Thrombotic thrombocytopenic purpura complicating Sjogren's syndrome

Steinberg, A D

doi:10.1001/jama.215.5.757

Cited by 26 publications

(15 citation statements)

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“…A review of the literature revealed six other such cases (Table 1). 8,[25][26][27] Three (patients 1, 5, 7 in Table 1) of the total seven cases of TTP complicating SS (including ours) had cellular infiltration or immunological findings in the glomeruli. All three patients had a rapidly fatal course despite plasma exchange.…”

Section: Discussionmentioning

confidence: 72%

Thrombotic thrombocytopenic purpura complicating Sj�gren?s syndrome with crescentic glomerulonephritis and membranous nephritis

et al. 2004

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The association of either thrombotic thrombocytopenic purpura (TTP) or crescentic glomerulonephritis with Sjögren's syndrome is rare. We report a case of TTP appearing after the diagnosis of SjOgren's syndrome with crescentic glomerulonephritis and membranous nephropathy. Circulating immune complex was detected, and immune complex deposits were shown along the capillary walls of renal biopsy specimens. Despite steroid pulse therapy and plasma exchange therapy, the patient died. The etiology of TTP is unclear. This case is important when considering the etiology of TTP related to autoimmune disease.

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Section: Discussionmentioning

confidence: 72%

Thrombotic thrombocytopenic purpura complicating Sj�gren?s syndrome with crescentic glomerulonephritis and membranous nephritis

et al. 2004

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“…Although thrombotic thrombocytopenic purpura (TTP) occurs in association with many connective tissue diseases [1][2][3][4][5], cases of TTP which occur during the course of scleroderma have been relatively rare. In this paper we present a patient with a rapidly progressive diffuse form of systemic sclerosis and TTP.…”

Section: Introductionmentioning

confidence: 72%

Thrombotic thrombocytopenic purpura in a patient with a diffuse form of systemic sclerosis

Sadamoto

Saito

Ogawa

et al. 1999

Japanese Journal of Rheumatology

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This paper presents a patient with a rapidly progressive diffuse form of systemic sclerosis, in whom a clinical course thrombotic thrombocytopenic purpura (TTP) also developed. In spite of treatment with plasmapheresis, predonisolone, aspirin and vinc¡ she died of pulmonary bleeding. From the analysis of four other reported cases of scleroderma with TTP and this case, the outcome of TTP seems to be closely related to clinical manifestations. It is suggested that a diffuse forro of scleroderma with interstitional manifestations would develop a worse outcome; on the other hand, a limited form without interstitional manifestations has a successful outcome.

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“…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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Thrombotic thrombocytopenic purpura complicating Sjogren's syndrome

Cited by 26 publications

References 0 publications

Thrombotic thrombocytopenic purpura complicating Sj�gren?s syndrome with crescentic glomerulonephritis and membranous nephritis

Thrombotic thrombocytopenic purpura complicating Sj�gren?s syndrome with crescentic glomerulonephritis and membranous nephritis

Thrombotic thrombocytopenic purpura in a patient with a diffuse form of systemic sclerosis

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

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