Frequência elevada de calcinose em dermatomiosite juvenil: estudo de fatores de risco

Clemente, Gleice; Piotto, Daniela Gerent Petry; Barbosa, Cássia Maria Passarelli Lupoli; Peracchi, Octávio Augusto Bedin; Len, Cláudio Arnaldo; Hilário, Maria Odete Esteves; Terreri, Maria Teresa

doi:10.1590/s0482-50042012000400007

Cited by 23 publications

(15 citation statements)

References 24 publications

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“…It was also related to disease control, chronicity and the presence severe skin involvement [13]. However in a study of 34 cases of JDM, no risk factors for calcinosis were found [1].…”

Section: Discussionmentioning

confidence: 99%

“…Calcinosis, a well-recognized sequela, is considered one of the features by which JDM differs from adult dermatomyositis as it generally occurs in 10% -70% of children with JDM [1]. Although it is not one of the diagnostic criteria, its progression may contributes to increased morbidity [8] and even mortality in JDM patients as it may continue even if the disease was controlled [9].…”

Section: Discussionmentioning

confidence: 99%

“…Calcinosis in JDM presents generally between 1 -3 years after diagnosis although it may presents at diagnosis or many years later [1].…”

Section: Discussionmentioning

confidence: 99%

“…Most of reports about the effectiveness of rituximab in treating myositis did not asses its efficacy on calcinosis, however it was found that early and aggressive treatment of JDM may result in complete regression of calcinosis [1].…”

Section: Discussionmentioning

confidence: 99%

“…Unlike adults with dermatomyositis, calcinosis is more common in JDM as it occurs in up to 70% of cases [1]. Although it may precede JDM diagnosis [2], its development was related to delayed diagnosis and treatment and chronicity of JDM [3].…”

Section: Introductionmentioning

confidence: 99%

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Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis

Alhemairi¹,

Muzaffer²

2017

OJRA

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Background: Calcinosis is an important sequela of JDM which may cause significant morbidity and mortality. There is no standard curative treatment for calcinosis but different agents were used with variable efficacy. We report the favorable outcome of rituximab on severe calcinosis in 4 JDM patients and present their clinical data. Patients and Methods: A retrospective chart review of 4 children with JDM and severe calcinosis who received rituximab for relapsing or polycyclic JDM course. Diagnosis and follow up of calcinosis was clinically and by X-ray. Review data included: age of patients at onset of JDM symptoms and diagnosis, clinical and laboratory criteria at diagnosis, disease course and duration of follow up. Data about calcinosis onset, sites, severity and its progression were also included. Further data about rituximab therapy included: dosage, side effects, other treatment used before, during or after this drug and outcome and duration of follow up of calcinosis after therapy. Results: 4 patients (2 male, 2 female), interval between onset of symptoms and diagnosis was 6 -12 months, course of JDM was polycyclic or relapsing, duration of follow up was 5 -7 years. Calcinosis was severe causing ulceration, recurrent skin infections and joint limitation. It was not improving despite treatment with different DMARDs and/or bisphosphonates, colchicine and warfarin. Reason to start rituximab was inadequate disease control with conventional DMARDs. All patients received steroids and more than one DMARD before starting rituximab and were continued thereafter, follow up after rituximab was 3 to 5 years. All patients had improvement in disease activity and frequency of admission especially due to complications of calcinosis. One patient had complete clearance of calcinosis for the last 5 years. Others had significant improvement in calcinosis with no new lesions, decreased sites and density and decreased calcinosis related contractures. There were no serious side effects to rituximab. Conclusion: Our study showed the favorable effect of rituximab in treatment of calcinosis in 4 patients with JDM-associated severe calcinosis when it was used with other conventional DMARDs.

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“…It was also related to disease control, chronicity and the presence severe skin involvement [13]. However in a study of 34 cases of JDM, no risk factors for calcinosis were found [1].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Calcinosis in JDM presents generally between 1 -3 years after diagnosis although it may presents at diagnosis or many years later [1].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis

Alhemairi¹,

Muzaffer²

2017

OJRA

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Identification of Clinical Features and Autoantibodies Associated With Calcinosis in Dermatomyositis

et al. 2014

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IMPORTANCE Prior studies have estimated that up to 20% of adults with dermatomyositis (DM) have calcinosis, which can lead to significant morbidity. Identification of risk factors may provide a better understanding of the pathogenesis and ultimately therapy for this difficult clinical problem. Risk factors for calcinosis in adults with DM have not been extensively studied. OBJECTIVES To determine the prevalence of calcinosis and to identify associated clinical features in a cohort of extensively phenotyped adults with DM. DESIGN, SETTING, AND PARTICIPANTS A cross-sectional study of 126 patients diagnosed as having DM from January 1, 2006, through January 1, 2013, was performed. Patients were adults (≥18 years of age) attending the Stanford University Medical Center clinic. MAIN OUTCOMES AND MEASURES Calcinosis, defined as the presence of calcium deposition in the skin and subcutaneous tissues on physical examination. RESULTS Fourteen patients (11.1%) had calcinosis, with the extremities most commonly involved. Patients with vs those without calcinosis had a longer disease duration (median, 6.9 years; range, 2.4–18.1; vs median, 3.9 years; range, 0.2-19.2 years; P = .003) and more fingertip ulcers (50.0% vs 9.3%, P < .001). An association between calcinosis and both interstitial lung disease and anti–MDA-5 autoantibodies was identified, but this association did not persist in multivariate models that adjusted for fingertip ulcers. Fingertip ulcers and disease duration were strongly associated with calcinosis in all multivariate models, independent of the underlying autoantibody present. Autoantibodies to NXP-2 were associated with calcinosis (odds ratio, 15.52; 95% CI, 2.01-119.90), whereas anti–transcriptional intermediary factor 1-γ antibodies were protective (odds ratio, 0.2; 95% CI, 0.01-0.99) in multivariate analyses that adjusted for fingertip ulcers and other covariates. CONCLUSIONS AND RELEVANCE Calcinosis was a relatively uncommon clinical feature in our cohort of adults with DM. Our data suggest that calcinosis is positively associated with longer disease duration, fingertip ulcers, and NXP-2 autoantibodies and negatively associated with transcriptional intermediary factor 1-γ antibodies. A common vascular mechanism may underlie the development of both calcinosis and fingertip ulcers in patients with DM.

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Lower extremity lipedema, upper extremity lipodystrophy and severe calcinosis complicating juvenile dermatomyositis

Pavlov-Dolijanovic¹,

Stupar

Gavrilov³

et al. 2014

Rheumatol Int

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Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood. Significant proportions of patients have residual weakness, muscle atrophy, joint contractures, and calcinosis. Recently, new clinical findings, such as lipodystrophy accompanied with increased fat deposition in certain areas, have been reported. So far, it is not known whether the redistribution of body fat may be the type of lipedema of lower extremity. We describe a 39-year-old woman who was diagnosed with JDM at the age of 7. Later she developed symmetrical lipodystrophy of upper extremities and symmetrical lipedema of lower extremities (making 2 and 58.3 % of total body fat mass, respectively), with multiple calcified nodules in the subcutaneous tissues. These nodules gradually increased in size despite therapy. Capillaroscopy findings showed scleroderma-like abnormalities. ANA and anti-U1RNP antibodies were positive. Similar cases with simultaneous occurrence of the lipedema of lower extremities, lipodystrophy of upper extremities, and severe calcinosis complicating JDM have not been published so far. We showed that the calcinosis and lipodystrophy were associated with short duration of active disease. Also, we display case that raises the question whether it is possible overlapping autoimmune diseases revealed during follow-up.

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Frequência elevada de calcinose em dermatomiosite juvenil: estudo de fatores de risco

Cited by 23 publications

References 24 publications

Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis

Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis

Identification of Clinical Features and Autoantibodies Associated With Calcinosis in Dermatomyositis

Lower extremity lipedema, upper extremity lipodystrophy and severe calcinosis complicating juvenile dermatomyositis

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