Concurrent idiopathic multicentric Castleman disease with TAFRO and Sjögren’s syndrome in a young patient: a challenging diagnostic overlap

Idiopathic multicentric Castleman disease (iMCD) with TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, organomegaly) (iMCD-TAFRO) is a rare, life-threatening disorder marked by severe systemic inflammation and multiorgan failure, and early recognition and timely initiation of immunomodulatory therapy are critical for positive outcomes.Although current diagnostic criteria for iMCD-TAFRO exclude patients with concomitant autoimmune diseases such as Sjögren's syndrome, an increasing number of case reports suggest that these conditions may coexist, challenging existing definitions and suggesting a potential physiopathological overlap.This case highlights the diagnostic challenge posed by the coexistence of these conditions and underscores the need for an individualized assessment integrating clinical, laboratory, and immunological findings.