Revealing Facts About Pulmonary Sarcoidosis in Pakistan

Abstract

PURPOSE: Pulmonary sarcoidosis is prevalent worldwide with variable clinical course. The disease remains hidden or misdiagnosed as tuberculosis in developing countries. However, very little investigative work has been done to elucidate clinical behavior, diagnostic strategy and outcome of treatment in Pakistan. METHODS: A retrospective cohort study was conducted on patients with pulmonary sarcoidosis diagnosed at a tertiary care hospital from 1999 to 2009. RESULTS: 78 patients were enrolled. Mean age at presentation was 42.8 years(Range, 21-74 years). Female to male ratio was 1.3:1 and 65% of patients above 40 years were females. Overall, 19.2% of patients were completely asymptomatic at presentation and 93% of symptomatic patients had acute symptoms.15% patients were smokers.93.4% patients presented with stage I or II disease. One third of patients were treated for presumed tuberculosis before presentation. ACE levels were done in 17 patients and raised in 61%. 49% cases were diagnosed with gallium scan, 40% cases with tissue biopsy. Gallium scan was 90% sensitive with excellent correlation with computed tomography scan findings. Specific treatment was indicated in 48% of symptomatic patients. 90% of them being treated with oral steroids. Mean duration of treatment was 15 months (Range, 2-44 months) and median follow up was 23.5 months (Range, 3.6-130 months). 58% patients showed remission, 18% were still on treatment with favorable response, 5% showed unfavorable response, 2.5% died of disease and 15% of patients had relapse. Those who were asymptomatic or had mild symptoms (52%) did well and did not require specific therapy. CONCLUSION: Our study indicates that patients are mostly presenting at advanced age and 81.8% of patients are symptomatic at presentation. Significant number of patients are misdiagnosed as tuberculosis. Biopsy and gallium scan are good diagnostic tools. Half of symptomatic patient requiring treatment show good response to steroids. CLINICAL IMPLICATIONS: Pulmonary sarcoidosis is prevalent in Pakistan. Gallium scan has a role to support diagnosis in areas where tuberculosis is endemic. Results with oral steroids are promising, further prospective studies are proposed to know long term benefits.