A case of adult-onset Still’s disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema

Masuyama, Atsushi; Kobayashi, Hitomi; Kobayashi, Yasuyuki; Yokoe, Isamu; Sugimura, Yusuke; Maniwa, Keiichiro; Satō, Hiroshi; Ishida, Toru; Hatanaka, Y.

doi:10.1007/s10165-012-0650-9

Cited by 8 publications

(5 citation statements)

References 15 publications

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“…An early sign that should raise the index of suspicion for TTP is development of acute vision impairment. In extreme cases, multiple thrombi formation and massive cytokine release causing progressive vascular permeability, worsening brain edema and, ultimately, rapid death may also occur [53].…”

Section: Thrombotic Thrombocytopenic Purpuramentioning

confidence: 99%

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Life-threatening complications of adult-onset Still’s disease

2014

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Adult-onset Still's Disease (AOSD) since its description in 1971 has proven to be a very complex and challenging disease entity. This rare auto-inflammatory disease is classically described by the "Still's triad" of fever, rash, and arthritis, although the atypical cases frequently outnumber the typical ones. The exact pathogenesis and etiologic factors responsible for the clinical features remain largely obscure, despite recent suggestive cytokine biology findings. Diagnosis is made on clinical grounds, following the exclusion of mimickers of infectious, autoimmune or neoplastic etiology, with the additional consideration of non-specific laboratory abnormalities such as peripheral leukocytosis and elevation of serum ferritin and other acute phase reactants. The disease manifestations are protean and can include diverse complications, affecting multiple organ systems. Moreover, the severity of the organ involvement can vary considerably, representing a wide spectrum from the self-limited to severe. The mainstay of therapy has evolved from the traditional use of corticosteroids and oral immunosupressants to the newer targeted treatments with biologic agents. The scope of this review is to alert the clinician to the existence of life-threatening AOSD complications, namely the macrophage activation syndrome, disseminated intravascular coagulopathy, thrombotic thrombocytopenic purpura, diffuse alveolar hemorrhage, and pulmonary arterial hypertension. Such knowledge may lead in earlier recognition, prompt treatment, and, ideally, improved patient outcomes.

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Section: Thrombotic Thrombocytopenic Purpuramentioning

confidence: 99%

“…There have been at least 11 published cases of TTP/HUS associated with AOSD, five of which with presenting renal failure [56]. Masuyama et al reported a case of AOSD complicated by TTP that resulted in retinal microangiopathy and rapidly fatal cerebral edema [53].…”

Section: Thrombotic Thrombocytopenic Purpuramentioning

confidence: 99%

Life-threatening complications of adult-onset Still’s disease

2014

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“…The association of AOSD with TMA is rare, and to our knowledge, fewer than 30 cases have been reported in the English literature (78,(80)(81)(82)(83). In most cases, TMA occurred during an AOSD flare, and AOSD treatment was associated with TMA resolution, which argues for a pathophysiological link between AOSD and TMA (78).…”

Section: Thrombotic Microangiopathymentioning

confidence: 99%

Complications of adult-onset Still’s disease and their management

Mitrovic

Fautrel

2018

Expert Review of Clinical Immunology

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Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder in which management and treatment have considerably progressed over the past decade. Despite wide use of interleukin (IL)-1 or IL-6 inhibitors, serious complications remain possible. Areas covered: A comprehensive literature search in MEDLINE via Pubmed was performed to review AOSD's severe and sometimes life-threatening complications: reactive hemophagocytic lymphohystiocytosis, coagulation disorders, fulminant hepatitis, cardiac or pulmonary complications and amyloid A amyloidosis. Expert commentary: Early recognition and prompt management is essential to significantly decrease morbi-mortality. The key question is to determine whether the complication is related to the disease itself or related to or favored by (e.g. infection) the ongoing treatment. For all severe AOSD-related complications, high-dose corticosteroids and supportive measures remain the first-line treatment. In case of inadequate response, combination with IL-1 or IL-6 blockers is justified. Cyclosporine A and etoposide remain of interest, especially in case of reactive hemophagocytic lymphohysitocytosis. Plasma exchange may be useful in case of thrombotic microangiopathy. In the near future, new biologic or non-biologic drugs targeting IL-18 or other cytokines or kinases could be of help.

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“…Supportive care in an ICU, with plasma exchange being central [75] 2. High-dose steroids intravenously and then orally Other immunomodulatory agents proposed for refractory cases include anakinra [74], tocilizumab [76], intravenous immunoglobulins, cyclophosphamide, azathioprine, cyclosporine A, and rituximab [60,74]. Despite this treatment, mortality associated with TMA remains high, up to 20% [60].…”

Section: Coagulation Disordersmentioning

confidence: 99%