BAL lymphocytosis is an important feature (1, 7); serum-specific immunoglobulins might also be helpful (1, 155, 156). Prevalence of HP is highest among older individuals (i.e., 65 yr and older, with the average patient receiving a diagnosis in their fifth or sixth decade) (34). (A) Scanning magnification view showing multiple sections of a right-lower-lobe biopsy specimen. No CPG or recommendation can consider all potential clinical circumstances. An estimated 85 to 95 percent of people exposed to causative substances either never develop hypersensitivity pneumonitis or they experience a mild immune reaction with no obvious signs or symptoms or disease. 2. It also emphasizes the difficulty in making a definitive diagnosis of HP (particularly fibrotic/chronic HP), which is the reason that a diagnosis of HP requires a multidisciplinary approach that includes radiologists and pathologists. Clinical manifestations of hypersensitivity pneumonitis may closely mimic other interstitial lung diseases, and the disease onset is usually insidious. In the only study that enrolled patients with known or suspected HP, bleeding of any severity occurred in 4% (95% CI, 0–10%) and pneumothoraces occurred in 27% (95% CI, 14–40%). Voting results: recommendation for, 10; suggestion for, 18; no recommendation or suggestion, 0; suggestion against, 3; recommendation against, 0. Although the specific features that satisfy each domain are different for fibrotic and nonfibrotic HP, a single approach is used for all patients who have a clinical presentation consistent with HP. Serum IgG testing against potential antigens associated with HP distinguished patients with HP from exposed control subjects with a sensitivity and specificity of 90% and 91%, respectively, derived by pooling eight studies. For nonfibrotic HP, we use consensus descriptions of the inflammatory and often reversible changes established in the literature (116–119). While some signs and symptoms occur in several types of hypersensitivity pneumonitis, they may vary in severity. Question 1: Should patients with newly detected ILD on chest radiographs or a CT scan of the chest, with or without an overt history of exposures capable of causing ILD in the patient’s environment at home, work, or elsewhere, be subjected to formal questioning using a questionnaire to raise the possibility that a) potential inciting agents of HP are the etiology of the ILD and b) the diagnosis of the ILD is HP? Analysis of lung changes at expiration may increase diagnostic confidence in nonfibrotic HP and is necessary for better characterization of heterogeneous lung attenuation in both forms of the disease. On the basis of evolving knowledge and clinical experience, the guideline committee concluded that patients should be classified as having fibrotic HP or nonfibrotic HP, as determined by the predominant presence or absence of radiological and/or histopathological fibrosis. Probable HP refers to cases in which only some of the features described above are present. During the physical exam, your doctor will use a stethoscope to listen carefully to your lungs while you breathe. It is the right course of action for >95% of patients. One study enrolled 19 patients with definite or probable HP and used clinical history, a 22-item questionnaire, and serum IgG testing against HP-associated antigens to identify potential inciting agents of HP. Hematoxylin and eosin staining was used. Inclusion of TBBx in the multimodality diagnostic approach of patients with ILD increased the likelihood of arriving at a diagnosis compared with an approach without TBBx (RR, 1.67; 95% CI, 1.21–2.30). A.G.N. served on an advisory committee for Chiesi, Novartis, and Teva; served as a speaker for Boehringer Ingelheim and Menarini; received research support from Asten France and SOS Oxygene; and received other transfers of value from GlaxoSmithKline, Novartis, and Roche. Question 2: Should patients with newly detected ILD on chest radiographs or a CT scan of the chest, with or without an overt history of exposures capable of causing ILD in the patient’s environment at home, work, or elsewhere, undergo serum testing for IgG antibodies against specific antigens to raise the possibility that a) potential inciting agents of HP are the etiology of the ILD and b) the diagnosis of the ILD is HP? (D) High-magnification photomicrograph showing one of the isolated Schaumann bodies illustrated in C. Magnification, 400×. The patient representative indicated that he would choose a TBBx in the hope of avoiding SLB, even with the knowledge that a second procedure would be necessary if the TBBx were nondiagnostic. Questions about disease behavior range from prevention to the phenotyping of HP. You may print one copy of this document at no charge. Hypersensitivity pneumonitis (HP) is categorized as acute, subacute, and chronic based on … If you have been diagnosed with subacute or chronic hypersensitivity pneumonitis, your doctor may recommend follow-up testing to see how well your treatment is working and if your disease is improving, stable, or worse. It should be emphasized that clinicians should apply the recommendations within this CPG in the clinical context of each individual patient, considering the patient’s values and preferences, and should not consider any recommendations as mandates. If your doctor is able to identify the environmental substance that causes your hypersensitivity pneumonitis, he or she will recommend that you adopt the following avoidance strategies. This occurs most frequently in the distinction between fibrotic HP and IPF (12), reflecting the overlapping features and lack of a single, definitive gold-standard test for both diagnoses. §Updates to the classification of IIPs by Travis and colleagues (330) and diagnostic guidelines for idiopathic pulmonary fibrosis (20, 128) tightly link a UIP pattern with idiopathic pulmonary fibrosis and an NSIP pattern with idiopathic NSIP. C.M. Nonspecific markers of … The overwhelming majority of individuals in this situation would want the recommended course of action and only a small minority would not. Sources of Antigens Known to Cause HP. The antigens to which serum IgG were measured varied from study to study. 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Childhood HP is uncommon but may represent 50% of all childhood ILDs (68–70). 2. Bronchiolar obstruction is also present in an HRCT pattern combining three different lung densities (GGO, lobules of decreased attenuation and vascularity, and normal-appearing lung) that is highly specific to fibrotic HP (123). 1. Fibrotic HP differs from nonfibrotic HP in that the underlying chronic interstitial pneumonia and/or bronchiolitis is complicated by fibrosis. If you have hypersensitivity pneumonitis, your body’s immune system reacts strongly to certain inhaled substances, causing inflammation especially in the interstitium or interstitial space. • Combination of areas of low and high attenuation that can correspond to two main situations: a. (E) Photomicrograph from another section illustrated in A showing an isolated Schaumann body (arrow) in the fibrotic peribronchiolar interstitium. CT angiography should be preceded by a noncontrast chest HRCT scan to detect new ground-glass changes that raise the probability of acute exacerbation in the absence of pulmonary embolisms. Your doctor will need to rule out other causes including infection and order repeat chest imaging tests. 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Poorly formed nonnecrotizing granulomas, • Loose clusters of epithelioid cells and/or multinucleated giant cells ± intracytoplasmic inclusions, • Situated in peribronchiolar interstitium, terminal air spaces, and/or organizing pneumonia (Masson bodies). Magnification, 88×. served as a consultant for eResearch Technology and MedQIA; and served as a speaker for Boehringer Ingelheim. It typically results from an immune-mediated reaction provoked by an overt or occult inhaled antigen in susceptible individuals. (C) Higher-magnification view showing expansion of the peribronchiolar interstitium by a cellular infiltrate of mononuclear inflammatory cells (upper left) and isolated Schaumann bodies (arrows) at the edge of the biopsy specimen. Other features are less frequently identified (e.g., episodes of symptoms and symptoms 4–8 h after exposure) (12), likely reflecting variable proportions of fibrotic and nonfibrotic HP in previous studies. American Thoracic Society; European Respiratory Society; World Association of Sarcoidosis and Other Granulomatous Disorders. This combination of findings is often present in a single biopsy specimen. HP shares features of other acute and chronic pulmonary diseases; as a result, fibrotic/chronic HP can be misdiagnosed as idiopathic pulmonary fibrosis (IPF) or another idiopathic interstitial pneumonia (IIP) (12). Additional features may be useful in the context of an MDD to increase or decrease the diagnostic confidence of HP on a case-by-case basis, but these are not sufficiently sensitive or specific to justify inclusion in formal diagnostic criteria. A multidisciplinary committee of international experts have released a new clinical practice guideline on the diagnosis of adult hypersensitivity pneumonitis (HP), an immune-mediated inflammatory lung and small airway disease. It was approached in a consensus fashion and informed by a nonsystematic review of the literature. Pathology of chronic hypersensitivity pneumonitis what is it? Learn more about the immune system of the lungs and how it contributes to hypersensitivity pneumonitis. Hypersensitivity pneumonitis diagnosis based on incorporation of imaging, exposure assessment, BAL lymphocytosis, and histopathological findings. Serum IgG testing against potential antigens associated with HP distinguished patients with HP from unexposed control subjects with a sensitivity and specificity of 93% and 100%, respectively, derived by pooling seven studies. The location of exposure can be occupational, household related, or recreational. If your condition is not diagnosed or well controlled by treatment, it can lead to irreversible lung damage and other potentially fatal complications. Due to a variable radiographic presentation, it may not be meaningful to give a differential diagnosis for hypersensitivity pneumonitis per se. 1. M.L.S. Thus, making a definitive or highly confident diagnosis is usually beneficial to patients. The inflammatory infiltrate is typically polymorphic in that it includes smaller numbers of plasma cells and occasionally eosinophils in some patients. Chest x-ray, HRCT, and pulmonary function tests are done routinely. Our TOPMed program has the potential to address recommendations made at our 2013 Prevention of Chronic Lung Diseases meeting and that remain relevant to hypersensitivity pneumonitis research. suggests performing serum IgG testing that targets potential antigens associated with HP (suggestion, very low confidence in the estimated effects). This inflammation makes breathing difficult. Three additional HRCT features have also been described in nonfibrotic HP: uniform and subtle GGO, airspace consolidation, and lung cysts (124–126). If avoidance strategies do not work for your condition, your doctor may prescribe corticosteroids or other immunosuppressive medicines to treat your condition. The evidence was discussed, and then recommendations were formulated by a multidisciplinary committee of experts in the field of interstitial lung disease and HP using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach. It was informed by National Academy of Medicine–adherent guideline methodology, including a full systematic review for each question and the formulation, writing, and grading of recommendations using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach. Voting results: recommendation for, 1; suggestion for, 13; no recommendation or suggestion, 6; suggestion against, 7; recommendation against, 1. The guideline committee recognized an urgent need to improve knowledge on several topics, including 1) understanding the nature and pathophysiology of HP, 2) diagnostic approaches, 3) disease behavior and natural history, and 4) therapeutic approaches. Among the studies that enrolled patients with ILD or DLD, two studies reported the pneumothorax rate (7%; 95% CI, 1–13%), two studies reported periprocedural mortality (no cases; one study monitored for 24 h and the other monitored for 6 mo), two studies reported respiratory exacerbation or failure (no cases), four studies reported moderate-to-severe bleeding (4%; 95% CI, 0–8%), and six studies reported severe bleeding (no cases). Definition of abbreviations: CT = computed tomography; GGO = ground-glass opacity; HP = hypersensitivity pneumonitis. The diagnostic yield could not be calculated for patients with known HP, but 41% (95% CI, 25–56%) of such patients who underwent TBBx were confirmed to have HP. Table 5. Decision aids may be useful to help individuals make decisions consistent with their values and preferences. Definition of abbreviations: HFC-134a = hydrofluorocarbon 134a; HP = hypersensitivity pneumonitis; MIC = methylisocyanate; NDI = naphtylene-1,5-diisocyanate; TMI = trimethylindium. Most committee members considered testing convenient and adequate for generating supportive data; however, they acknowledged that testing is insufficient for confirming or excluding a diagnosis of HP because the test characteristics are inferior to most screening tests currently in use. Depending on your condition, your doctor also may prescribe some of the following supportive therapies. The approach should be reevaluated as new evidence accumulates. HP results from breathing in specific environmental allergens. Two important things to know: Treatment is more successful when hypersensitivity pneumonitis is diagnosed in the early stages of the disease, before permanent irreversible lung damage has occurred. *Exposure assessment includes a thorough clinical history and/or serum IgG testing against potential antigens associated with HP and/or, in centers with the appropriate expertise and experience, specific inhalational challenge testing as described in References 9, 323, 324, and 325. 5. Reconstruction of thin-section CT images (≤1.5 mm): • Using a high-spatial-frequency algorithm, • Iterative reconstruction algorithm if validated on the CT unit (if not, filtered back projection), • Supine position: inspiratory (volumetric) and expiratory (sequential or volumetric) acquisitions, • Prone (optional): only inspiratory scans (can be sequential or volumetric), • Inspiratory scans obtained at full inspiration. To maximize their potential, HP-specific registries are needed. The scanning protocol for the evaluation of suspected HP is identical to the protocol described in the ATS/ERS/JRS/ALAT diagnosis-of-IPF guidelines (Table 3) (20). (B) Low-magnification photomicrograph showing one of the sections illustrated in A, characterized by a pattern of patchy fibrosis with subpleural honeycomb change that resembles usual interstitial pneumonia. For patients with nonfibrotic HP, a recommendation was made in favor of obtaining bronchoalveolar lavage (BAL) fluid for lymphocyte cellular analysis, and suggestions for transbronchial lung biopsy and surgical lung biopsy were also made. Researchers are beginning to map genetic variations in immune system proteins that may increase the risk for developing hypersensitivity pneumonitis. The need for a thorough history and a validated questionnaire to identify potential exposures was agreed on. Photomicrographs of surgical lung biopsy specimens from two different sites in a patient with fibrotic hypersensitivity pneumonitis. ‡Fibrotic HP may show classic features of nonfibrotic HP (cellular HP) in less fibrotic or nonfibrotic areas; if present, this combination of findings is a histological clue to the diagnosis of HP. We are committed to advancing science and translating discoveries into clinical practice to promote the prevention and treatment of heart, lung, blood, and sleep disorders including hypersensitivity pneumonitis. Such questionnaires should include ambient and occupational causes of HP relevant to the patient’s geographical location and cultural habits. It is the right course of action for >50% of patients. Hypersensitivity pneumonitis is a rare immune system disorder that affects the lungs. For diagnostic approaches, important needs include the validation and standardization of questionnaires, BAL lymphocytosis threshold, specific antibodies, and biomarkers. Comparative analysis of inflammatory and immunocompetent cells in patients with sarcoidosis and hypersensitivity pneumonitis, Clinical studies on human alveolar macrophages: II. Methods: Systematic reviews were performed for six questions. served on an advisory committee, served as a speaker, and received research support from Boehringer Ingelheim; and served as a consultant for Boehringer Ingelheim and Orinove. If the condition goes untreated or is not well controlled over time, the chronic inflammation can cause irreversible scarring of the lungs that may severely impair their ability to function. Post-procedural exacerbations or respiratory failure occurred in 2% (95% CI, 1–3%). Recommended radiation dose for the inspiratory volumetric acquisition: • Strong recommendation to avoid “ultra–low-dose CT” (<1 mSv), • Generic term referring to a patchwork of regions of differing attenuation on inspiratory CT images, • Term only used for description of inspiratory CT images, • Can reflect the presence of vascular disease, airway abnormalities, or ground-glass interstitial or airspace infiltration. In other patients, each of several biopsy sites may demonstrate only a subset of findings, requiring review of all specimens to appreciate the complete set of features required for a confident histological diagnosis of HP. Results: The guideline committee defined HP, and clinical, radiographic, and pathological features were described. High-resolution CT scans may be more informative than lung function tests at assessing disease progression. Occasionally, lung transplants are used to treat severe chronic disease in some patients. Patients with fibrotic HP are more likely to be older, have an unidentified inciting agent, and have a lower vital capacity (VC), diffusion capacity, and percentage of lymphocytes in their BAL fluid than patients with nonfibrotic HP (36). §§Definition and Diagnostic Criteria sections leader. Lung biopsy often plays a pivotal role in ascertaining a diagnosis of HP. served on an advisory committee for Ad Alta, Boehringer Ingelheim, Bristol Myers Squibb, Promedior, and Roche; served as a consultant for Boehringer Ingelheim and Roche; and received research support from Actelion, Avalyn Pharma, Biogen, Boehringer Ingelheim, Bristol Myers Squibb, Galapagos, and Roche. In addition, new techniques like genomic classifiers and artificial intelligence to improve diagnosis and prognosis need to be assessed. Algorithm for the diagnostic evaluation of possible hypersensitivity pneumonitis (HP). Less frequently, there may be constitutional symptoms such as weight loss, flu-like symptoms (chills, low-grade fever, and malaise), chest tightness, and wheezing, as well as physical examination findings of rales and cyanosis (1). The choice, dose, and duration of these medicines will depend on your condition and medical history. Methods: Systematic reviews were performed for six questions. The following are common signs and symptoms of acute, subacute, and chronic hypersensitivity pneumonitis. This often requires sampling of more than one site. Neither PBM nor airway-centered fibrosis is unique to HP, and they therefore do not by themselves establish the diagnosis (140, 152), but they are characteristic and tend to be more profuse in patients with fibrotic HP compared with patients with fibrotic IIPs (140). Voting results: recommendation for, 8; suggestion for, 11; no recommendation or suggestion, 5; suggestion against, 3; recommendation against, 0. For patients with newly identified ILD whose differential diagnosis includes fibrotic HP, the guideline committee suggests BAL with lymphocyte cellular analysis (suggestion, very low confidence in the estimated effects). Microchimerism has been identified in a larger fraction of patients with HP compared with patients with IPF and healthy women; among women with HP, microchimerism is associated with a lower diffusion capacity (111). In others, the interstitial pneumonia may have a more uniform and diffuse distribution without honeycomb change and may more closely resemble a fibrotic NSIP pattern (“NSIP-like”). Over time, some people with chronic hypersensitivity pneumonitis develop clubbing of fingers and toes and irreversible pulmonary fibrosis. Although HP is characteristically associated with an inhaled antigen, exposures may not be identified, despite a thorough evaluation in patients with otherwise typical features of HP (some experts have used the term “cryptogenic HP” or “HP of undetermined cause”) (9, 14, 15, 21, 30, 31). The well-circumscribed, tight cluster of epithelioid cells (macrophages) is affiliated with a characteristic pattern of circumferential lamellar fibrosis. (C) In this high-magnification photomicrograph of a surgical lung biopsy specimen, the giant cells are largely obscured by cytoplasmic Schaumann bodies (arrow), another nonspecific but characteristic feature of the granulomatous response in HP. Regardless of which CPG is followed, the initial steps are similar, and ongoing diagnostic evaluation may be redirected on the basis of the MDD. Another study enrolled 46 patients with IPF and used a nine-item questionnaire, serum IgG testing against HP-associated antigens, and bronchial-challenge testing to identify potential inciting agents of HP. However, smoking can worsen chronic hypersensitivity pneumonitis and cause complications. 1. Distinguishing fibrotic HP from fibrotic IIPs requires identification of centriacinar fibrotic lesions and the features described in nonfibrotic HP. The typical HP pattern (Table 5) relies on the identification of diffusely distributed HRCT findings that include features of lung infiltration (i.e., ground-glass opacity [GGO], mosaic attenuation) plus at least one HRCT abnormality suggestive of small airway disease. Like that observed in nonfibrotic HP, ill-defined centrilobular nodules and mosaic attenuation can be seen (7, 122). (F) Low-magnification photomicrograph of a right-middle-lobe biopsy specimen from the same patient showing features more closely resembling nonfibrotic hypersensitivity pneumonitis. As such, diagnosis relies on the integration of a variety of factors, including history of antigen exposure, precipitating antibodies to the offending antigen, clinical features, BAL, and radiological and pathologic abnormalities. It represents a collaborative effort among the American Thoracic Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax. Lung fibrosis in HP most frequently manifests as irregular fine or coarse reticulation with architectural lung distortion, sometimes with septal thickening, that can be seen alone or in association with traction bronchiectasis in areas of GGO. served on an advisory committee and as a consultant for Boehringer Ingelheim; and received research support from Roche. 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