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AB0570 Igg4 Related Disease: Protean Clinical Manifestations and A Diagnostic Conundrum
  1. S. Chittalae1,
  2. V. Singh1,
  3. P. Efthimiou2,3
  1. 1Medicine, New York Methodist Hospital, Brooklyn
  2. 2Medicine, Weill Cornell Medical College, New York
  3. 3Rheumatology, New York Methodist Hospital, Brooklyn, United States

Abstract

Background -

Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic fibro-inflammatory condition characterized by tumefactive lesions, dense lymphoplasmacytic infiltrate, storiform fibrosis and obliterative phlebitis. Serum IgG4 levels are usually elevated in 70–90% of cases. The mean age at diagnosis is 60 years with a striking male preponderance. (Male:Female – 8:3)

Objectives To present a single center experience with IgG4-RD and the diagnostic and therapeutic challenges encountered.

Methods A retrospective case review at an urban teaching hospital was done over a year after the availability of quantitative assessment of IgG4 levels and tissue immuno-histological staining techniques. This revealed four cases of IgG4-RD involving the pancreas, retroperitoneum, kidney and meninges demonstrating its multi-systemic nature.

Results We encountered a M:F ratio of 3:1 with mean age at presentation being 66 year

Case 1: A 76-year-old male with significant weight loss, erroneously diagnosed with pancreatic tail malignancy, underwent distal pancreatectomy and was subsequently diagnosed with AIP on biopsy.

Case 2: A 71-year-old male s/p left partial nephrectomy 4 years ago with an incidental finding of right kidney mass (2x2x2cm). After undergoing right partial nephrectomy, he was diagnosed with IgG4-related kidney disease.

Case 3: A 56-year-old male with abdominal pain and worsening renal function had MRI findings of a heterogeneous retroperitoneal mass attached to the right kidney. Biopsy revealed a diagnosis of IgG4-retroperitoneal fibrosis.

Case 4: A 57-year-old female presented with headache, bilateral lower extremity weakness and gait abnormality. Elevated serum IgG4, leptomeningeal enhancement on MRI and dural biopsy all suggested IgG4-related pacchymeningitis.

Conclusions IgG4-RD often presents as a diagnostic conundrum. There is a need to increase clinician awareness of IgG4-RD, since early identification and subsequent treatment with immunomodulators may prevent disease progression and prevent complications and/or unnecessary surgeries. PET/CT may prove to be a useful adjunctive imaging technique to investigate for multi-system involvement. Over reliance on serum IgG4 levels for screening may result in missed diagnosis in a significant percentage of patients.

Disclosure of Interest None declared

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