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J Nephropathol. 2021;10(2): e21.
doi: 10.34172/jnp.2021.21
  Abstract View: 365
  PDF Download: 187

Case Report

Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome

Irthe Van Assche 1 * ORCID logo, Thomas Malfait 2 ORCID logo, Edwin Steenkiste 3 ORCID logo, Ignace Vandewiele 2, Bart Maes 2 ORCID logo

1 Department of Pathology, Universitair Ziekenhuis Leuven, Herestraat 49, 3000 Leuven, Belgium
2 Department of Nephrology, Algemeen Ziekenhuis Delta, Wilgenstraat 2, 8800 Roeselare, Belgium
3 Department of Pathology, Algemeen Ziekenhuis Delta, Wilgenstraat 2, 8800 Roeselare, Belgium

Abstract

Introduction: Cryoglobulinemia is a condition where complexes of one or more different classes of immunoglobulins precipitate at low temperatures and become soluble again at higher temperatures. Cryoglobulins are typically categorized as types I to III, based on their immunoglobulin composition. Mixed cryoglobulinemia (type II and III) is most often associated with constitutional symptoms, such as fatigue, myalgia, arthralgia, sensory or motor changes (peripheral neuropathy) and palpable purpura (cutaneous vasculitis). Twenty to thirty percent of the affected patients suffer from membranoproliferative glomerulonephritis.

Case Presentaion: We discuss a case of a 45-year-old woman with a history of Sjögren’s syndrome and mixed cryoglobulinemia who presented with acute renal failure, nephritic syndrome, vasculitislike rash on the legs and non-healing skin ulcer. Further investigations confirmed type II mixed cryoglobulinemia associated with cutaneous leukocytoclastic vasculitis and membranoproliferative glomerulonephritis leading to end-stage renal disease (ESRD).

Conclusion: Mixed cryoglobulinemia secondary to primary Sjögren’s syndrome (pSS) is rare and reported in 22.5% of cases of non-infectious cryoglobulinemic glomerulonephritis. Long-term renal prognosis is good with only 9% of these patients evolving to ESRD. Nevertheless, the long-term overall survival is poor with severe infections as the leading cause of death.

Keywords: Cryoglobulinemia, Sjogren's syndrome, Glomerulonephritis, Membranoproliferative, Rituximab, Renal insufficiency, Cutaneous vasculitis, End-stage renal disease
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Submitted: 01 May 2020
Accepted: 26 May 2020
ePublished: 04 Jun 2020
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