Complication Of Juvenile Dermatomyositis Research Articles

Mining for Gems: Early Detection of Calcinosis in Juvenile Dermatomyositis Using Low-Dose Computed Tomography.

Calcinosis, a common complication in juvenile dermatomyositis (JDM), affects up to 40% of patients and can be associated with uncontrolled disease activity and morbidity.1A 9-year-old female with a 17-month history of JDM presented with worsened bilateral knee pain.

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: case reports and a review of the literature

BackgroundTo summarize the characteristics of gastrointestinal (GI) perforation in anti-nuclear matrix protein 2 (NXP2) antibody-associated juvenile dermatomyositis (JDM).MethodsFive patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed.ResultsFive patients, including four females and one male, were included in the study. The age of onset of these patients ranged from 3.3 to 9.5 years with the median age of 5.0 years. When these patients were complicated by GI perforation, childhood myositis assessment score (CMAS) ranged from 1 to 5 with the median score of 2. Myositis-specific antibody (MSA) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients. One patient failed to undergo a repair as the perforation was high in position. For the other three patients, perforation repair was successful, of which two patients failed due to recurrent perforation. At 24 months postoperative follow-up, one patient was in complete remission on prednisone (Pred) and methotrexate (MTX) treatment, and her ureteral stricture had disappeared. The other four patients died. Adding these cases with 16 other patients described in the literature, the symptom at onset was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most commonly occurred in the duodenum, although it also occurred at multiple sites or was recurrent. The mortality rate of GI perforation in JDM was 38% (8/21).ConclusionsAll the five perforation cases in our study subjected to MSA analysis were anti-NXP2 antibody positive. The symptom at onset was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations prevented early diagnosis. GI perforation may be a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

Clinical significance of subcutaneous fat and fascial involvement in juvenile dermatomyositis

Objectives: Subcutaneous involvement, including calcinosis and panniculitis, is a more common complication in juvenile dermatomyositis (JDM) than in adult dermatomyositis. Magnetic resonance imaging (MRI) is useful for evaluating disease distribution. We investigated the clinical significance of subcutaneous involvement in JDM.Methods: Thighs and hips in 18 newly diagnosed JDM patients were evaluated with fat-suppression MRI. Bilateral muscle, fascial and subcutaneous fat involvement were scored from 0 to 8 points according to the severity of distribution on MRI. Associations between clinical manifestations, serum muscle enzymes, and MRI scores were also evaluated.Results: Abnormal MRI findings in muscle, fascia and subcutaneous fat were observed in 18, 18, and 10 patients, respectively. Subcutaneous fat scores were significantly higher in early-diagnosed JDM patients (diagnosed less than 2 months from onset) than in late-diagnosed JDM patients (diagnosed later) (p = .025). Serum aldolase was elevated in all patients, although only eight demonstrated elevated serum creatine phosphokinase. Serum aldolase was significantly correlated with MRI scores for subcutaneous fat (p < .0001, ρ = .787) and fascia (p = .013 ρ = 0.574), but not muscle. Additionally, serum aldolase was significantly correlated with serum triglycerides (p = .009, ρ = 0.629).Conclusion: Subcutaneous fat involvement is a characteristic finding in early-diagnosed JDM and correlates with elevated serum aldolase.

Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA)

BackgroundThere is no standardized approach to the management of JDM-associated calcinosis and its phenotypes. Current knowledge of treatment outcomes is confined to small series and case reports. We describe physician perspectives toward diagnostic approach, classification and treatment directly targeting calcinosis, independent of overall JDM therapy.MethodsAn electronic survey of 22 questions was organized into sections regarding individual practices of assessment, classification and treatment of calcinosis, including perceived successes of therapies. Invitations to complete the survey voluntarily and anonymously were sent to CARRA physician members and the Pediatric Rheumatology Bulletin Board, an electronic list-serv. Results were analyzed by descriptive statistics and chi-square analyses.ResultsOf 139 survey responses, 118 were included in analysis. Of these, 70% were based in the USA and 88 (75%) were CARRA members. Only 17% of responders have seen more than 20 cases of calcinosis, and only 28% perform screening imaging studies on new JDM diagnoses. Increasing systemic immunosuppression is first-line therapy for 67% of respondents. Targeted therapy against calcinosis is most often instituted for symptomatic patients. IVIG and bisphosphonates are most frequently used and considered most successful, but many other agents are used. Experienced physicians are more likely to use bisphosphonates, calcium channel blockers and topical sodium thiosulfate (p< 0.002 or lower).ConclusionsCoexisting JDM disease activity influences whether calcinosis is considered active disease or targeted directly. Experience treating JDM-related calcinosis is low, as are rates of formal screening for calcinosis. Experienced physicians are more likely to use non-immunosuppressive treatments.

A case of juvenile dermatomyositis with esophago-pleural fistula caused by spontaneous lower esophageal perforation

Juvenile dermatomyositis (JDM) is a multisystem disease of uncertain origin that results in nonsuppurative inflammation of striated muscle and skin. Gastrointestinal perforation including esophagus is a well-recognized complication of JDM. A one year and nine months old girl with JDM was recently seen with esophago-pleural fistula. The fistula was caused by spontaneous lower esophageal perforation near the gastro-esophageal junction. The treatment consisted of adequate drainage of perforation site, total parenteral nutrition, long-term intravenous hydrocortisone, and broad-spectrum antibiotics. Although the duration of the fistula closing was long, the fistula spontaneously obliterated without other surgical management. This is a rare case of JDM with esophago-pleural fistula caused by spontaneous lower esophageal perforation.

Calcinosis as a complication of juvenile dermatomyositis (JDM)

Results 17/42 developed calcinosis associated with earlier onset of the disease, male predominance and chronic. Calcium deposits were classified as: superficial nodular, deep nodular, mass, linear or mixed deposits and calcinosis universalis. 80% had two or more types of deposits. The anatomical areas more frequently affected were the thighs and forearms. Complications included: drainage, chronic ulcers, mass effect, mechanical blockage of the joints and infections. Severe calcinosis was also associated with significant muscle atrophy loss and lipodystrophy.

A case of juvenile dermatomyositis with esophago-pleural fistula caused by spontaneous lower esophageal perforation

Juvenile dermatomyositis (JDM) is a chronic multisystem disease characterized by nonsuppurative inflammation of striated muscle and skin. Gastrointestinal perforation including esophagus is a well-recognized complication of JDM. Corticosteroid therapy in JMD may be the cause of the ulceration and perforation of the gastrointestinal tract regardless of the diseasecourse. We describe a rare case of a one year and nine months old girl with juvenile dermatomyositis (JDM) who developed spontaneous lower esophageal perforation with esophago-pleural fistula. Although the duration of the fistula closing was long, the fistula spontaneously obliterated without other surgical management. This is a rare case of JDM with esophago-pleural，fistula caused by spontaneous lower esophageal perforation

Lipodystrophy and Dermatomyositis in Kids

Lipodystrophy -- a clinical condition characterized by loss of subcutaneous fat -- has been recognized as a complication of juvenile dermatomyositis (JDMS). In addition, lipodystrophy is associated with hirsutism, acanthosis nigricans, insulin resistance, and hyperlipidemia. These investigators examined 20 patients with JDMS for clinical and metabolic abnormalities associated with lipodystrophy. The 20 patients were categorized and assigned to 3 groups: In group 1 were 4 patients with lipodystrophy. Group 2 contained …

Streptococcal myositis as a complication of juvenile dermatomyositis

The infection of muscle is an infrequent condition. We report on a patient with a juvenile form of dermatomyositis who developed infectious myositis caused by Streptococcus pyogenes. The inflammatory myopathy probably favoured the colonization of muscle during a bacteremia related to the skin lesions. The main forms of streptococcal myositis, which can currently be differentiated by means of imaging techniques, are discussed in addition to its treatment and prognosis.

Calcinosis Cutis in Juvenile Dermatomyositis: Remarkable Response to Aluminum Hydroxide Therapy

<h3>To the Editor.—</h3> Calcinosis cutis may be the most troublesome and debilitating complication in juvenile dermatomyositis. The pathophysiology of this peculiar state is unknown. Treatment, so far, remains unsatisfactory. We report a case of juvenile dermatomyositis with calcinosis cutis that showed remarkable response to orally administered aluminum hydroxide. <h3>Report of a Case.—</h3> A 13-year-old girl was admitted to our department in August 1986. The patient suffered from multiple erythematous, slightly elevated, papular eruptions over the cheek, trunk, both arms, and both legs, associated with proximal muscle weakness and fever, at the age of 2 years. The diagnosis of juvenile dermatomyositis was made in another hospital, and the patient was treated with low-dose orally administered prednisolone thereafter. In 1982, a flare-up of skin lesions occurred and was followed one year later by multiple yellow-white nodules and plaques protruding over the forehead, back, and upper extremities. On examination, the child appeared with

Spontaneous pneumothorax: A complication of juvenile dermatomyositis

Spontaneous pneumothorax: A complication of juvenile dermatomyositis