Renal thrombotic microangiopathies/thrombotic thrombocytopenic purpura in a patient with primary Sjögren's syndrome complicated with IgM monoclonal gammopathy of undetermined significance.

Rheumatology International
Tomohiro KogaKatsumi Eguchi

Abstract

Thrombotic microangiopathy (TMA)/thrombotic thrombocytopenic purpura (TTP) is a rare but potentially lethal condition requiring rapid recognition, diagnosis, and initiation of therapy. We experienced a case of a 61-year-old woman with primary Sjögren's syndrome (pSS) complicated with severe renal TMA/TTP following IgM monoclonal gammopathy of undetermined significance (MGUS). She was admitted to our hospital for further evaluation of hypergammaglobulinema, acute renal failure, and severe thrombocytopenia. She had been diagnosed with pSS 13 years prior to admission. Histological examination of her kidney revealed fibrin thrombi in the glomeruli and arterioles, a finding that is consistent with TMA/TTP. The patient was subsequently treated with plasma exchange, which resulted in a successful outcome without any complications. This rare case suggests that it is important to make a therapeutic decision based on appropriate and prompt pathological diagnosis.

References

Aug 1, 1987·Kidney International·G Remuzzi
Dec 6, 2001·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·N BossiniR Maiorca
Aug 10, 2002·Medicine·Manuel Ramos-CasalsMiguel Ingelmo
Mar 28, 2003·Journal of the American Society of Nephrology : JASN·Han-Mou Tsai
Mar 6, 2007·Seminars in Nephrology·Michael J FischerJerrold S Levine

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Citations

Sep 25, 2014·Frontiers in Pediatrics·Johannes HoferThomas Giner
Dec 22, 2016·Kidney International·Aishwarya RavindranSanjeev Sethi
Jun 1, 2018·Clinical Journal of the American Society of Nephrology : CJASN·Larissa HigginsNelson Leung
Dec 15, 2020·Rheumatology and Therapy·Jozélio F Carvalho, Yehuda Shoenfeld

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