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Patients with common variable immunodeficiency (CVID) can develop granulomatous-lymphocytic interstitial lung disease (GLILD), which is associated with increased morbidity and mortality. Treating GLILD is a significant challenge because it is rare and can be pathologically heterogeneous. Here we describe two cases of patients with CVID-associated GLILD with biopsies demonstrating loosely organized tertiary lymphoid structures (TLSs). Based on the pivotal role that B cells play in TLS initiation and maintenance, we hypothesized that using rituximab monotherapy for B-cell depletion alone would be sufficient for the disruption of the pathologic process underlying GLILD. These two cases demonstrate that adapting a strategy of B cell depletion monotherapy may be effective in TLS-associated conditions such as GLILD.

A 66-year-old man presented with dry cough and shortness of breath on exertion of 6 months' duration. There were no complaints of fever and hemoptysis. His history was significant for recurrent episodes of respiratory tract infections over the previous 4 years. He had also had episodes of recurrent otitis media and pus discharge from the left ear for 3 years, with progressive loss of hearing. There was no history of recurrent skin infections or diarrhea. He was treated symptomatically with antibiotics by local general practitioners. He was a nonsmoker and did not drink alcohol, and there was no history of environmental or occupational exposure. He had been known to have diabetes for 10 years. He had negative results for the presence of HIV and hepatitis B surface antigen.

SESSION TITLE: Fellows Chest Infections Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: A patient with sarcoidosis presented with persistent shortness of breath after being diagnosed with a virus and suspected sarcoidosis flare He was treated with supportive measures and steroids, but did not improve In a patient receiving immunosuppression to control an autoimmune disease, it is important to have a high suspicion for opportunistic infections CASE PRESENTATION: 51-year-old male with history of sarcoidosis was admitted three times in three weeks with fevers, chills, sweats, non-productive cough, and headaches A computerized tomography (CT) of the chest showed extensive mediastinal/hilar adenopathy and worsening of pre-existing parenchymal lung disease (figure 1) During the first two hospital admissions, the patient tested positive for coronavirus OC43 strain (not COVID-19) and was treated supportively He was also treated with steroids for a possible flare of his sarcoidosis Because of lack of improvement, fungal infection was considered Cultures from bronchoscopy with bronchoalveolar lavage and transbronchial biopsy of the right upper lobe revealed Histoplasma capsulatum (figure 2) Because of persistent headaches, the patient underwent a lumbar puncture which also demonstrated Histoplasma capsulatum in the cerebrospinal fluid He was diagnosed with disseminated histoplasmosis and started on amphotericin B Symptoms resolved and respiratory status returned to baseline He completed about 1 1/2 weeks of amphotericin before being transitioned to itraconazole He completed three months of itraconazole before being switched to posaconazole due to drug intolerances Urine histoplasma antigen was undetectable at 6-month follow-up Immunosuppressive regimen was judiciously decreased to balance controlling his sarcoidosis while limiting risk for reigniting the histoplasmosis and developing future opportunistic infections DISCUSSION: Histoplasmosis is the most prevalent endemic mycosis in the United States, typically found in the soil from bird droppings in the Midwest However, it can be found on the east coast, and patients receiving immunosuppression to control their autoimmune disease are at risk for fungal infections such as histoplasmosis This patient was receiving infliximab, a tumor necrosis factor antagonist that has been associated with histoplasmosis Although discontinuing or at least reducing immunosuppressant agents is standard of care while treating histoplasmosis, it is unclear when to restart them However, a retrospective analysis of 98 cases of histoplasmosis reported that it appears safe to resume immunosuppression, and even tumor necrosis factor antagonist therapy, if antifungal therapy was administered for at least 12 months with an appropriate clinical response CONCLUSIONS: Opportunistic infections must be ruled out in the setting of any immunosuppression, which can further exacerbate the acute illness and vastly increase mortality Reference #1: Kasper DL, Fauci AS, Hauser SL, et al Histoplasmosis Harrison’s Infectious Diseases 3rd Ed 2017;111:996-999 Reference #2: Vergidis P, Avery RK, Wheat LJ, et al Histoplasmosis Complicating Tumor Necrosis Factor-α Blocker Therapy: A Retrospective Analysis of 98 Cases Clinical Infectious Diseases 2015;61(3):409-17 Reference #3: Kobayashi T, Cho C Mediastinal granuloma due to histoplasmosis in a patient on infliximab Cleveland Clinic Journal of Medicine 2019;86(9);579-81 DISCLOSURES: No relevant relationships by Paul Cook, source=Web Response No relevant relationships by Poorvi King, source=Web Response No relevant relationships by Ogugua Obi, source=Web Response No relevant relationships by William Wooten, source=Web Response

Background Pathologic features of end-stage pulmonary sarcoidosis (ESPS) are not well defined; anecdotal reports have suggested that ESPS may mimic usual interstitial pneumonia (UIP). We hypothesized that ESPS has distinct histologic features. Methods Twelve patients who received a diagnosis of pulmonary sarcoidosis and underwent lung transplantation were included. Control subjects were 10 age- and sex-matched lung transplant patients with UIP. Hematoxylin and eosin-stained tissue sections were examined for the following features: extent/pattern of fibrosis; presence and quantity (per 10 high-power fields) of fibroblast foci and granulomas; distribution and morphology of granulomas; and presence and extent of honeycomb change. Extent of fibrosis and honeycomb change in lung parenchyma was scored as follows: 1 = 1% to 25%; 2 = 26% to 50%; 3 = 51% to 75%; 4 = 76% to 100% of lung parenchyma. Results Eight of 12 cases demonstrated histologic findings typical of ESPS. All showed well-formed granulomas with associated fibrosis distributed in a distinct lymphangitic fashion. Granulomas were present in hilar or mediastinal lymph nodes from six of six patients with ESPS and none of eight control subjects. The extent of fibrosis, honeycomb change, and fibroblast foci was significantly lower in ESPS cases compared with control cases. Two patients with remote histories of sarcoidosis showed histologic features of diseases other than ESPS (UIP and emphysema) without granulomas. Two patients with atypical clinical findings demonstrated nonnecrotizing granulomas combined with either severe chronic venous hypertension or UIP. Conclusions ESPS and UIP have distinct histopathologic features in the lungs. Patients with a pretransplant diagnosis of sarcoidosis may develop other lung diseases that account for their end-stage fibrosis.

The diagnosis of sarcoidosis is made by the combination of clinical features and biopsy results. The clinical features of sarcoidosis can be quite variable. We developed a Sarcoidosis Diagnostic Score (SDS) to summarize the clinical features of patients with possible sarcoidosis.Biopsy-confirmed patients with sarcoidosis seen during a 7-month period at the University of Cincinnati sarcoidosis clinic were prospectively identified. Patients with nonsarcoidosis seen at the same clinic were used as control patients. Using a modified World Association of Sarcoidosis and Other Granulomatous Disorders organ assessment instrument, we scored all patients for presence of biopsy, ≥1 highly probable symptom, and ≥1 at least probable symptom for each area. Two sarcoidosis scores were generated: SDS biopsy (with biopsy) and SDS clinical (without biopsy).The 980 evaluable patients were divided into two cohorts: an initial 600 patients (450 with biopsy-confirmed sarcoidosis, 150 control patients) to establish cutoff values for SDS biopsy and an SDS clinical and a validation cohort of 380 patients (103 biopsy-confirmed patients with sarcoidosis and 277 control patients). The best cutoff value for SDS biopsy was ≥ 6 (sensitivity, 99.3%; specificity, 100%). For the total the 980 patients, an SDS clinical ≥ 3 had a sensitivity of 90.6%, specificity of 88.5%, and a likelihood ratio of 7.9. An SDS clinical score ≥ 4 had a lower sensitivity of (76.9%) but higher specificity (98.6%).For sarcoidosis, the presence of specific clinical features, especially multiorgan involvement, can enhance the diagnostic certainty. The SDS scoring system quantitated the clinical features consistent with sarcoidosis.

Endobronchial ultrasonographically guided transbronchial needle aspiration (EBUS-TBNA) of thoracic structures is a commonly performed tissue sampling technique. The use of an inner-stylet in the EBUS needle has never been rigorously evaluated and may be unnecessary.In a prospective randomized single-blind controlled clinical trial, patients with a clinical indication for EBUS-TBNA underwent lymph node sampling using both with-stylet and without-stylet techniques. Sample adequacy, diagnostic yield, and various cytologic quality measures were compared.One hundred twenty-one patients were enrolled, with 194 lymph nodes sampled, each using both with-stylet and without-stylet techniques. There was no significant difference in sample adequacy or diagnostic yield between techniques. The without-stylet technique resulted in adequate samples in 87% of the 194 study lymph nodes, which was no different from the with-stylet adequacy rate (82%; P = .371). The with-stylet technique resulted in a diagnosis in 50 of 194 samples (25.7%), which was similar to the without-stylet group (49 of 194 [25.2%]; P = .740). There was a high degree of concordance in the determination of adequacy (84.0%; 95% CI, 78.1-88.9) and diagnostic sample generation (95.4%; 95% CI, 91.2-97.9) between the two techniques. A similar qualitative number of lymphocytes, malignant cells, and bronchial respiratory epithelia were recovered using each technique.Omitting stylet use during EBUS-TBNA does not affect diagnostic outcomes and reduces procedural complexity.ClinicalTrials.Gov: No. NCT 02201654; URL:www.clinicaltrials.gov.

A 54-year-old French man was admitted for evaluation of a chronic nodular lesion of the tongue and mandibular lymphadenopathy. He reported active tobacco and cannabis smoking as well as excessive alcohol use. He also reported frequent use of cocaine for several months and a past addiction to IV heroin. He had traveled abroad as a journalist and lived for several months in Columbia and Venezuela 12 years ago. His medical history included chronic hepatitis C infection successfully treated with interferon and ribavirin 6 years ago and high BP.

Background Comparisons of lung manifestations in primary pulmonary vs disseminated nontuberculous mycobacterial disease have not been well described. The clinical, histopathologic, and radiologic disease manifestations of primary pulmonary or disseminated nontuberculous mycobacterial disease were compared in an autopsy series. Methods Medical and microbiologic records, autopsy reports, histopathologic slides of the lungs, and chest CT scans were reviewed on patients at the National Institutes of Health with nontuberculous mycobacterial disease who died between 1996 and 2010. Results The 11 patients with primary pulmonary nontuberculous mycobacterial disease were predominantly female (n = 9), with symptom onset at median 50 (range 35, 71) years and time from onset until death of 12 (3, 34) years. Bronchiectasis with cavity formation and necrotizing bronchocentric granulomatous inflammation predominated but extrapulmonary infection was absent. The five patients with disseminated disease and systemic immune defects were all men with age at onset of 2 (0.33, 33) years and time from onset of disease until death of 9 (1, 31) years. Miliary nodules and/or consolidation with poorly formed granulomatous inflammation were noted in the three disseminated patients with mycobacterial lung involvement. Significant extrapulmonary infection was noted in all five with a relative paucity of lung findings. Conclusions Nontuberculous mycobacteria can cause progressive, fatal disease. Primary pulmonary disease is bronchocentric and lacks extrathoracic infection consistent with impaired airway surface defenses. In contrast, fatal disseminated infections involving the lung have hematogenous spread, extensive extrathoracic disease, and a distinct pulmonary histopathology consistent with systemic immune dysfunction.

A white woman in her 80s presented to the ED with nonproductive cough, fever, and 2 weeks of progressive generalized weakness. Previously ambulatory, she now was so weak she required assistance out of bed.

American College of Chest Physicians (CHEST) clinical practice guidelines on the evaluation of pulmonary nodules may have low adoption among clinicians in Asian countries. Unique patient characteristics of Asian patients affect the diagnostic evaluation of pulmonary nodules. The objective of these clinical practice guidelines was to adapt those of CHEST to provide consensus-based recommendations relevant to practitioners in Asia.A modified ADAPTE process was used by a multidisciplinary group of pulmonologists and thoracic surgeons in Asia. An initial panel meeting analyzed all CHEST recommendations to achieve consensus on recommendations and identify areas that required further investigation before consensus could be achieved. Revised recommendations were circulated to panel members for iterative review and redrafting to develop the final guidelines.Evaluation of pulmonary nodules in Asia broadly follows those of the CHEST guidelines with important caveats. Practitioners should be aware of the risk of lung cancer caused by high levels of indoor and outdoor air pollution, as well as the high incidence of adenocarcinoma in female nonsmokers. Furthermore, the high prevalence of granulomatous disease and other infectious causes of pulmonary nodules need to be considered. Therefore, diagnostic risk calculators developed in non-Asian patients may not be applicable. Overall, longer surveillance of nodules than those recommended by CHEST should be considered.TB in Asia favors lesser reliance on PET scanning and greater use of nonsurgical biopsy over surgical diagnosis or surveillance. Practitioners in Asia are encouraged to use these adapted consensus guidelines to facilitate consistent evaluation of pulmonary nodules.

Irecently consulted on a young woman who had acquired fever, profuse pulmonary shadowing, and liver function abnormalities following a 1997 visit from her residence in upstate New York to the Mississippi Valley. An open-lung biopsy at that time revealed noncaseating epithelioid granulomas (NCG) consistent with sarcoidosis; fungal elements were not evident. The treating physician initiated prednisone therapy, but after a week, a histoplasmoFor editorial comment see page 6 sis complement fixation titer of 512, later supplemented by isolation of Histoplasma capsulatum organisms from the biopsy sample, led to its replacement with a short course of amphotericin B, followed by months of itraconazole therapy, with the expectation that her progressive disseminated histoplasmosis would resolve. She was unavailable for follow-up until 2001, when she was re-referred for evaluation of increasingly severe shortness of breath. Prednisone had been provided by her primary physician. A chest radiograph showed profuse pulmonary shadowing; high-resolution CT demonstrated findings characteristic of advanced stage IV sarcoidosis; pulmonary function tests revealed a severe restrictive defect accompanied by a marked reduction in diffusing capacity. Prednisone was discontinued because of the concern that her histoplasmosis

Study objectives To assess the efficacy and complications of different interventional bronchoscopic techniques used to treat airway complications after lung transplantation. Design Retrospective study. Setting Heart-lung transplant unit of a university hospital. Patients From November 1986 to January 2000, interventional bronchoscopy was performed in 41 of 312 lung transplant recipients (13.1%) for tracheobronchial stenosis, bronchomalacia, granuloma formation, and dehiscence. Interventions Dilatation, stent placement, laser or forceps excision. Measurements and results Mean (± SE) improvement in FEV 1 in 26 patients undergoing dilatation for a stenotic or a combined lesion was 93 ± 334 mL or 8 ± 21%. In seven of these patients not proceeding to stent placement, mean improvement in FEV 1 was 361 ± 179 mL or 21 ± 9%. Patients needing stent placement after dilatation had a mean change in FEV 1 after dilatation of − 5 ± 325 mL or 3 ± 23%, and an improvement of 625 ± 480 mL or 52 ± 43% after stent insertion. Mean improvement in FEV 1 for patients treated with stent insertion for bronchomalacia was 673 ± 30 mL or 81 ± 24%. Complications of airway stents were migration (27%), mucous plugging (27%), granuloma formation (36%), stent fracture (3%), and formation of a false passage (6%). Mortality associated with interventional bronchoscopy was 2.4% (1 of 41 patients). For patients with airway complications successfully undergoing interventional bronchoscopy, the overall 1-year, 3-year, and 5-year survival rates were 79%, 45%, and 32%, respectively, vs 87%, 69%, and 56% for those without airway complications (p Conclusion Only a small number of patients with airway stenosis after lung transplantation will respond to bronchial dilatation alone. Patients with airway complications after lung transplantation have a higher mortality than patients without airway complications.

Study objective: Airway obstruction (AO) in sarcoidosis is reported to be associated with respiratory symptoms, increased morbidity, and an increased mortality risk. Because AO in sarcoidosis may result from several causes, the therapeutic benefit of corticosteroids is difficult to determine. The aim of this study was to evaluate the therapeutic response of AO attributable to sarcoid granulomas in the bronchial wall. Patients: We selected 11 patients who had sarcoidosis with AO (defined as FEV1/vital capacity [VC] < 70%) associated with sarcoid granulomas on an endobronchial biopsy. Exclusion criteria were history of asthma, smoker or exsmoker, stage 4 disease, evidence of extrinsic compression by enlarged lymph nodes, and localized endobronchial stenosis seen during fiberoptic bronchoscopy. Interventions: We compared the results of pulmonary function tests and clinical, radiologic, and biological findings at baseline with those obtained at the time of the last pulmonary function tests available, between the sixth and 12th months of treatment. Eight patients took oral corticosteroids (20 to 60 mg/d initially), one received IV methylprednisolone pulses, another took oral hydroxychloroquine, and the last one received IM methotrexate. Measurements and results: With treatment, FEV1 and FEV1/VC significantly improved in eight patients (72%), normalized in four patients, and was unchanged in the remaining three patients. The mean FEV1 increased from 60.8 6 10.8% to 76 6 13.7% of the predicted value (p < 0.02). VC did not change significantly. FEV1/VC increased from 76.1 6 6.4% to 87.6 6 10.7% of the predicted value (p < 0.01). Dyspnea on exertion and other clinical findings were attenuated in 10 patients; the chest radiograph improved in 9 patients, and normalized in 5 patients. The mean serum angiotensin-converting enzyme level decreased from 112 6 48 to 58 6 40 IU/mL (p < 0.05), and normalized in four patients. Conclusion: The present study indicates that AO caused by sarcoid granulomas in the bronchial wall can be either partially or completely reversed by treatment with a concomitant attenuation of pulmonary symptoms. (CHEST 1999; 116:1194 ‐1199)

A 62-year-old man was referred to Castle Hill Hospital, East Yorkshire, United Kingdom, with a 1-year history of productive cough and a 2-month history of hemoptysis which had failed to respond to treatment with antibiotics. There was little of note in his past medical history apart from long-standing wheezing and an uncomplicated nasal polypectomy performed 18 months previously. He was otherwise in good health, and there were no other systemic abnormalities. He had a 40 pack-year history of smoking but was not on any medication. Clinical examination showed a fit-looking man with no clubbing or lymphadenopathy. He was afebrile with a pulse of 90 beats per minute and BP of 120/70 mm Hg. Chest examination revealed bilateral wheezing. Results of the rest of the examination were normal. Laboratory investigations showed a hemoglobin level of 14.8 g/dL, a WBC count of 10.4 3 10/L, and a platelet count of 305 3 10/L. There was a 14% eosinophilia and the erythrocyte sedimentation rate was 27 mm/h. Urine microscopy was normal with no evidence of hematuria or proteinuria. Immunologic screening showed a weakly positive antinuclear antibody titer at 1:20, the rheumatoid factor positive at 1:40, and negative antineutrophil cytoplasmic antibodies. The serum IgE level was elevated at . 1,000 U/mL (reference range, 25 to 120 U/mL). Serum precipitins to Aspergillus fumigatus culture were positive. Perfusion lung scanning and Doppler ultrasound of the legs showed no evidence of pulmonary emboli or venous thrombosis, respectively. Spirometry showed an FEV1 of 2.1 L, an FVC of 3.6 L (predicted, 3.5 and 4.2 L, respectively). Fiberoptic bronchoscopy was normal; BAL showed no evidence of malignant tumor, and culture of BAL fluid revealed no pathologic organisms. The chest radiograph (Fig 1) showed cavitating nodules in the upper lobe of the left lung and lower lobe of the right lung. A CT scan of the thorax (Fig 2) showed bilateral multiple cavitating nodules of varying size and wall thickness in the lower, middle, and upper lobes of the right lung and in the upper and lower lobes of the left lung. There was no mediastinal adenopathy, pleural effusion, or evidence of interstitial lung disease. A CT-guided biopsy of the lung lesion was unsuccessful, and the patient then underwent a video-assisted thoracoscopic lung biopsy of the middle lobe of the right lung; the specimen removed through this procedure led to the diagnosis. What is the diagnosis?

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is superior to conventional transbronchial needle aspiration (cTBNA) in the staging of lung cancer. However, its efficiency in diagnosis of sarcoidosis when combined with endobronchial biopsy (EBB) and transbronchial lung biopsy (TBLB) has not been studied. This randomized controlled trial compares diagnostic yield of EBUS-TBNA vs cTBNA in combination with EBB and TBLB.Patients with clinical diagnosis of sarcoidosis were randomized 1:1 to EBUS-TBNA or cTBNA. All patients underwent TBLB and EBB. The primary outcome was detection of granulomas. The secondary end points were the individual and cumulative yields of various procedures, serious adverse events, and procedure time.Of the 130 patients, sarcoidosis was diagnosed in 117 (62 cTBNA, 55 EBUS-TBNA). The two groups were similar at baseline. Granulomas were demonstrated in 104 (53 cTBNA, 51 EBUS-TBNA) patients and were similar in two groups (85.5% vs 92.7%, P = .34). Individually, EBUS-TBNA had the highest yield (41 of 55, 74.5%), which was better than cTBNA (30 of 62, 48.4%, P = .004) or EBB (40 of 111, 36.3%, P.0001) but not TBLB (78 of 112, 69.6%, P = .54). Adding EBB/TBLB to cTBNA led to an increase in granuloma detection, whereas the addition of TBLB (but not EBB) significantly enhanced the yield of EBUS-TBNA. The procedure time was significantly longer with EBUS-TBNA. No major adverse events occurred.Individually, EBUS-TBNA has the highest diagnostic yield in sarcoidosis, but it should be combined with TBLB for the optimal yield. The diagnostic yield of cTBNA (plus EBB and TBLB) is similar to EBUS-TBNA plus TBLB.ClinicalTrials.gov; No.: NCT01908868; URL: www.clinicaltrials.gov.

The cause of pulmonary necrotizing granulomas is often unclear, even after histologic examination. Our aim was to determine the clinical significance of histologically unexplained necrotizing granulomas.Pulmonary necrotizing granulomas surgically resected at the Mayo Clinic (1994-2004) were retrieved and reviewed retrospectively. Cases in which a cause was evident at the time of initial histologic examination were excluded. The analysis cohort comprised 131 completely resected histologically unexplained pulmonary necrotizing granulomas. Clinical and laboratory information was abstracted from medical records, chest CT scans were reviewed, histologic slides were reexamined, and additional ancillary studies were performed in selected cases.A cause was determined on review in more than one-half of the histologically unexplained necrotizing granulomas (79 of 131, 60%) by reexamining histologic slides (47), incorporating the results of cultures (26), fungal serologies (14), and other laboratory studies (eight), and correlating histologic findings with clinical and radiologic information (13). Infections accounted for the majority (64 of 79), the most common being histoplasmosis (37) and nontuberculous mycobacterial infections (18). Noninfectious diagnoses (15 of 79) were rheumatoid nodule (five), granulomatosis with polyangiitis (Wegener) (five), sarcoidosis (four), and chronic granulomatous disease (one). Many cases remained unexplained even after extensive review (52 of 131, 40%). Most of these patients received no medical therapy and did not progress clinically or develop new nodules (median follow-up, 84 months).A cause, the most common being infection, can be established in many surgically resected pulmonary necrotizing granulomas that appear unexplained at the time of initial histologic diagnosis. Patients whose granulomas remain unexplained after a rigorous review have a favorable outcome. Most do not develop new nodules or progress clinically, even without medical therapy.

Background: Previous studies have demonstrated a relation between fungal exposure at home and the risk of sarcoidosis. Other studies have shown that β-glucan, a cell wall agent in fungi, was present in BALF of patients with sarcoidosis. At higher levels there was a suppression of iNKT-cells and IL-10 and a relation to the severity of granuloma infiltration. The present study was undertaken prospectively to investigate the presence of β-glucan in lymph nodes of patients with sarcoidosis. The amount was also measured in Kveim9s agent and in Propionibacterium acnes and Mycobacterium gordonii cultures. Material and methods: TBNA lymph nodeaspiration by flexible fiberoptic bronchoscopy in regions R4 or R7 was performed in 33 patients with newly diagnosed sarcoidosis, stages II and I. The samples were treated to retrieve the non-soluble part of β-glucan and analysed using a Limulus based assay. Different CFUs/mL of P.acnes and M.gordonii in one ml of saline solution, as well as samples of Kveim9s agent were also analysed. Results: There was a significant relationship between the amount of non-soluble β-glucan in lymph nodes and the extent of granuloma formation in lung parenchyma (r= 0.849; p P. acnes also contained different levels of β-glucan as did those of M. gordonii although at lower levels. Comments: The results support a conclusion that non-soluble β-glucan and thus a higher level of fungal exposure is involved in the pathogenesis of sarcoidosis through a depression of the normal immune defence system in susceptible patients.

Study design Between February 1, 1984, and February 1, 1989, fiberoptic bronchoscopy was performed on 2,692 patients, 592 of whom had bronchoalveolar lavage (BAL). One hundred twenty-eight patients with 16 percent or more lymphocytes in BAL fluid (BALF) were selected for further study. The group included 27 patients with sarcoidosis, 28 with nonsarcoidosis interstitial lung disease (ILD), 22 with lung infection (organism isolated), 31 with inflammation (presumed infection, no organism isolated), 14 with neoplasm, and 6 with bronchial hyperreactivity. Methods The percentages of lymphocytes, B lymphocytes, and T lymphocytes, the CD4/CD8 ratio and the percentages of neutrophils and eosinophils were analyzed individually and in combination for discrimination between the sarcoidosis and nonsarcoidosis patients and compared with the diagnostic accuracy of multiple noneaseating granuloma (MNG) on a simultaneous transbronchial biopsy (Tbbx). Results Neither the percentages of lymphocytes, T lymphocytes, or B lymphocytes discriminated sarcoidosis from nonsarcoidosis patients. Sarcoidosis patients had higher CD4/CD8 ratios, fewer neutrophils, and 1 percent or less eosinophils in the BAL cell populations. An analysis of CD4/ CD8 ratios, and percentages of neutrophils and eosinophils individually revealed that a CD4/CD8 ratio of 4:1 or greater had a positive predictive value of 94 percent in distinguishing sarcoidosis from other ELD but a sensitivity of only 59 percent. The positive predictive value of CD4/CD8 ratio of 4:1 or greater fell to 50 percent in separating sarcoidosis from all other diseases. A CD4/CD8 ratio of less than 1:1 has a 100 percent negative predictive value to exclude the diagnosis of sarcoidosis. Finding 1 percent or less neutrophils in BAL had an 80 percent positive predictive value in distinguishing sarcoidosis from nonsarcoidosis ILD and 51 percent for distinguishing sarcoidosis from all other disease groups. The CD4/CD8 ratio and the percentages of neutrophils and eosinophils also were combined and analyzed for the diagnosis of sarcoidosis. Conclusion Results showed a BALF with a CD4/CD8 ratio of 2:1 or greater, 1 percent or less neutrophils, and 1 percent or less eosinophils has essentially the same specificity and positive predictive value as MNG on Tbbx in distinguishing sarcoidosis from nonsarcoidosis disease. The combination of finding MNG in a Tbbx specimen plus a BALF CD4/ CD8 ratio of 4:1 or greater had a 100 percent positive predictive value in separating sarcoidosis from other ILD and an 81 percent value in separating sarcoidosis from all other disease. Finding MNG in a Tbbx specimen plus a BALF with a CD4/CD8 ratio of 2:1 or greater, 1 percent or less neutrophils, and 1 percent or less eosinophils had a 93 percent positive predictive value in distinguishing sarcoidosis from both nonsarcoidosis ILD and all other diseases.