Background Sarcoidosis is a systemic granulomatous disease, frequently affecting lungs, eyes and skin, although it may damage other organs, among them the musculoskeletal system
Objectives To describe the clinical characteristics and radiological pattern in a cohort with predominantly pulmonary sarcoidosis, and to determine the relationship between the levels of angiotensin converting enzyme (ACE), pulmonary radiological stage and sarcoidosis course (chronification or remission)
Methods Data from 2328 patients in an Interstitial Lung Diseases consultation during the first half of 2016 were analyzed. Out of these, 50 had sarcoidosis.The delay in the diagnosis of sarcoidosis was defined as the difference in years between the diagnostic suspicion and diagnosis of sarcoidosis.
Chi square tests were used, assuming an error of the first species not higher than 0.05, in order to: 1. Study the association between angiotensin converting enzyme (ACE) levels and binary variables (extrapulmonary symptoms, radiological stage and evolution of S) 2. Determine the association between evolution, the radiological stage and the presence of extrapulmonary symptoms.
Results We included 29 (58%) women and 21 (42%) men, (mean age of 44±11.7 years). Initial diagnosis: 88% S, 8% lymphoma and 4% tuberculosis. Of the 44 diagnosed cases of S, 24 were on the first visit, 11 the following year and 1 seven years later. Of the 4 lymphomas, 2 were diagnosed of S that same year and the other 2 were diagnosed the following year. Of the 2 tuberculosis, one was diagnosed of S in one year and the other at 4 years.
The most frequent extrapulmonary manifestations were cutaneous 24%, followed by the articular, cardiac and ocular in 10%, neurological 8% and renal 4%. In 6% of patients, the first clinical manifestation of the disease was bilateral arthritis of the ankles, The ACE title is increased in 62% of patients, normal in 34%. The mean and standard deviation of the title of patients with an increased ACE value was 150.5 and 53.4 IU/L, respectively. In all patients, x-ray and high resolution tomography were performed, with stage 2 being the most frequent (44%), followed by 3 (20%), 0 and 1 (14%) and 4 (8%). Histological confirmation was obtained by transbronchial biopsy (66%), cutaneous (12%) or lymph node biopsy (12%) in 90% of the patients. 90% of patients have been treated with oral glucocorticoids and 42% associate immunosuppressive therapy.
The ACE levels showed no statistical association with any of the variables studied, although a very clear association (p=0.04754) was observed between the course of the disease and the presence of extrapulmonary symptoms: from the 25 patients without extrapulmonary symptoms, only in 35% of cases the process become chronic.
Conclusions Our results, in general, coincide with what is published in the literature. In our cohort, initial diagnosis of S was relatively high (28/50 =56%), while misdiagnosis was relatively low (6/50 =12%). The level of ACE does not seem to be clearly associated with the presence of extrapulmonary symptoms, nor with the course of S. However, the presence of extra-pulmonary symptoms seems to lead to a chronification
Disclosure of Interest None declared