy volume had no influence on histopathology yield (ANOVA, p value .8)., Conclusions: The number and volume of the biopsy specimens in SLB did not seem to influence diagnosis. Based on our results, we believe a single sample from a representative area may be sufficient for diagnosis. Randomized prospective trials should be performed to optimize SLB for ILD., (Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.)

Objectives: To evaluate whether the location and number of lung biopsies obtained by video-assisted thoracoscopy (VAT) influence the diagnosis of diffuse interstitial lung disease (ILD). To assess the applicability of an Ambulatory Surgery Program (ASP)., Methods: Prospective, multicenter study of VAT lung biopsies due to suspected ILD from January 2007 to December 2009, including 224 patients from 13 Spanish centers (mean age 57.1 years; 52.6% females). Data were prospectively collected in every institution and sent to the coordination center for analysis., Results: The most affected areas in high resolution chest CT were the lower lobes (55%). Bronchoscopy was performed in 84% and transbronchial biopsy in 49.1%. In 179 cases (79.9%), more than one biopsy was performed, with a diagnostic agreement of 97.2%. A definitive histopathologic diagnosis was obtained in 195 patients (87%). Idiopathic pulmonary fibrosis was the most frequent diagnosis (26%). There were no statistically significant factors that could predict a greater diagnostic yield (neither anatomical location nor number of biopsies). Seventy patients (31.3%) were included in an ASP. After discharge, there were complications in 12 patients (5.4%), similar between patients admitted postoperatively (9/154: 5.8%) and those included in an ASP (3/70: 4.3%)., Conclusions: Anatomical location and number of lung biopsy specimens did not seem to influence the diagnosis. The patients included in an ASP had a complication rate comparable to that of the hospitalized, so this procedure can be included in a surgical outpatient program. Lung biopsy obtained by VAT is a powerful and safe tool for diagnosis of suspected ILD, resulting in a definitive diagnosis for the majority of patients with a low morbidity rate., (Copyright © 2011 SEPAR. Published by Elsevier Espana. All rights reserved.)

Background: Induced sputum is a non-invasive method for studying pulmonary inflammation., Objectives: To assess pulmonary inflammation by analysis of induced sputum specimens in patients with systemic sclerosis and lung involvement, and to determine whether there is a correlation with the pulmonary function alterations in these patients., Methods: Twenty-five patients with systemic sclerosis were included (20 women). Patients were divided into 3 groups according to the type of lung involvement: group 1, diffuse interstitial lung disease (n=10); group 2, those with pulmonary arterial hypertension (n=7), and group 3, patients with systemic sclerosis without lung involvement (n=8). All patients underwent a complete lung function study. Induced sputum samples were obtained and differential cell count was performed by optic microscopy., Results: The mean percentage of sputum neutrophils was 85%, 71%, and 75% for groups 1, 2, and 3, respectively. A significant negative correlation between sputum total cell count and DLCO was seen in group 1 and group 3 (r=-0.733, P=.016; and r=-0.893, P=.007, respectively). This negative correlation was not observed in group 2., Conclusions: Pulmonary inflammation was present in all patients with systemic sclerosis included in the study, regardless of the presence of documented signs of pulmonary involvement. This finding suggests that induced sputum could be helpful for detecting early abnormalities indicative of subclinical pulmonary involvement in patients with systemic sclerosis., (Copyright © 2011 SEPAR. Published by Elsevier Espana. All rights reserved.)

The interstitial lymphoid pneumonia (LIP) is an uncommon disorder, described as non-neoplastic lung lymphoid tissue hyperplasia and classified as an interstitial lung disease. It has been described in association with HIV infection, autoimmune disorders, policlonal hypergammaglobulinemia and less frequently, with hypogammaglobulinemia. We report the case of a 66 year old female patient with a history of diabetes, Sjogren syndrome and hypertension. She was referred to our hospital due to a dry cough and dyspnea (FC II-III). The physical examination showed bilateral dry crackles and splenomegaly. Laboratory studies showed thrombocytopenia (50 000/ mm3) and hypogammaglobulinemia. A computed tomography thoracic scan showed interstitial bilateral basal lung infiltrates with small peribroncovascular nodules. A lung biopsy was made by thoracoscopy and reported LIP. Initial treatment consisted of oral methilprednisone, 40 mg daily, and once a month intravenous gammaglobulin 500 mg/kg, with good clinical and radiological response. A splenectomy was done due to persistent thrombocytopenia associated with hypersplenism, with good response of the platelets counts. No lymphoid clonal or other associated disease was detected.

Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease, of unknown etiology, affecting almost exclusively women. Microscopically LAM consists of a diffuse proliferation of smooth muscle cells. LAM can occur without evidence of other diseases (sporadic LAM) or in conjunction with tuberous sclerosis complex (TSC). It presents with progressive breathlessness or with recurrent pneumothorax or chylothorax. We report a 33 year-old woman with a history of recurrent pneumothorax. Computed tomography (CT) scans showed numerous thin-walled cysts throughout the lungs, a characteristic finding in LAM. A pulmonary biopsy was compatible with the diagnosis and HMB-45 monoclonal antibodies were positive. Treatment with Sirolimus was started).

We report the case of a 40-year-old woman diagnosed with interstitial lung disease due to long-term nitrofurantoin therapy. Despite severely distorted bronchiolar architecture and honeycombing confirmed by computed tomography of the thorax, transbronchial biopsy showed a pattern of acute/subacute interstitial pneumonitis and the symptoms and radiographic findings disappeared within 1 month after administration of prednisone. This case shows that nitrofurantoin-induced lung disease may run a benign course and respond favorably to corticosteroids, even when there is radiographic evidence of established lung fibrosis. Transbronchial biopsy might be useful for assessing the reversibility of pulmonary lesions associated with nitrofurantoin.

Objective: Study of the bronchoalveolar lavage (BAL) fluid in some interstitial lung diseases can reveal patterns typical to each disease and that can support the diagnosis. The objective of this study was to perform a descriptive analysis of the cytologic study and of the lymphocyte subpopulations in BAL fluid from patients with interstitial lung disease., Material and Methods: In this prospective, observational study of 562 patients between January 1991 and January 2005, BAL fluid was analyzed to determine the distribution of cell populations and of lymphocyte subsets: CD3, CD4, CD8, CD3(+)CD4(-)CD8(-), and CD56., Results: The mean age was 53.4 years and 53.3% of the patients were women. The following diseases were studied: idiopathic pulmonary fibrosis (n=132), sarcoidosis (n=123), connective tissue diseases (n=133), cryptogenic organizing pneumonia (n=89), and extrinsic allergic alveolitis (n=85). Isolated lymphocytic alveolitis was common in sarcoidosis and extrinsic allergic alveolitis. Mixed alveolitis was the most common pattern in the other interstitial lung diseases. The CD4:CD8 ratio was the most useful parameter. It was high in sarcoidosis (median, 2.3); the ratio was low or inverted in the other interstitial lung diseases, with median values of 1.76 in idiopathic pulmonary fibrosis, 0.45 in extrinsic allergic alveolitis, 0.35 in cryptogenic organizing pneumonia, and 0.33 in the connective tissue diseases., Conclusions: BAL parameters, in association with clinical and radiologic data, help to discriminate between interstitial lung diseases. BAL should therefore be considered a very useful tool in clinical management, particularly when pulmonary biopsy is not conclusive or is not possible.

Polymyositis is a rare collagen disease that can involve the lungs. Between 5% and 30% of patients with polymyositis present interstitial lung disease at diagnosis or during the course of disease. Onset is usually insidious and involves dyspnea and nonproductive cough. Several histopathological findings are associated with polymyositis and the most common is nonspecific interstitial pneumonia. The prognosis of interstitial lung disease associated with polymyositis is better than that of idiopathic pulmonary fibrosis, since most patients respond to treatment with corticosteroids and immunosuppressants. We report the case of a 60-year-old woman with dyspnea and muscle weakness who was diagnosed with polymyositis and interstitial lung disease (radiography indicated possible nonspecific interstitial pneumonia). The patient responded well to prednisone and methotrexate.

Idiopathic interstitial pneumonias represent a diverse group of lung diseases with diffuse effects on the lung parenchyma. In 2002, the American Thoracic Society/European Respiratory Society consensus classification unified the descriptions of the different entities encompassed by idiopathic interstitial pneumonias. Despite this broad consensus there are still some entities without a clear definition and others, such as idiopathic bronchiolocentric interstitial pneumonia, that were only later described. We present the case and outcome of a woman diagnosed with idiopathic bronchiolocentric interstitial pneumonia by lung biopsy.

The objective of this study was to determine the morbidity, mortality and diagnostic yield of video assisted thoracoscopy (VATS) and thoracotomy lung biopsy in interstitial lung disease (ILD). Clinical records of 71 patients were retrospectively analyzed. There was no difference in mean hospital stay, intensive care unit stay and duration of chest tube drainage in patients with VATS (n = 52) compared with those undergoing open thoracotomy (n = 17). Complications rate (22.2% vs. 21.0%, p = 1.0000) and operating mortality (9.2 vs. 15.7%, p = 0.2738) were also similar. Overall, complications occurred in 16 patients (22.5%). Thirty-day mortality rate was 11.2% (n = 8). Prevalence of immunosupression (4/8 vs. 9/63, p = 0.0325) was significantly higher in the group of patients who died. No surviving patients had higher values of plasmatic urea (50 +/- 20.1 mg/dl vs. 31.2 +/- 10.3 mg/dl, p = 0.0013) or lower values of preoperative oxygen saturation (SaO2): 82.7 +/- 14.8% vs. 92.8 +/- 3.4%, (p = 0.0009). Eleven patients had an acute illness. Those patients did not show a higher complication rate (4/11 vs. 10/45, p = 0.4390) but mortality was significantly higher (4/11, 36.3% vs. 3/45, 7.1%, p = 0.0223). Biopsy allowed a specific histologic diagnosis in 100% of patients and changed therapy in 66.7%. We conclude that surgical lung biopsy is a safe and useful procedure in patients with ILD. However the higher mortality rate in patients with acute symptoms, immunocompromise, or in respiratory failure must be balanced against potential benefits of altering treatment decisions.

Objective: Diffuse interstitial lung diseases (DILD) form a group of diseases which affect the alveolar interstitial space and share very similar clinical, radiological, and functional features, making lung biopsy essential for establishing diagnosis, prognosis, and treatment in many cases. We aimed to see whether there was agreement in histopathological diagnosis among different groups of pathologists in their assessment of these diseases., Material and Methods: Biopsies were studied from 33 patients suffering from noninfectious, nontumorous DILD. The biopsies had been assessed by 2 groups of pathologists: one specializing in this type of disease and another which was not a specialist group., Results: There was disagreement in the histology reports of 10 out of the 33 cases studied (30.3%): 9 cases in the group of 22 cases of idiopathic interstitial pneumonia (40.9%) and 1 in the group of 3 DILD with known or associated causes. No discrepancies were found, however, in the diagnosis of primary DILD or DILD associated with other, less well-defined processes., Conclusions: We believe that idiopathic interstitial pneumonias are the DILD which pose most problems for pathologists. Therefore, the study of DILD requires specific dedication by pathologists and other professionals and specialists.

Lymphoid interstitial pneumonia (LIP) is a rare entity characterized by the infiltration of interstitial tissues and alveolar spaces by lymphocytes, plasma cells, and other lymphoreticular structures. The etiology of LIP is unknown, although associations with autoimmune and infectious factors have been described. The incidence of LIP has risen in recent years, mainly in children with acquired immunodeficiency syndrome (AIDS), while remaining less common in the adult population. No agreement has been reached regarding the diagnostic tests necessary for a firm diagnosis although suspicion is usually based on clinical and radiographic findings, with confirmation provided by examination of histological samples. The most common treatment is corticosteroids, either alone or in combination with other immunosuppressant agents although no evidence from controlled trials is available and cases have been reported in which LIP resolved in AIDS patients with antiretroviral therapy alone. We report the case of a human immunodeficiency virus-infected adult who was diagnosed with LIP by open lung biopsy and who responded to antiretroviral drugs with no need for associated corticosteroid therapy.

We report a 50 years old female, admitted to the hospital due to malaise lasting 7 months, with diffuse bilateral lung infiltrates and a progressive respiratory insufficiency, dying one month after admission. A transbronchial biopsy disclosed an extensive amyloid infiltration. The necropsy confirmed this finding and showed a systemic primary amyloidosis associated to a multiple myeloma.

Histopathology plays an important role in the diagnosis of diffuse interstitial lung disease. New endoscopic techniques allow performance of sampling procedures that have the same diagnostic accuracy and fewer complications that open lung biopsy (OLB). We evaluated our experience with video thoracoscopic lung biopsy (VTLB) in comparison with OLB in terms of diagnostic accuracy, duration of pleural drainage and hospital stay, number and size of samples and complications. Thirty patients who underwent VTLB from March 1987 to January 1995, and 28 patients who underwent OLB from May 1987 to January 1995 were studied retrospectively. Diagnosis was achieved in the VLTB group in 96.66% of cases and in the OLB group in 92.85%. The number of specimens obtained was 1.5 (SD 0.5) in the VTLB group (p = 0.47). Overall specimen size expressed in cm3 was 13.3 (SD-15.4) in the VTLB group and 18.5 (SD 21) in the OLB group (p = 0.284). Length of pleural drainage in hours was 52 (SD 39.9) in the VTLB group and 89.1 (SD 47) in the OLB group (p = 0.002). Length of hospital stay in days was 3.5 (SD 2) in the VTLB group and 8.7 (3.5) in the OLB group (p = 0.001). Complications were fewer and less severe after VLTB. VLTB is as useful as OLD for obtaining lung biopsies in diffuse interstitial lung disease. VLTB also causes fewer complications and is less expensive because drainage times and hospital stays are shorter.

The aim of this work was to evaluate and compare the results of lung biopsies performed by videothoracoscopy (VT) and minithoracotomy (MT) in diffuse interstitial disease between 1992 and 1994, during which period 45 lung biopsies were performed on patients (20 women) with a mean age of 56 years. Thirty-one biopsies were performed by VT (group I) and 14 by MT (group II). For all patients we recorded duration of procedure, days to discharge after surgery, total time with a pleural drain in place, and volume of tissue sample. Diagnosis was possible in all cases. Mean duration of surgery was 49.3 minutes for group I and 58.2 minutes for group II. Mean time to discharge was 6.7 days for group I and 10.1 for group II. Mean time of pleural drainage was 3.8 days for group I and 5.9 days for group II. Mean volume of tissue sample was 6.5 cm3 for group I and 5.18 cm3 for group II. One patient died in group I from causes unrelated to surgical technique. In conclusion, lung biopsy with VT is effective and useful for diagnosing diffuse interstitial lung diseases. The sample obtained is in every way comparable to that obtained by thoracotomy. Mean hospital stay and mean time of postoperative drainage decreases significantly (p < 0.05) with VT.

Our objective was to assess the capacity of clinical and laboratory information to predict findings in the lung biopsy in interstitial lung diseases (ILD). We studied 121 patients with ILD as a cohort recruited in our institute from 1983 to 1987 with the diagnosis of hypersensitivity pneumonitis (HP) and usual interstitial pneumonia (UIP). Histologic diagnosis (HP vs UIP) and degree of fibrosis (< 50% of the biopsy surface vs > or = 50%) were used as the gold standard to compare a series of clinical and laboratory variables in the initial assessment. We used a stepwise logistic regression model to predict the biopsy results. The model was calculated in half of the patients selected by random sampling, and the calculated model was tested in the other half of the patients. Variables found to predict degree of fibrosis were (with relative risk RR and 95% confidence interval): a radiographic pattern of honeycombing (RR 5.0 from 0.9-29), digital clubbing (RR 8 from 1.4-48) and gender (RR 2.9 from 0.4-20). This model classified correctly 72% of the biopsies, with a sensitivity of 0.38, a specificity of 0.85 and a kappa of 0.25 +/- 0.19 (p = 0.17 NS). For histologic diagnosis (NIU vs NH), the model included gender (RR 6.6, 1.3-33), honeycombing (RR 1.6, from 0.4-6.0), digital clubbing (RR 4.6, from 1.2-18), and vital capacity expressed as percent of predicted (RR 0.96, from 0.92-1.00).(ABSTRACT TRUNCATED AT 250 WORDS)

We present a patient with hemochromatosis whose first sign of disease was an accidental finding of interstitial patterns on a chest film and whose transbronchial biopsy showed traces of iron (Fe) in alveolar macrophages and septal matter. We then looked for deposits in other locations, finding Fe ([+]: 6.964 micrograms) in the dry weight of a cylindrical sample of liver tissue. A Medline search on CD-ROM for 1989 through May of 1994 revealed no references to similar cases.

A 63-year-old woman with follicular bronchiolitis presenting as diffuse interstitial pneumopathy is described. Diagnosis was made by open lung biopsy and the patient was treated in our department until death. The course of disease and response to treatment was similar to that observed in cases of idiopathic pulmonary fibrosis. No associated collagen disease was found. This report reviews the histological and clinical signs of this disease, as well as treatment.

We describe a woman with lymphoid interstitial pneumonia diagnosed by open lung biopsy following a profile of unproductive cough, weakness and bilateral lung infiltrates. The patient was also diagnosed as having common variable immunodeficiency based on hypogammaglobulinemia, repeated sinusitis and persistent diarrhea. Exfoliative cytology of pleural effusion revealed the development of non-Hodgkin's lymphoma. We review the associations among these processes and consider the suggestion made by other authors that lymphoid interstitial pneumonia be considered a prelymphomatous process.

A 17 years old female with hepatosplenomegaly and pulmonary infiltrates since the age of four is presented. Sea-blue histiocyte disease, a sphingolipidosis, was diagnosed by liver biopsy, bone marrow aspirate and bronchoalveolar lavage. The relevance of bronchoalveolar lavage as a diagnostic method in cases of systemic diseases with pulmonary involvement is emphasized.