Introduction: Sarcoidosis is a multisystem granulomatous disease with a highly heterogeneous clinical course. Despite current guidelines, risk stratification in routine clinical practice remains challenging. The prognostic value of several baseline markers, particularly serum angiotensin-converting enzyme (ACE) remains debated.
Objectives: To characterize the demographic, clinical, and functional profile of a real-world cohort of patients with pulmonary sarcoidosis, and to identify baseline clinical, functional, and biochemical factors associated with long-term radiological remission and therapeutic burden.
Materials and Methods: Retrospective review of 47 patients with pulmonary or intrathoracic sarcoidosis, at a secondary care hospital, over an eight-year period ending December 2024. Demographic, clinical, laboratory, functional, imaging, and bronchoalveolar lavage (BAL) data were collected. Baseline serum ACE and pulmonary function tests were defined as those obtained within ±365 days of diagnosis. Between-group comparisons used Fisher's exact, chi-square, and Mann-Whitney U tests; p<0.05 was significant.
Results: The cohort was predominantly male (55.3%), with mean age at diagnosis of 43.8±12.0 years. Löfgren syndrome was present in 19.1%, and extra-pulmonary involvement in 63.2% of non-Löfgren patients. Most patients presented with Scadding stage II (53.2%) or I (36.2%). Baseline ACE was elevated (>70 U/L) in 37.2%. Systemic treatment was required in 66.0% (corticosteroids 59.6%; methotrexate 40.4%); biologics were used in selected refractory cases (adalimumab n=4; infliximab n=4). Complete radiological resolution was achieved in 44.7%. Löfgren syndrome (88.9% vs. 34.2%; p=0.006) and normal baseline ACE (59.3% vs. 18.8%; p=0.013) were associated with higher remission rates. Elevated baseline ACE and reduced DLCO (<75%) were associated with polypharmacy (p=0.032 and p=0.035). Advanced baseline Scadding stage was associated with progression to fibrosis (p=0.014). BAL lymphocytosis and CD4/CD8 ratio showed no association with long-term outcomes.
Conclusions: Baseline serum ACE, Scadding stage and DLCO were associated with outcome, supporting early, multidimensional risk stratification. Patients with elevated ACE, parenchymal involvement, or impaired diffusion capacity may warrant closer clinical and functional monitoring.