Prise en charge par échanges plasmatiques d'un purpura thrombotique thrombocytopénique au cours d'une maladie de Still : à propos d'un cas

Robert, Véronique; Eszto, P.; Perrotez, J.-L.; Galzin, M; Poussel, J.F.

doi:10.1016/j.annfar.2006.01.014

Cited by 8 publications

(3 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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

View full text Add to dashboard Cite

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.

show abstract

Section: Introductionmentioning

confidence: 99%

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

et al. 2015

View full text Add to dashboard Cite

show abstract

“…It is used to confirm secondary character of this disorder. 13 But, these assays were not always available in our laboratory.…”

Section: Discussionmentioning

confidence: 99%

“… 21 , 22 Rare therapeutic trials suggested lower rates of relapse after splenectomy. 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 …”

Section: Discussionmentioning

confidence: 99%

Coexistence of thrombotic thrombocytopenic purpura and adult‐onset Still's disease

Salah

Bouattour

Turki

et al. 2022

Clinical Case Reports

View full text Add to dashboard Cite

show abstract

Thrombotic microangiopathy in adult-onset Still's disease: case report and review of the literature

Šalamon

Morović‐Vergles

2009

Wien Klin Wochenschr

View full text Add to dashboard Cite

Coexistence of thrombotic microangiopathy and adult-onset Still's disease is extremely rare. There is increasing evidence that this association could be more than just coincidental. We report on the case of a 34-year-old male diagnosed with adult-onset Still's disease and successfully treated with intravenous glucocorticoids. Nine months after onset the patient exhibited the presence of asymptomatic thrombocytopenia during treatment with chloroquine. The physical status was unremarkable except for pallor of the skin and mucosa. Laboratory evaluation revealed profound thrombocytopenia and hemolytic anemia. Coombs' tests were negative; renal function tests were all normal. The peripheral blood smear showed frequent schistocytes. Based on the presence of thrombocytopenia and microangiopathic hemolytic anemia, and with the exclusion of other known causes, the patient was diagnosed with thrombotic microangiopathy and successfully treated with plasma exchange and intravenous glucocorticoids. We also review the literature on the association between adult-onset Still's disease and thrombotic microangiopathy; our case is the 15(th) report on such an association. The mean age at onset of adult Still's disease in these cases was 31.60 years and the interval between the diagnosis of Still's disease and the onset of thrombotic microangiopathy ranged from 3 days to 17 years, with a female/male ratio of 2 : 1. In more than half the patients thrombotic microangiopathy occurred within the first 6 months after the diagnosis of the Still's disease. Eleven of the 15 (73%) patients were treated with plasmapheresis in addition to glucocorticoid therapy: eight of 11 (73%) had complete remission, the other three had permanent visual impairment and/or digital ischemia. Of the four patients who were not treated with plasmapheresis, two died, one developed end-stage renal disease and one had complete remission. Awareness of the possible development of thrombotic microangiopathy in patients with adult-onset Still's disease is critical, so that treatment can be initiated early and the complications and recurrence of thrombotic microangiopathy prevented. Patients with adult-onset Still's disease should be closely monitored for signs and symptoms of thrombotic microangiopathy during the first six months after diagnosis of the Still's disease.

show abstract

Prise en charge par échanges plasmatiques d'un purpura thrombotique thrombocytopénique au cours d'une maladie de Still : à propos d'un cas

Cited by 8 publications

References 4 publications

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

Coexistence of thrombotic thrombocytopenic purpura and adult‐onset Still's disease

Thrombotic microangiopathy in adult-onset Still's disease: case report and review of the literature

Contact Info

Product

Resources

About