Diagnosis can frequently be made based on history, physical findings, radiology and serologic reaction to suspected allergen, without biopsy ... Centrilobular accentuation of peribronchiolar fibrosis is very suggestive of hypersensitivity pneumonitis. 132, No. Context: - Chronic hypersensitivity pneumonitis (CHP) has emerged from obscurity during the past 15 years and is now recognized as a very common form of fibrosing interstitial pneumonia but one that is frequently misdiagnosed both clinically and on surgical lung biopsy as usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) or fibrotic nonspecific interstitial pneumonia. Hypersensitivity pneumonitis is an immunologically induced lung disease with features indicative of both immune complex–mediated and T-cell–mediated immune responses. Hypersensitivity pneumonitis is an immunologically mediated parenchymal lung disease which involves the alveolar walls and terminal bronchioles. Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia. Guidelines for the clinical evaluation of hypersensitivity pneumonitis Richerson HB, Bernstein IL, Fink JN, et al. 42 Scopus citations. Hypersensitivity pneumonitis is an important group of conditions in which the lung is sensitized by contact with a variety of agents and in which the response to reexposure consists of an acute pneumonitis, with inflammation of the smaller bronchioles, alveolar wall edema, and a… Hypersensitivity pneumonitis (HP) is a spectrum of immune-mediated disorders characterized by diffuse inflammation of interstitial lung, terminal bronchioli, and alveoli. 2Department of Pathology, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada V5Z 1M9. Animated Mnemonics (Picmonic): https://www.picmonic.com/viphookup/medicosis/ - With Picmonic, get your life back by studying less and remembering more. The clinical picture and pathology of hypersensitivity pneumonitis is the same regardless of the cause. The clinical picture and pathology of hypersensitivity pneumonitis is the same regardless of the cause. Treatment The most important thing you can do is avoid the dust that caused your hypersensitivity pneumonitis. Guidelines for the clinical evaluation of hypersensitivity pneumonitis. Hypersensitity pneumonitis, caused by inhalation of various antigens, is characterized by interstitial mononuclear cell infiltration, nonnecrotizing granulomas, cellular bronchiolitis, and fibrosis. Chronic hypersensitivity pneumonitis (CHP) has emerged from obscurity during the past 15 years and is now recognized as a very common form of fibrosing interstitial pneumonia but one that is frequently misdiagnosed both clinically and on surgical lung biopsy as usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) or fibrotic nonspecific interstitial pneumonia. STUDY. Hypersensitivity pneumonitis, aka allergic alveolitis, results from an … Get the latest public health information from CDC: https://www.coronavirus.gov, Get the latest research information from NIH: https://www.nih.gov/coronavirus, Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. Armando E. Fraire (2008) Hypersensitivity Pneumonitis: A Commentary.Archives of Pathology & Laboratory Medicine: February 2008, Vol. Hypersensitivity pneumonitis (HP) is a group of immunologically mediated lung diseases caused by the inhalation of environmental agents in susceptible individuals. 2015 Jun;110:45-53. doi: 10.1016/j.atmosenv.2015.03.039. This is caused by breathing in mould that grows on hay, straw and grain. Although much progress has been made in the understanding of these immune responses, there remains a number of unexplained features. Hypersensitivity Pneumonitis A Perspective From Members of the Pulmonary Pathology Society Archives of Pathology & Laboratory Medicine, Vol. RECENT FINDINGS: The pathological features of chronic hypersensitivity pneumonitis comprise overlapping usual interstitial pneumonia-like pattern with subpleural patchy fibrosis, alternating normal alveoli and fibroblastic foci, a nonspecific interstitial pneumonia-like pattern, and centrilobular fibrosis. —The classic histopathology of hypersensitivity pneumonitis (HSP) is well known but variations do occur and at times the diagnosis can be difficult. Autopsy cases of chronic hypersensitivity pneumonitis have demonstrated not only upper lobe contraction but also lower lobe contraction, mimicking usual interstitial pneumonia pattern and diffuse alveolar damage. Purpose: To retrospectively compare in patients with chronic hypersensitivity pneumonitis (HP) the computed tomographic (CT) imaging features suggestive of fibrosis with pathologic evidence of fibrosis at surgical lung biopsy and to compare a usual interstitial pneumonia (UIP) pattern at CT with survival. CONTEXT: - Hypersensitivity pneumonitis (HP) is a lung disease that develops in susceptible individuals after inhalational exposure to an organic antigen or chemical compound. CONCLUSION. male presents with dyspnea, cough, NO IgE and NO eosinophilia. It is triggered by exposure to avian proteins present in the dry dust of the droppings and sometimes in the feathers of a variety of birds. Sufferers are commonly exposed to … Hypersensitivity pneumonitis: radiology and pathology aspect 1. Epub 2018 Dec 18. 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). Hypersensitivity pneumonitis (HP) is a lung disease causing inflammation (swelling and sensitivity) of the lung tissue. 2009 Dec;33(12):1765-70. doi: 10.1097/PAS.0b013e3181bb2538. CTD-ILD. HP is caused by repeated inhalation of non-human protein, which can be of natural plant or animal origin or can be the result of a chemical conjugated to a human airway protein, such as albumin. It is a thingy one may get quizzed on. So, hypersensitivity pneumonitis is when a person’s immune system reacts excessively to something that’s inhaled, causing lung inflammation. The clinical, radiological, and pathological features of chronic hypersensitivity pneumonitis have recently been described. Hypersensitivity pneumonitis is a rare immune system disorder that affects the lungs. J Allergy Clin Immunol. Am J Clin Pathol. Chronic (fibrotic) hypersensitivity pneumonitis (HP) and fibrosing interstitial pneumonias associated with connective tissue disease (CTD-ILD) can be difficult to distinguish in biopsy specimens. NLM (Image courtesy of Rodney A. Schmidt, MD, Department of Pathology, University of … In Hypersensitivity pneumonitis. Am J Respir Crit Care Med. Chronic hypersensitivity pneumonitis (CHP) refers to hypersensitivity pneumonitis where there is radiological evidence of fibrosis and represents the end-stage of repeated or persistent pneumonitis 7. 2Department of Pathology, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada V5Z 1M9. The pathological features of chronic hypersensitivity pneumonitis comprise overlapping usual interstitial pneumonia-like pattern with subpleural patchy fibrosis, alternating normal alveoli and fibroblastic foci, a nonspecific interstitial pneumonia-like pattern, and centrilobular fibrosis. Hypersensitivity refers to an abnormal and excessive immune reaction, and pneumonitis refers to an inflammation of the lungs.. From Libre Pathology. Hypersensitivity pneumonitis (HP) happens if your lungs develop an immune response – hypersensitivity - to something you breathe in which results in inflammation of the lung tissue - pneumonitis. National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error. This study reviews the previously reported information and provides new insights into the pathological features of chronic hypersensitivity pneumonitis. The left photo shows a chronic granulomatous interstitial inflammation with giant cells. doi: 10.1164/rccm.202005-2032ST. Signs and symptoms. 2, pp. Follow Dr. Pernick's blog by clicking. Hypersensitivity pneumonitis is a rare disorder caused by an immune system response in the lungs after breathing in certain triggers. High-resolution CT plays an important role in the diagnosis of HP. Reprints: Andrew Churg, MD, Department of Pathology, Vancou-ver General Hospital, JPPN 1401, 910 W 10th Ave, Vancouver, BC V5Z 1M9, Canada (email: achurg@mail.ubc.ca). Epub 2015 Mar 20. It is caused by inhalation of organic dusts. STUDY. Pathology of hypersensitivity pneumonitis. Hypersensitivity pneumonitis is an inflammatory process involving the small airways and interstitium. CTD-ILD. Examples include farmer’s lung due to inhalation of Thermoactinomyces in moldy hay, bird-fancier’s or pigeon-breeder’s lung due to inhaled avian proteins, bagassosis from inhalation of T. vulgaris in moldy sugar cane, et al. This website is intended for pathologists and laboratory personnel but not for patients. Hanzawa S, Tateishi T, Takemura T, Okada Y, Yamada Y, Noda M, Miyazaki Y, Inase N. Intern Med. 2012 Dec;61(6):1026-35. doi: 10.1111/j.1365-2559.2012.04322.x. Hypersensitivity pneumonitis (HSP) is a poorly understood entity typically caused by exposure to an inciting antigen such as fungi, thermophilic bacteria or animal protein. Histopathologic analysis of sixteen autopsy cases of chronic hypersensitivity pneumonitis and comparison with idiopathic pulmonary fibrosis/usual interstitial pneumonia. Raghu G, Remy-Jardin M, Ryerson CJ, Myers JL, Kreuter M, Vasakova M, Bargagli E, Chung JH, Collins BF, Bendstrup E, Chami HA, Chua AT, Corte TJ, Dalphin JC, Danoff SK, Diaz-Mendoza J, Duggal A, Egashira R, Ewing T, Gulati M, Inoue Y, Jenkins AR, Johannson KA, Johkoh T, Tamae-Kakazu M, Kitaichi M, Knight SL, Koschel D, Lederer DJ, Mageto Y, Maier LA, Matiz C, Morell F, Nicholson AG, Patolia S, Pereira CA, Renzoni EA, Salisbury ML, Selman M, Walsh SLF, Wuyts WA, Wilson KC. This is particularly true in the chronic stage of the disease. Arch Pathol Lab Med. Recent findings: It is an inflammation of the alveoli (airspaces) within the lung caused by hypersensitivity to inhaled organic dusts. Hypersensitivity pneumonitis. This kind of hypersensitivity pneumonitis can lead to permanent lung scarring. The syndrome varies in intensity, clinical presentation, and natural history depending on the inciting agent, as well as the intensity of exposure. The present review focuses on the pathological features of chronic hypersensitivity pneumonitis and presents that centrilobular fibrosis and bridging fibrosis are the important hallmarks of chronic hypersensitivity pneumonitis, even with a usual interstitial pneumonia-like pattern. HHS Airway-centered interstitial fibrosis - an under-recognized subtype of diffuse parenchymal lung diseases. 1 A number of offending agents have been identified , many of which are associated with a name referring to the resulting clinicopathologic condition (ie, farmer's lung, hot tub lung, byssinosis, etc). Overview; Fingerprint; Abstract. Learn more about causes, risk factors, prevention, signs and symptoms, complications, diagnosis, and treatments for hypersensitivity pneumonitis, … Hypersensitivity pneumonitis. According to the causative antigen, HP may have different names: Disease mechanism may not be immune mediated, endotoxin from bacterial contamination of cotton may play a role, HP is classified into acute, subacute and chronic, however there is a lack of consensus for the criteria, Influenza-like syndrome a few hours after exposure to an antigen: fever, dyspnea, cough, crackles may be detected on chest auscultation, Symptoms resolve several hours after antigen removal, Repeated acute episodes of farmer’s lung leads to centriacinar emphysema (, Slowly progressive respiratory failure over weeks to months, Fever, dyspnea, cough, fatigue, crackles may be detected on chest auscultation, Probably results from continuous low level exposure to the antigen, Slowly progressive and insidious respiratory failure; often without acute episodes, Dyspnea, cough, fatigue, weight loss, fine crackles on chest auscultation, Restrictive pattern on pulmonary function tests, Decreased diffusing capacity of the lung for carbon monoxide (DLCO), Often associated with bird antigen exposure, Acute exacerbation followed by respiratory deterioration within 1 - 2 months can occur; usually without further antigen exposure (, Diagnosis is based on clinical, radiological (high resolution computed tomography, HRCT) and pathological examination, Surgical lung biopsy is often necessary to differentiate subacute and chronic hypersensitivity pneumonitis from other interstitial lung disease; however, it is rare for acute hypersensitivity pneumonitis to be biopsied, Although several diagnostic criteria have been proposed, none are widely accepted, A large cohort study by HP Study Group suggested clinical predictors for the diagnosis of HP (, Bronchoalveolar lavage is supportive in the diagnosis but lacks standardization (, Increased lymphocyte percentage ≥ 30% for nonsmokers or exsmokers or ≥ 20% for current smokers; a normal lavage rules out the presence of active HP (, CD4 / CD8 ratiois usually decreased in HP but can be increased as high as in sarcoidosis; CD4 / CD8 ratio is now considered to vary by clinical conditions such as causative antigen and smoking status, Inhalation challenge is supportive but lacks standardization (, Antigen exposure at the workplace or home or direct inhalation of the specific antigen after a period of avoidance provokes symptoms of HP and decreases FVC in 8 - 12 hours, The patient should be monitored at least for 24 hours after the inhalation in case of severe attack of HP, Serum IgG antibody to causative antigens may be increased; however, serum antibody could be positive in 31% of non HP subjects (, Avian antigens: pigeon, parakeet, budgerigar, chicken, Increased serum KL-6, often over 1000 IU (normal limit is < 500 IU), Findings on the chest X-ray vary from nonspecific change, especially in acute and subacute hypersensitivity pneumonitis, to upper lobe predominant fibrosis of chronic HP, Typically, HRCT shows ground glass opacity and centrilobular nodular opacity with / without emphysema and fibrosis, It is often challenging to differential HP from other interstitial lung diseases on HRCT such as, HRCT features to differentiate chronic HP from NSIP and UIP (, Lobular areas with decreased attenuation and vascularity, Absence of lower zone predominance of abnormalities, Many patients show favorable prognosis compared to other interstitial lung diseases and improve with appropriate treatment (, Interstitial fibrosis and emphysema are associated with worse prognosis, and once developed, may remain or progress despite treatment (, Smoking is also associated with worse prognosis (, 12 year old boy with HP due to feather duvet (, 37 year old man with HP due to shiitake mushroom spores (, 45 year old man with HP due to metalworking fluid (, 45 year old man with pneumomediastinum as a primary manifestation of chronic HP (, 72 year old woman with HP and combined pulmonary fibrosis and emphysema (, Avoidance of antigen is the key of HP management, Oral or systemic corticosteroids are considered for severe case or when the antigen is not removable; however, steroids do not change long term outcome and are not standardized (, Diffuse involvement with mild to moderate increase in lung weight, Bronchocentric fibrotic changes may be seen, Poorly formed nonnecrotizing granulomas or interstitial giant cells with cholesterol clefts, Well formed granulomas can be found but may raise differential diagnosis with sarcoidosis if the granulomas are numerous and predominant, Airway centered inflammation with little fibrosis, Neutrophilic infiltration with / without capillaritis, Airway centered infiltration with fibrosis, Lymphocytic infiltration with granulomas or giant cells, Predominantly airway centered inflammation with diffuse fibrotic change, Bridging fibrosis (fibrotic band connecting bronchioles with each other and with lobular septa) and peribronchiolar metaplasia can be a diagnostic clue to differentiate HP from IPF (, Byssinosis bodies (hemosiderin coated strands of fiber within fibrous tissue) can be found in byssinosis, Bronchoalveolar lavage fluid shows lymphocytosis (see, Intra-alveolar fibrin deposition is suggestive for acute HP, also for acute fibrinous and organizing pneumonia, Typical bronchoalveolar lavage of HP shows lymphocytosis, Necrotizing granuloma is more suggestive for tuberculosis, Organizing pneumonia can be seen in HP but it is not specific, Weight loss is suggestive for HP according to the large cohort study (see. It is caused by inhalation of organic dusts. Hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis (EAA), represents a group of pulmonary disorders mediated by an inflammatory reaction to inhalation of an allergen that can lead to lung fibrosis. Acute hypersensitivity pneumonitis. In his immunopathology article, Dr Woda examines the pathogenesis of the … Exposure to stuffs... e.g. Micrograph of hypersensitivity pneumonitis, the histologic correlate of bird fancier's lung. NSIP pattern is also common in hypersensitivity pneumonitis. However, we cannot answer medical or research questions or give advice. Trichrome stain. Arch Pathol Lab Med—Vol 142, January 2018 Chronic Hypersensitivity Pneumonitis—Churg et al 109 This is particularly true in the chronic stage of the disease.  |  2020 Aug 1;202(3):e36-e69. Atmos Environ (1994). 2018;35(3):218-229. doi: 10.36141/svdld.v35i3.6432. IPAF. Acta Biomed. 2019 Apr 15;58(8):1145-1150. doi: 10.2169/internalmedicine.1142-18. Pathology of hypersensitivity pneumonitis. Chronic HP was previously considered a rare interstitial lung disease (ILD), but it has emerged as a common and confusing entity over the last two decades. Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease-Associated Interstitial Lung Disease. There are a variety of things that can cause hypersensitivity pneumonitis when you breathe them in, including fungus, molds, bacteria, proteins, and chemicals. This case meets the classification criteria for rheumatoid arthritis (Ann Rheum Dis 2010;62:2569). Allergens are substances that cause an allergic reaction in the body. Hypersensitivity pneumonitis (HP) is a group of immunologically mediated lung diseases caused by the inhalation of environmental agents in susceptible individuals. Epub 2012 Aug 8. © Copyright PathologyOutlines.com, Inc. Click, Hypersensitivity pneumonitis [title] or Extrinsic allergic alveolitis [title], J Investig Allergol Clin Immunol 2015;25:237, nonspecific interstitial pneumonia (NSIP), Connective tissue disease related interstitial pneumonia, Idiopathic nonspecific interstitial pneumonia, Respiratory bronchiolitis related interstitial lung disease, Usual interstitial pneumonia / idiopathic pulmonary fibrosis, Clin Med Insights Circ Respir Pulm Med 2016;9:123, Leslie: Practical Pulmonary Pathology - A Diagnostic Approach, Second Edition, 2011, Cagle: Lung and Pleural Pathology, First Edition, 2015, Also known as extrinsic allergic alveolitis, A complex health syndrome of varying intensity, clinical presentation and natural history, Due to immunologically induced inflammation of lung parenchyma in response to inhalation of a large variety of antigens (, An interstitial pneumonia with acute to chronic respiratory failure caused by inhalation exposure to a variety of natural or chemical antigens, Histologically characterized by airway centered inflammation with fibrosis and poorly formed nonnecrotizing granulomas, Also called extrinsic allergic alveolitis, Prevalence and incidence vary by climate, geographical condition, living environment and agricultural practice, UK incidence is 0.9 per 100,000 person per year (, Higher prevalence and mortality rate in farmers and agricultural industries (, Smoking is related to lower prevalence but worse prognosis (, Predominant in middle to upper lobes of the lung; usually bilateral, Gene polymorphisms related to the acquired immune response may predispose to HP (, Major histocompatibility complex class 2 (MHC class II), Transporter associated with Antigen Processing 1 (TAP1), Antigen exposure results in early formation of type III immune complexes, followed by type IV delayed hypersensitivity, HP is developed through inhalation and exposure to a causative antigen. Hypersensitivity pneumonitis is an inflammatory process involving the small airways and interstitium. This inflammation makes breathing difficult. The pathological features of chronic hypersensitivity pneumonitis comprise overlapping usual interstitial pneumonia-like pattern with subpleural patchy fibrosis, alternating normal alveoli and fibroblastic foci, a nonspecific interstitial pneumonia-like pattern, and centrilobular fibrosis. Clinically, HSP is often divided into acute, subacute and chronic forms. Jump to navigation Jump to search. Hypersensitivity pneumonitis (HP) or extrinsic allergic alveolitis (EAA) is a rare immune system disorder that affects the lungs. The pathological picture of chronic hypersensitivity pneumonitis is, however, complicated; it is sometimes difficult to differentiate chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia, nonspecific interstitial pneumonia, and connective-tissue-related lung disease. Would you like email updates of new search results? Hypersensitivity pneumonia and HIV infection in occupational settings: a case report from northern Italy. Findings on the chest X-ray vary from nonspecific change, especially in acute and subacute hypersensitivity pneumonitis, to upper lobe predominant fibrosis of chronic HP Typically, HRCT shows ground glass opacity and centrilobular nodular opacity with / without emphysema and fibrosis Pathology of Hypersensitivity Pneumonitis. The biopsy shows a typical, fibrotic NSIP pattern. The triggering particles are usually in the range of 1-5 micrometers in size 5. It can lead to irreversible lung scarring over time. Sarcoidosis In all cases, infectious organisms, especially fungi and mycobacteria must be ruled out Areas of chronic hypersensitivity pneumonitis may be identical to UIP Identification of any of these features should prompt efforts to identify an allergen Laboratory Medicine and Pathology; Research output: Contribution to journal › Review article › peer-review. caused by intense prolonged exposure to inhaled inorganic dusts and occupational antigens; NO IgE or eosinophilia. Diagnosis of Hypersensitivity Pneumonitis in Adults. It used to be called extrinsic allergic alveolitis (EAA). 2,3 Hypersensitivity pneumonitis is often divided into acute, subacute, and chronic forms. We welcome suggestions or questions about using the website. Hypersensitivity pneumonitis is reviewed with regard to causative agents, occupations, industries, and workers at risk, epidemiology, pathology, clinical description and diagnostic criteria, methods of prevention, and research needs. 192-194.  |  Hypersensitivity pneumonitis (HSP) is a poorly understood entity typically caused by exposure to an inciting antigen such as fungi, thermophilic bacteria or animal protein. Gell & Coombs classification. Figure 1 Photomicrograph shows the two most common and most characteristic histopathologic features of hypersensitivity pneumonitis: lymphocytic infiltrates within the interstitium, sometimes referred to as cellular interstitial pneumonitis (arrowheads), and a poorly formed granuloma (arrow). Please enable it to take advantage of the complete set of features! Bridging fibrosis between peribronchiolar area and perilobular areas is an outstanding feature of chronic hypersensitivity pneumonitis. caused by intense prolonged exposure to inhaled inorganic dusts and occupational antigens; NO IgE or eosinophilia. Takemura T(1), Akashi T, Ohtani Y, Inase N, Yoshizawa Y. Pathology of hypersensitivity pneumonitis c. Introduction. Purpose of review: NSIP pattern is also common in hypersensitivity pneumonitis. Hypersensitivity pneumonitis, also known as extrinsic allergic alveolitis, is an immunologically mediated dis- ease caused by inhalation of various antigens containing a variety of organic dusts and chemicals [1â 5].Farmer's lung is the classical and most studied example of hypersensitivity pneumonitis [6â 10]. It occurs in some people after they breathe in certain substances they encounter in the environment. —The classic histopathology of hypersensitivity pneumonitis (HSP) is well known but variations do occur and at times the diagnosis can be difficult. These substances trigger their immune systems, causing short- or long-term inflammation, especially in a part of the lungs called the interstitium. Spectrum of fibrosing diffuse parenchymal lung disease. Hypersensitivity pneumonitis (HP) is a spectrum of immune-mediated disorders characterized by diffuse inflammation of interstitial lung, terminal bronchioli, and alveoli. Purpose: To retrospectively compare in patients with chronic hypersensitivity pneumonitis (HP) the computed tomographic (CT) imaging features suggestive of fibrosis with pathologic evidence of fibrosis at surgical lung biopsy and to compare a usual interstitial pneumonia (UIP) pattern at CT with survival. A Schaumann body, which is a marker of a previously present granuloma, is illustrated in the inset (hematoxylin-eosin, original magnifications ×25 and ×200 [inset]) PRESENTED IN RADIOLOGICAL-PATHOLOGICAL CONFERENCE 14 JULY 2015 BY T.CHAYOVAN Hypersensitivity Pneumonitis: Essential Radiologic and Pathologic Findings Teri J. Franks, MD, Jeffrey R. Galvin, MD Surgical Pathology Clinics Volume 3, Issue 1, Pages 187-198 (March 2010) DOI: … moldy hay - Farmer's lung, atypical mycobacteria - hot tub lung. The pathological picture of chronic hypersensitivity pneumonitis is, however, complicated; it is sometimes difficult to differentiate chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia, nonspecific interstitial pneumonia, and connective-tissue-related lung disease. Hypersensitivity pneumonitis (HP) or extrinsic allergic alveolitis (EAA) is a rare immune system disorder that affects the lungs. Lung biopsy. 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