Mimic or coincidentally? TAFRO syndrome and systemic lupus erythematosus: A case-based review

ABSTRACT Castleman’s disease (CD) is a rare, systemic disease with histopathological features of angiofollicular lymph node hyperplasia. In the literature, there are case-level reports that mimic or coexist with systemic lupus erythematosus (SLE) clinically and in the laboratory. Is this condition two separate diseases or is it an imitation of each other? A 73-year-old female patient was admitted to our clinic with arthritis, lymphadenopathy, fever, weight loss, and malar rash. He had a history of idiopathic thrombocytopenic purpura and thrombosis in the right leg tibialis posterior and dorsalis pedis arteries. Excisional lymphadenopathy biopsy indicated a diagnosis of hyaline-vascular-type CD. She had anti-nuclear antibody >1/80 homogeneous pattern, anti-double stranded DNA (anti-dsDNA), Anti-Smith (Sm) antibody positivity, hypocomplementemia (C3 and C4), pleural effusion, and pericardial effusion. For this reason, the classification criteria of the European League Against Rheumatism/American College of Rheumatology were studied. Clinical findings, idiopathic thrombocytopenic purpura history, antibody positivity, malar rash, and arthritis led us to the diagnosis of SLE. She was treated with 1 mg/kg/day prednisolone and hydroxychloroquine 200 mg 2 × 1. Azathioprine 2.5 mg/kg daily was added to the patient whose complaints did not improve. In the follow-ups, she completely recovered clinically and laboratory. SLE and CD are systemic diseases that overlap in many ways. The literature review shows that these two diseases may mimic each other or may coexist. This situation may be a reflection of a pathophysiological process that has not yet been clarified. This confusing process also affects the treatment decision. This confusing process also affects the treatment decision.