Pulmonary Sarcoidosis pp 65-78 | Cite as
Acute Pulmonary Exacerbation of Sarcoidosis
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Abstract
Sarcoidosis patients frequently experience an acute exacerbation of pulmonary sarcoidosis (APES). Despite the frequent occurrence of APES, there is a paucity of information regarding its definition, diagnostic criteria, diagnosis, and treatment. APES is a significant clinical problem that adversely affects the quality of life of sarcoidosis patients. We propose the following clinical criteria for the diagnosis of APES: the development or worsening of pulmonary symptoms over at least a 1-month period in patients with known sarcoidosis that cannot be not explained by an alternative cause, combined with a decline in spirometry (≥10 % from previous baseline FVC and/or FEV1). Risk factors for APES include previous treatment with corticosteroids, Black race, and fibrocystic sarcoidosis. The pathogenesis of APES may involve direct or indirect effects of pulmonary granulomatous inflammation. Although relatively low-dose corticosteroid (20 mg of daily prednisone equivalent) for a relatively short time (3 weeks duration) is usually adequate to resolve APES, the corticosteroid tapering schedule and the prognosis of APES are unknown. We believe that further attention to APES will improve the quality of life of sarcoidosis patients.
Keywords
Sarcoidosis Pulmonary exacerbation Definition Risk factors Diagnosis Differential diagnosis Treatment CorticosteroidsAbbreviations
- APES
Acute pulmonary exacerbation of sarcoidosis
- ART
Antiretroviral therapy
- CT
Computed tomography
- HIV
Human immunodeficiency virus
- HLA
Human leukocyte antigen
- INF
Interferon
- SAPH
Sarcoidosis-associated pulmonary hypertension
- TNF
Tumor necrosis factor
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