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Idiopathic Bronchiolocentric Interstitial Pneumonia Samuel A Idiopathic Bronchiolocentric Interstitial Pneumonia Samuel A. Yousem, MD, Sanja Dacic, MD, PhD Department of Pathology, University of Pittsburgh Medical Center, Presbyterian University Hospital, Pittsburgh, Pennsylvania plugs of fibromyxoid connective tissue, and poorly The authors report 10 patients with a distinctive formed granulomas (1–3). This triad of morphologic idiopathic bronchiolocentric interstitial pneumonia changes allows the pathologist to direct the pulmo- having some histologic similarities to hypersensitiv- nologist to question the patient for specific inhala- ity pneumonitis. Bronchiolocentric interstitial tional exposures. We have accumulated 10 cases pneumonia has a marked predilection for women that have very similar morphologic findings to hy- (80%) and occurs in middle age (40–50 years). Chest persensitivity pneumonitis, with the exclusion of radiographs and pulmonary function tests show in- interstitial granulomas, in which extensive investi- terstitial and restrictive lung disease, while the his- gations failed to reveal a cause for the inflamma- tologic appearance is that of a centrilobular inflam- tion. The clinicopathologic features of this idio- matory process with small airway fibrosis and pathic bronchiolocentric interstitial pneumonia are inflammation that radiates into the interstitium of the basis of this report which suggests a more ag- the distal acinus in a patchy fashion. Granulomas gressive and life threatening biologic behavior for are not identified. At a mean followup of approxi- bronchiolocentric interstitial pneumonia than nor- mately 4 years in nine patients, 33% of patients mally associated with hypersensitivity pneumonitis were dead of disease and 56% had persistent or or some of the other conditions in the histologic progressive disease suggesting a more aggressive differential diagnosis. course than hypersensitivity pneumonitis and non- specific interstitial pneumonia, the two major dis- ease processes in the differential diagnosis. Whether MATERIALS AND METHODS Bronchiolocentric interstitial pneumonia is a unique entity or not, the pattern of bronchiocentric The surgical pathology files of the Department of injury to the lung in the absence of known causes Pathology of the University of Pittsburgh Medical and its clinical presentation as interstitial lung dis- Center–Presbyterian University Hospital were ease, warrants further investigation of this unusual searched for all cases of unclassified interstitial interstitial process. pneumonia or cases “suggestive of” hypersensitiv- ity pneumonia, diagnosed by open lung biopsy KEY WORDS: Bronchiolitis obliterans, Hypersensi- from 1987–2002. Twenty-three cases were charac- tivity pneumonitis, Nonspecific interstitial pneumo- terized by the presence of a bronchiolocentric in- nia, Organizing pneumonia, Respiratory bronchi- terstitial pneumonia lacking interstitial epithelioid olitis, Usual interstitial pneumonia. cell aggregates, interstitial giant cells, or poorly Mod Pathol 2002;15(11):1148–1153 formed non-necrotizing granulomas. Eight cases occurring in patients with known autoimmune dis- Hypersensitivity pneumonitis is a prototype for ease (five with rheumatoid arthritis, two with bronchiolocentric chronic interstitial lung disease Sjögren’s syndrome, one with scleroderma) were with a distinctive histology that is characterized by excluded because their pulmonary disease was a lymphocytic bronchiolitis, a patchy interstitial al- most likely related to their underlying rheumato- veolitis with occasional interstitial and airspace logic condition. Four cases were eliminated be- cause they were likely to be drug-induced (three Copyright © 2002 by The United States and Canadian Academy of methotrexate, one amiodarone) and one patient Pathology, Inc. had a history of hard metal exposure. VOL. 15, NO. 11, P. 1148, 2002 Printed in the U.S.A. Date of acceptance: August 5, 2002. The remaining 10 patients had their medical Address reprint requests to: Samuel A. Yousem, Department of Pathology, records extensively evaluated for allergies and for University of Pittsburgh Medical Center, Presbyterian University Hospital, Room A610, 200 Lothrop Street, Pittsburgh, PA 15213-2582; e-mail: known causes of hypersensitivity pneumonitis. [email protected]; fax: 412-647-3399. Questionnaires were completed by referring pul- DOI: 10.1097/01.MP.0000037309.04985.B4 monologists, and/or patients which specifically 1148 asked about exposure to birds, window air condi- In nine patients, symptoms developed insidiously tioners, noxious gases, or dusty environments, in- with only one case (Patient 3) having a more acute cluding work on farms. Each pulmonologist indi- course (5 months). cated that, as best as possible, they could Chest radiographs showed bibasilar interstitial “confidently exclude the possibility of hypersensi- infiltrates in nine patients with one patient having tivity pneumonitis from your clinical differential normal chest radiographs. Two patients had alveo- diagnosis.” Included in this examination were spe- lar infiltrates superimposed on the underlying re- cific questions with regard to medications with ticular and reticulonodular interstitial disease. known pulmonary toxicity and other medical and Honeycomb lung was not reported in any case. pulmonary conditions that could cause respiratory High resolution CT scans or their reports were disease, e.g., bronchiectasis, asthma, aspiration, available for review in only six cases and confirmed cigarette smoking. the above findings, and excluded large and small Pathologic features that were assessed included the airway disease. Pulmonary function tests showed following: distribution of the inflammatory infiltrate, restrictive lung disease with reduced lung volumes presence of a patchy interstitial pneumonitis, acute (total lung capacity less than 80% of predicted for bronchiolitis, intraluminal fibromyxoid connective age) and abnormalities of carbon monoxide diffus- tissue plugs, peribronchiolar scarring, muscular hy- ing capacity (less than 80% of predicted) in six perplasia of the small airways, peribronchiolar fibro- cases. No patient had significant airflow obstruc- sis, air space organization (“organizing pneumonia”), tion despite a history of current or previous ciga- bronchiolar metaplasia, desquamative interstitial rette smoking in four patients. Two patients did pneumonia-like reactions, lymphoid aggregates, have a previous diagnosis of gastroesophageal re- mucostasis, presence of eosinophils and vascular flux disease. One patient had history of a skin bi- atherosclerosis. As noted previously, the presence opsy showing “morphea” but lacked the diagnosis of interstitial epithelioid histiocytes, giant cells, and of systemic sclerosis. All 10 patients had negative granulomas were exclusionary. antinuclear antibody studies; four had negative rheumatoid factor. No patient had a history of in- flammatory bowel disease. RESULTS Followup information was available in nine pa- Ten patients comprised the study group (Table tients and ranged from 4–108 months (mean 48.2 1). Eight were women (80%), two were men (20%); months). All nine patients received steroid therapy seven were Caucasian and three were African- (dosage range 30–60 mg/day) and two received Americans. Ages at the time of biopsy ranged from Cyclophosphamide (2 mg/kg/day). Three patients 28–69 years with a mean of 46.7 years and a median died of pulmonary disease (48, 60, 96 months), five of 44.0 years. Patients presented with a history of patients were alive with persistent or progressive respiratory complaints that ranged from five to 24 pulmonary disease and one patient had no evi- months (mean 10.0 months; median 13 months). dence of disease at 84 months followup. The most common complaints were shortness of breath (eight) and dry cough (five) although wheez- ing, chest pain, and dyspnea on exertion (two each) Histopathology were also noted. Sputum production and a history The most striking feature of these 10 cases was of “recurrent pneumonia” was noted in one patient. the centrilobular and bronchiolocentric nature of TABLE 1. Clinical Data—Idiopathic Bronchiolocentric Interstitial Pneumonia Presenting Signs and Smoking Case Number Age/Race/Sex Chest Radiographs Status Symptoms History 1 28/W/F SOB, cough Interstitial infiltrates Ϫ DOD, 48 months 2 49/W/F Cough, wheeze, Interstitial infiltrates ϩ AWSD, 108 months chest pain 3 41/B/M SOB, chest pain Interstitial infiltrates Ϫ NED, 84 months 4 65/W/F SOB Normal Ϫ DOD, 60 months 5 36/W/F SOB, DOE Interstitial infiltrates ϩ AWPD, 13 months 6 53/W/F Cough, recurrent Interstitial and alveolar Ϫ AWSD, 4 months pneumonia infiltrates 7 46/B/F SOB, cough Interstitial infiltrates ϩ Lost to F/U 8 69/W/F SOB Interstitial infiltrates Ϫ DOD, 96 months 9 41/W/M SOB, DOE, cough, Interstitial infiltrates with ϩ AWPD, 9 months wheeze focal ground glass change 10 39/B/F SOB, cough Interstitial infiltrates Ϫ AWPD, 12 months W, Caucasian; B, African-American; M, male; F, female; SOB, shortness of breath; DOE, dyspnea on exertion; ϩ, positive; Ϫ, negative; NED, no evidence of disease; DOD, dead of disease; AWDS, alive with stable disease; AWPD, alive with progressive disease. Bronchiolocentric Interstitial Pneumonia (S.A. Yousem and S. Dacic) 1149 the chronic inflammatory cell infiltrate (Fig. 1). At low magnification, the small airways were cuffed by an irregular band of lymphocytes and plasma cells (“mild intensity” in eight cases and “marked inten- sity” in two) with the infiltrate extending
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