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Chronic Hypersensitivity Pneumonitis: A Prospective Cohort Analysis of an Under-Recognized Clinical Entity
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A1104 - Chronic Hypersensitivity Pneumonitis: A Prospective Cohort Analysis of an Under-Recognized Clinical Entity
Author Block: H. Barnes1, A. Watson2, S. Ellis1, N. Goh3, G. P. Westall4, A. E. Holland5, I. Glaspole6; 1AIRMED, Alfred Hospital, Melbourne, Australia, 2School of Physiotherapy, La Trobe University, Melbourne, Australia, 3Austin Hospital, Melbourne, Australia, 4Alfred Hospital, Melbourne, Melbourne, Australia, 5La Trobe University / Alfred Health, Melbourne, Australia, 6Alfred Hospital, Footscray, Australia.
Rationale: Chronic hypersensitivity pneumonitis (CHP) is an immune mediated interstitial lung disease, caused by an antecedent exposure to an offending agent. It can be difficult to accurately diagnose, but may improve with immunomodulatory treatment. Little is currently known about patient characteristics and prognosis.We sought to determine what characteristics suggested a diagnosis of CHP, and what factors might influence disease trajectory and mortality.
Methods: We collected data from all patients with a consensus diagnosis of CHP from the Alfred Interstitial Lung Diseases Registry: a prospective, longitudinal cohort registry commenced in 2002.
Results: Fifty-five patients with a consensus diagnosis of CHP were identified (mean age 55±10yrs; 43% male). Dyspnea was the most common symptom at disease onset, clubbing common (40%) but not crackles (9%). Over 50% had a clinical exposure identified, including 30% with avian exposure, however, only 37% underwent avians precipitans testing and none were positive. Multi-disciplinary meeting (MDT) clinical diagnostic confidence was only definite in 10%, probable in 61% and equivocal in 30%. Reticulation in a subpleural and peribronchovascular distribution, ground glass changes, and centrilobular nodules, with associated bronchiectasis, was most commonly present radiologically. Usual interstitial pneumonia with multinucleated histiocytes was the predominant histopathological feature on biopsy (27%) or explant (21%). Bronchoalveolar lavage was rarely performed. Immunomodulatory treatment was used in 70% of patients, most commonly prednisolone (45%). Oxygen supplementation was used in 20%. Nine-percent were deceased at time of follow up, and 5% underwent lung transplantation. The average rate of decline per year was FEV1 150±10mL, FVC 117±29mL, and %TLCO 2±18%. The rate of decline was not affected by identification of clinical exposure (p=0.7) or immunomodulatory treatment (p=0.4).
Conclusion: This data describes the natural history of CHP patients, however is limited by small patient numbers. Further larger registry research is required to aid more accurate MDT diagnosis, predict prognostic factors, and response to treatment.
Author Block: H. Barnes1, A. Watson2, S. Ellis1, N. Goh3, G. P. Westall4, A. E. Holland5, I. Glaspole6; 1AIRMED, Alfred Hospital, Melbourne, Australia, 2School of Physiotherapy, La Trobe University, Melbourne, Australia, 3Austin Hospital, Melbourne, Australia, 4Alfred Hospital, Melbourne, Melbourne, Australia, 5La Trobe University / Alfred Health, Melbourne, Australia, 6Alfred Hospital, Footscray, Australia.
Rationale: Chronic hypersensitivity pneumonitis (CHP) is an immune mediated interstitial lung disease, caused by an antecedent exposure to an offending agent. It can be difficult to accurately diagnose, but may improve with immunomodulatory treatment. Little is currently known about patient characteristics and prognosis.We sought to determine what characteristics suggested a diagnosis of CHP, and what factors might influence disease trajectory and mortality.
Methods: We collected data from all patients with a consensus diagnosis of CHP from the Alfred Interstitial Lung Diseases Registry: a prospective, longitudinal cohort registry commenced in 2002.
Results: Fifty-five patients with a consensus diagnosis of CHP were identified (mean age 55±10yrs; 43% male). Dyspnea was the most common symptom at disease onset, clubbing common (40%) but not crackles (9%). Over 50% had a clinical exposure identified, including 30% with avian exposure, however, only 37% underwent avians precipitans testing and none were positive. Multi-disciplinary meeting (MDT) clinical diagnostic confidence was only definite in 10%, probable in 61% and equivocal in 30%. Reticulation in a subpleural and peribronchovascular distribution, ground glass changes, and centrilobular nodules, with associated bronchiectasis, was most commonly present radiologically. Usual interstitial pneumonia with multinucleated histiocytes was the predominant histopathological feature on biopsy (27%) or explant (21%). Bronchoalveolar lavage was rarely performed. Immunomodulatory treatment was used in 70% of patients, most commonly prednisolone (45%). Oxygen supplementation was used in 20%. Nine-percent were deceased at time of follow up, and 5% underwent lung transplantation. The average rate of decline per year was FEV1 150±10mL, FVC 117±29mL, and %TLCO 2±18%. The rate of decline was not affected by identification of clinical exposure (p=0.7) or immunomodulatory treatment (p=0.4).
Conclusion: This data describes the natural history of CHP patients, however is limited by small patient numbers. Further larger registry research is required to aid more accurate MDT diagnosis, predict prognostic factors, and response to treatment.
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