Undifferentiated Connective Tissue Disease (UCTD)

Abstract

UCTD presents as a symptom or a set of symptoms suggesting systemic connective tissue disease (SCTD) occurring together with the positivity of autoantibodies, lasting longer than 12 months. Pulmonary involvement mostly with the picture of non-specific interstitial pneumonia (NSIP) is the part of UCTD very frequently. Criteria for the diagnosis of IIP within UCTD are not strict: at least one symptom of SCTD, at least one sign of a systemic inflammatory process, predominance of hazy opacities on chest HRCT scan, and histopathological proof of NSIP. Fifty percent of patients with NSIP fulfill these criteria when compared to only 5 % of patients with a confirmed diagnosis of idiopathic pulmonary fibrosis (IPF). In our case report we are describing the patient fulfilling these diagnostic criteria but having the less frequent histopathologic pattern of UIP.