Tafro Syndrome - Mimicker of SLE

Abstract

Background: TAFRO SYNDROME mimics SLE. Differentiating both is essential for correct manangement. Case report: 40 year old lady came with complaints of fever, insidious onset of painless abdominal distension which was gradually progressive to form tense ascities in a span of 2 months. On examination she was pallor, having pedal edema, tense ascities and cervical and axillary lymph node enlargement (mazimum 1* 1 cm). Lymph nodes were firm, non matted, not fixed. On evaluation found to have pancytopenia. (Hb: 5.3 gm%, platelet count, Wbc-3600/cu mm, 90,000/cu mm), Her LFT -alkaline phosphate -639 U/L, Hyperbilirubinemia with total Bil of 1.73 mg/dl and direct fraction of 1 mg/dl and LDH of 139 U/L. Renal - 24 hour urine protien = 301 mg. She had malena at presentation and her Hb dropped to 3.2 gm% platelets count of 10,000. Ct abdomen showed -Mild to moderate ascites, mild hepatomegaly. Ascitic Tap of 3 litre was done which showed high SAAG and High protein. Ugi endoscopy was done which showed Antral gastritis and no feature of porat hypertension. Infective workup including tuberculosis came negative. Autoimmune work ANA - 2+ speckled. (1:100 dilution) Coombs test direct - 3+ C3 = 115 (90 to 180) C4 = 39.8 (10 to 40) Ds DNA = 121 (< 100 IU/ ml) Urine UP/UC = 0.48 24 hour urine protien = 301 mg Anti SSA ELISA test - 63 (< 20 RU/ml) Anti SM ELISA test 1 (< 20 Ru/ml) ELISA Anti RNP - 2 Ru/ml (< 20) APLA work up was within normal limit and lupus anticoagulant was mildy positve. She was satifying the ACR EULAR 2019 classification criteria for SLE and SLE can have lymphadenopathy features suggestive of castle man disease. She was intially diagnosed as SLE and treated with Sle protocol and was not reposnding. Lymphnode biopsy later was done suggestive of Hypervascular type of castle man disease and intiated on R - CEP (Rituximab, Cyclophosphamide, etosposide, prednsiolone) LEARNING POINT 1) SLE is a great mimicker of variety of other diseases and rheumatologist should be aware of the disease that mimics SLE. 2) TAFRO syndrome is variety of castleman disease. T-Thrombocytopenia A-Ascities F-Fever R-renal dysfunction O-Organomegaly 3) TAFRO syndrome has autoimmune manifestations that mimics SLE and it an aggressive variety of castleman disease