Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome
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. Mix...
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Society of Diabetic Nephropathy Prevention
2021-04-01
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| Series: | Journal of Nephropathology |
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| Online Access: | https://nephropathol.com/PDF/jnp-10-e21.pdf |
| _version_ | 1797827960860835840 |
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| author | Irthe Van Assche Thomas Malfait Edwin Steenkiste Ignace Vandewiele Bart Maes |
| author_facet | Irthe Van Assche Thomas Malfait Edwin Steenkiste Ignace Vandewiele Bart Maes |
| author_sort | Irthe Van Assche |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-09T12:57:55Z |
| format | Article |
| id | doaj.art-27284e9a588142ecb2238a3dcb776fe9 |
| institution | Directory Open Access Journal |
| issn | 2251-8363 2251-8819 |
| language | English |
| last_indexed | 2024-04-09T12:57:55Z |
| publishDate | 2021-04-01 |
| publisher | Society of Diabetic Nephropathy Prevention |
| record_format | Article |
| series | Journal of Nephropathology |
| spelling | doaj.art-27284e9a588142ecb2238a3dcb776fe92023-05-13T11:25:49ZengSociety of Diabetic Nephropathy PreventionJournal of Nephropathology2251-83632251-88192021-04-01102e21e2110.34172/jnp.2021.21jnp-15983Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndromeIrthe Van Assche0Thomas Malfait1Edwin Steenkiste2Ignace Vandewiele3Bart Maes4Department of Pathology, Universitair Ziekenhuis Leuven, Herestraat 49, 3000 Leuven, BelgiumDepartment of Nephrology, Algemeen Ziekenhuis Delta, Wilgenstraat 2, 8800 Roeselare, BelgiumDepartment of Pathology, Algemeen Ziekenhuis Delta, Wilgenstraat 2, 8800 Roeselare, BelgiumDepartment of Nephrology, Algemeen Ziekenhuis Delta, Wilgenstraat 2, 8800 Roeselare, BelgiumDepartment of Nephrology, Algemeen Ziekenhuis Delta, Wilgenstraat 2, 8800 Roeselare, BelgiumIntroduction: 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.https://nephropathol.com/PDF/jnp-10-e21.pdfcryoglobulinemiasjogren's syndromeglomerulonephritismembranoproliferativerituximabrenal insufficiencycutaneous vasculitisend-stage renal disease |
| spellingShingle | Irthe Van Assche Thomas Malfait Edwin Steenkiste Ignace Vandewiele Bart Maes Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome Journal of Nephropathology cryoglobulinemia sjogren's syndrome glomerulonephritis membranoproliferative rituximab renal insufficiency cutaneous vasculitis end-stage renal disease |
| title | Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome |
| title_full | Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome |
| title_fullStr | Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome |
| title_full_unstemmed | Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome |
| title_short | Mixed cryoglobulinemic glomerulonephritis and vasculitis in primary Sjögren’s syndrome |
| title_sort | mixed cryoglobulinemic glomerulonephritis and vasculitis in primary sjogren s syndrome |
| topic | cryoglobulinemia sjogren's syndrome glomerulonephritis membranoproliferative rituximab renal insufficiency cutaneous vasculitis end-stage renal disease |
| url | https://nephropathol.com/PDF/jnp-10-e21.pdf |
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