Two Cases of Mixed Connective Tissue Disease Complicated With Thrombotic Thrombocytopenic Purpura

Suzuki, Eiji; Kanno, Takashi; Asano, Tomohiko; Tsutsumi, Akito; Kobayashi, Hiroko; Watanabe, Hiroshi; Ohira, Hiromasa

doi:10.5387/fms.59.49

Cited by 10 publications

(7 citation statements)

References 14 publications

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“…In addition, however, early plasmapheresis is essential to reduce mortality. [6,11,12] Where plasmapheresis is not available, plasma infusion is a recommended albeit inferior alternative.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura in the presence of connective tissue disease and HIV infection: A diagnostic and therapeutic challenge in a resource- constrained setting

2016

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Thrombotic thrombocytopenic purpura is a catastrophic disease and may occur in the presence of other confounding diseases. We present a case of TTP in a patient with connective tissue disease and HIV infection, in whom the diagnosis and management of TTP was challenging. It is important to understand the various underlying mechanisms that drive TTP in the presence of these comorbid diseases, so that an appropriate treatment strategy can be initiated. Our patient failed an initial trial of plasma infusion alone, but responded well to plasma exchange.

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“…In addition, however, early plasmapheresis is essential to reduce mortality. [6,11,12] Where plasmapheresis is not available, plasma infusion is a recommended albeit inferior alternative.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura in the presence of connective tissue disease and HIV infection: A diagnostic and therapeutic challenge in a resource- constrained setting

2016

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“…A number of autoimmune disorders have been reported in association with TTP: systemic lupus erythematosus (SLE), the antiphospholipid syndrome, 9 – 11 antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, 12 – 14 Sjögren syndrome, 15 , 16 mixed connective tissue disorders, 17 and other systemic diseases, such as Still disease, 18 scleroderma, 19 and ankylosing spondylitis. 20 SLE is the most studied of these entities, with reports of cases occurring before, at the same time or after the diagnosis of TTP.…”

Section: Introductionmentioning

confidence: 99%

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

et al. 2015

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Autoimmune thrombotic thrombocytopenic purpura (TTP) can be associated with other autoimmune disorders, but their prevalence following autoimmune TTP remains unknown. To assess the prevalence of autoimmune disorders associated with TTP and to determine risk factors for and the time course of the development of an autoimmune disorder after a TTP episode, we performed a cross sectional study. Two-hundred sixty-one cases of autoimmune TTP were included in the French Reference Center registry between October, 2000 and May, 2009. Clinical and laboratory data available at time of TTP diagnosis were recovered. Each center was contacted to collect the more recent data and diagnosis criteria for autoimmunity. Fifty-six patients presented an autoimmune disorder in association with TTP, 9 years before TTP (median; min: 2 yr, max: 32 yr) (26 cases), at the time of TTP diagnosis (17 cases) or during follow-up (17 cases), up to 12 years after TTP diagnosis (mean, 22 mo). The most frequent autoimmune disorder reported was systemic lupus erythematosus (SLE) (26 cases) and Sjögren syndrome (8 cases). The presence of additional autoimmune disorders had no impact on outcomes of an acute TTP or the occurrence of relapse. Two factors evaluated at TTP diagnosis were significantly associated with the development of an autoimmune disorder during follow-up: the presence of antidouble stranded (ds)DNA antibodies (hazard ratio (HR): 4.98; 95% confidence interval (CI) [1.64–15.14]) and anti-SSA antibodies (HR: 9.98; 95% CI [3.59–27.76]). A follow-up across many years is necessary after an acute TTP, especially when anti-SSA or anti-dsDNA antibodies are present on TTP diagnosis, to detect autoimmune disorders early before immunologic events spread to prevent disabling complications.

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“…It is of interest to note, although the findings of a normal ADAMTS-13 does not support the diagnosis of TTP, rare cases have been reported of MCTD complicated by TTP with normal ADAMTS-13 levels. 8 Whereas plasma exchange is contraindicated with pure SRC, due to the possibility of a bradykinin mediated reaction resulting in severe hypotension, which is treated with the initiation of ACEi and strict blood pressure control.…”

Section: Discussionmentioning

confidence: 99%

Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity

Cheta

Rijhwani

Rust

2017

Journal of Investigative Medicine High Impact Case Reports

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Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity. In this article, we report a rare case of scleroderma renal crisis in a patient with MCTD who we treated with plasma exchange for clinical suspicion for an underlying thrombotic thrombocytopenia and mycophenolate mofetil for MCTD. The patient had multiple poor prognostic indicators yet made a full renal recovery in less than 3 months.

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Two Cases of Mixed Connective Tissue Disease Complicated With Thrombotic Thrombocytopenic Purpura

Cited by 10 publications

References 14 publications

Thrombotic thrombocytopenic purpura in the presence of connective tissue disease and HIV infection: A diagnostic and therapeutic challenge in a resource- constrained setting

Thrombotic thrombocytopenic purpura in the presence of connective tissue disease and HIV infection: A diagnostic and therapeutic challenge in a resource- constrained setting

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity

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