LAUSR

Background: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia and a deficient production of specific antibodies. Almost 30% of patients with CVID develop an autoinmmune and granulomatous disease, similar to clinical and histological sarcoidosis (S), it affects the lung fundamentally. This can lead to a misdiagnosis of S in a patient with CVID, this leads to inadequate treatment and increases the morbidity and mortality of the disease. Objectives: Describe the clinical and radiological characteristics of a cohort of patients diagnosed with S with predominant pulmonary involvent. Make a study of immunoglobulin (IG) levels to see the frequency of CVID in these patients. Methods: Retrospective descriptive study of patients treated in our Hospital (2008-2018), with diagnosis of S. The data was obtained by reviewing medical records. The delay in the diagnosis of S was defined as the difference in months between the initial diagnostic suspicion and the final diagnosis of S. In patients with low levels IG, we have done and expanded ID study. Results: 55 patients (31 women) were included, with a mean age of 52 ±12 years. The initial diagnosis was: 85% S, 10% lymphoma and 4% tuberculosis. The median of months from the start of the clinic to the diagnosis of S was 5.5 months. Regarding the clinic, 21% patients present fever at the beginning of the disease, and 65% extrathoracic localization (cutaneous was the most frequent in 27%, and renal was the least frequent 5%). Simple x-ray and high resolution tomography of chest were done in all patients. Pulmonary stage 2 was the most frequent (51%), followed by stage 3 (16%), stage 0 (14%) and stage 4 (9%). In 90% of the patients, histological confirmation was obtained by transbronchial (47%), cutaneous (11%) or lymph node biopsy (29%). Igs were normal in 87% of patients, only 4 patients had low IG levels (Ig G in 3 patients and Ig M in 1). An extended ID study was performed in these 4 patients, being diagnosed with CVID 3 patients. Results of the 4 patients and the differential characteristics between CVID and S in table 1. Differential diagnosis CVID Sarcoidosis Patient 1 Patient 2 Patient 3 Patient 4 Recurrent infections +++ +/- Yes Yes Yes Yes Autoimmunity +++ + Yes Yes No No Splenomegaly +++ +/- Yes Yes Yes Yes Hepatomegaly +++ +/- Yes Yes Yes Yes Low levels IG +++ +/- Yes Yes Yes Low levels IgM Clinic Pulmonary Pulmonary Pulmonary Pulmonary Radiological stage Stage 2 Stage 2 Stage 2 Stage 2 Histological granuloma +++ +++ Yes Yes Yes Yes Increase of CD4/CD8 in BAL + +++ Low levels Low levels Low levels Not done Treatment Corticoids Corticoids Corticoids Corticoids Conclusion: Although their clinical presentation and histological appearance may be identical, the management of these two conditions is very different. The difficulties in the differential diagnosis between S and CVID, shows the importance of a history of screening for recurrent infections and the measurement of IG levels before the diagnosis of possible S. Disclosure of Interests: None declared