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

Koga, Tomohiro; Yamasaki, Satoshi; Nakamura, Hideki; Kawakami, Atsushi; Furusu, Akira; Taguchi, Takashi; Eguchi, Katsumi

doi:10.1007/s00296-010-1569-0

Cited by 16 publications

(9 citation statements)

References 9 publications

(8 reference statements)

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“…Thrombotic microangiopathies also could play a role in the pathogenesis of further connective tissue diseases, especially in the renal involvement, such as systemic sclerodermia (101), Sjögrens Syndrome (102). …”

Section: Autoimmunity and Tmamentioning

confidence: 99%

Extra-Renal Manifestations of Complement-Mediated Thrombotic Microangiopathies

et al. 2014

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Thrombotic microangiopathies (TMA) are rare but severe disorders, characterized by endothelial cell activation and thrombus formation leading to hemolytic anemia, thrombocytopenia, and organ failure. Complement over activation in combination with defects in its regulation is described in an increasing number of TMA and if primary for the disease denominated as atypical hemolytic-uremic syndrome. Although TMA predominantly affects the renal microvasculature, extra-renal manifestations are observed in 20% of patients including involvement of the central nerve system, cardiovascular system, lungs, skin, skeletal muscle, and gastrointestinal tract. Prompt diagnosis and treatment initiation are therefore crucial for the prognosis of disease acute phase and the long-term outcome. This review summarizes the available evidence on extra-renal TMA manifestations and discusses the role of acute and chronic complement activation by highlighting its complex interaction with inflammation, coagulation, and endothelial homeostasis.

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Section: Autoimmunity and Tmamentioning

confidence: 99%

Extra-Renal Manifestations of Complement-Mediated Thrombotic Microangiopathies

et al. 2014

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“…1 The clinical courses of the present patients: a case 9, b case 10. The graphs display the haemoglobin, platelets, LDH, and creatinine values and the treatment interventions during the hospital course (PSL prednisolone, mPSL methylprednisolone) metalloproteinase levels [22], there are no case reports documenting the occurrence of anti-ADAMTS13 autoantibody as a cause of TTP with a suggestion of concurrent SS, as in our two cases. The present cases may be interpreted as representing an overlapping state involving two autoimmune disorders, typical TTP and SS, presenting with anti-ADAMTS13 autoantibody.…”

Section: Discussionmentioning

confidence: 81%

“…This is apparently a very rare association. A review of the literature reveals only eight additional cases of TTP or haemolytic uraemic syndrome (HUS) associated with pSS in about 40 years [22][23][24][25][26][27][28][29] (Table 1). These cases are similar in many respects, but they show variability in the extent of haemolysis (haemoglobin 6.8-11.0 g/dl), severity of thrombocytopaenia (platelets 5-104 9 10 3 /ll) and renal damage.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren’s syndrome: two cases and a literature review

Yamashita¹,

Takahashi²,

Kaneko³

et al. 2013

Modern Rheumatology

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An association between thrombotic thrombocytopenic purpura (TTP) and Sjögren's syndrome (SS) is rare. This is the first report of two patients with TTP who had inhibitory autoantibodies to ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats) complicating primary SS. A rapid diagnosis of TTP, which is a potentially lethal condition, made it possible to treat the two cases successfully. Only eight similar cases with TTP complicating SS have been reported in the literature. The possible presentation of primary SS without classic sicca symptoms, but with haematological abnormalities including TTP, should be recognised. Furthermore, it is important to measure ADAMTS13 activity and anti-ADAMTS13 antibodies, because TTP with SS seems to be a concurrent overlapping autoimmune disorder. We suggest that plasma exchanges in combination with corticosteroids should be administered as early as possible, since they appeared to be effective in treating TTP with SS, including in our cases.

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“…Her past medical history included bilateral small and contracted kidneys associated with end stage renal disease undergoing maintenance hemodialysis for 8 years. Physical examination revealed a 12 Abe et al 13 Schanttner et al 14 Campbell et al 15 Noda et al 16 Steinberg et al 17 Agapitos et al 18 Settefrati et al 19 Present case SS, Sjögren's syndrome; Hb, hemoglobin; LDH, lactate dehydrogenase; PLT, platelet; AKI, acute kidney injury; RA, rheumatoid arthritis; NA, not available; ANA, antinuclear antibody; RF, rheumatoid factor; LMWH, low-molecular-weight heparin; CPA, cyclophosphamide; PE, plasma exchange; PI, fresh frozen plasma infusion; PGI2, prostaglandin I2; PSL, prednisolone; HD, haemodialysis; ESRD, end stage renal disease.…”

Section: Case Presentationmentioning

confidence: 99%

Thrombotic microangiopathy complicating newly diagnosed Sjögren’s syndrome in a dialysis patient

et al. 2014

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Thrombotic microangiopathy (TMA) is rarely associated with Sjö gren's syndrome (SS). This is the first documented case of a patient undergoing chronic hemodialysis with SS who developed TMA. TMA is an infrequent, life-threatening multisystem disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. It is important to make a quick diagnosis of TMA to cure the reported case as early as possible. The patients with TMA should be diagnosed quickly, and in this case plasma exchange and corticosteroids in combination with cyclophosphamide have been associated with a recurrence free period. Cyclophosphamide has led to the development of treatment protocols using alternative immunosuppressive agents in patients with SS showing a poor response to plasmapheresis and potentially life-threatening manifestations. Further research is required to ascertain the sensitivity, specificity, efficacy, timing, cost-benefit ratio, and necessity of cyclophosphamide in the setting of TMA complicating SS.

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

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

Cited by 16 publications

References 9 publications

Extra-Renal Manifestations of Complement-Mediated Thrombotic Microangiopathies

Extra-Renal Manifestations of Complement-Mediated Thrombotic Microangiopathies

Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren’s syndrome: two cases and a literature review

Thrombotic microangiopathy complicating newly diagnosed Sjögren’s syndrome in a dialysis patient

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