Life-threatening systemic capillary leak syndrome in juvenile dermatomyositis

Meneghel, Alessandra; Martini, Giorgia; Birolo, Carolina; Tosoni, Alvise; Pettenazzo, Andrea; Zulian, Francesco

doi:10.1093/rheumatology/kex262

Cited by 8 publications

(5 citation statements)

References 7 publications

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“…In a recently reported French JDM cohort of 116 patients, vasculopathy-related complications were the main cause of admission to the intensive care unit, illustrating the severity and relevance of vascular involvement in JDM (46). These complications include life-threatening disorders like systemic capillary leak syndrome, recently also described in 3 patients with JDM (47).…”

Section: Signs Of Systemic Disease Activity In Jdm Based On Affected mentioning

confidence: 99%

Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools

et al. 2018

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Juvenile Dermatomyositis (JDM) is a systemic immune-mediated disease of childhood, characterized by muscle weakness, and a typical skin rash. Other organ systems and tissues such as the lungs, heart, and intestines can be involved, but may be under-evaluated. The inflammatory process in JDM is characterized by an interferon signature and infiltration of immune cells such as T cells and plasmacytoid dendritic cells into the affected tissues. Vasculopathy due to loss and dysfunction of endothelial cells as a result of the inflammation is thought to underlie the symptoms in most organs and tissues. JDM is a heterogeneous disease, and several disease phenotypes, each with a varying combination of affected tissues and organs, are linked to the presence of myositis autoantibodies. These autoantibodies have therefore been extensively studied as biomarkers for the disease phenotype and its associated prognosis. Next to identifying the JDM phenotype, monitoring of disease activity and disease-inflicted damage not only in muscle and skin, but also in other organs and tissues, is an important part of clinical follow-up, as these are key determinants for the long-term outcomes of patients. Various monitoring tools are currently available, among which clinical assessment, histopathological investigation of muscle and skin biopsies, and laboratory testing of blood for specific biomarkers. These investigations also give novel insights into the underlying immunological processes that drive inflammation in JDM and suggest a strong link between the interferon signature and vasculopathy. New tools are being developed in the quest for minimally invasive, but sensitive and specific diagnostic methods that correlate well with clinical symptoms or reflect local, low-grade inflammation. In this review we will discuss the types of (extra)muscular tissue inflammation in JDM and their relation to vasculopathic changes, critically assess the available diagnostic methods including myositis autoantibodies and newly identified biomarkers, and reflect on the immunopathogenic implications of identified markers.

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Section: Signs Of Systemic Disease Activity In Jdm Based On Affected mentioning

confidence: 99%

Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools

et al. 2018

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“…PM and DM are frequently associated with systemic sclerosis and mixed connective tissue disease in the context of an overlap syndrome [ 184 , 185 ]. It has also been reported that several cases of JDM have been complicated by systemic capillary leak syndrome (SCLS), a rare, life-threatening disorder characterized by severe hypotension, hypoalbuminaemia and hemoconcentration [ 186 ]. Lipodystrophy, hypertriglyceridemia and insulin resistance have also been associated with JDM [ 187 , 188 ].…”

Section: Clinical Featuresmentioning

confidence: 99%

Polymyositis and dermatomyositis – challenges in diagnosis and management

Yang

Chang

Lian

2019

Journal of Translational Autoimmunity

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Polymyositis (PM) and dermatomyositis (DM) are different disease subtypes of idiopathic inflammatory myopathies (IIMs). The main clinical features of PM and DM include progressive symmetric, predominantly proximal muscle weakness. Laboratory findings include elevated creatine kinase (CK), autoantibodies in serum, and inflammatory infiltrates in muscle biopsy. Dermatomyositis can also involve a characteristic skin rash. Both polymyositis and dermatomyositis can present with extramuscular involvement. The causative factor is agnogenic activation of immune system, leading to immunologic attacks on muscle fibers and endomysial capillaries. The treatment of choice is immunosuppression. PM and DM can be distinguished from other IIMs and myopathies by thorough history, physical examinations and laboratory evaluation and adherence to specific and up-to-date diagnosis criteria and classification standards. Treatment is based on correct diagnosis of these conditions.

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“…Although the cause and effect relationship between C1 inhibitor deficiency and dermatomyositis is not clear, C1 inhibitor deficiency will result in loss of inhibition of the classical complement pathway, leading to low serum C4 levels as a result of excessive consumption, and an activated complement system can induce inflammatory myopathy by deposition of the membrane attack complex in muscle tissues 33 . C1 inhibitor deficiency and life‐threatening, systemic capillary leak syndrome have been reported in a small number of pediatric cases, characterized by hypotension, hemoconcentration, hypoalbuminemia, hyponatremia and edema 26,34,35 . Also, generalized subcutaneous edema and anasarca have been reported as the presenting clinical findings in a few JDM cases 17,25,36 .…”

Section: Discussionmentioning

confidence: 99%

“…33 C1 inhibitor deficiency and life-threatening, systemic capillary leak syndrome have been reported in a small number of pediatric cases, characterized by hypotension, hemoconcentration, hypoalbuminemia, hyponatremia and edema. 26,34,35 Also, generalized subcutaneous edema and anasarca have been reported as the presenting clinical findings in a few JDM cases. 17,25,36 In these cases, pathogenesis is not certain, as C1 inhibitor levels were not reported and C4 level was only reported in some cases.…”

Section: Ta B L E 1 (Continued)mentioning

confidence: 99%

Angioedema‐like presentation as the presenting finding of juvenile myositis and juvenile dermatomyositis in 2 patients

Karaca

Güngör

Güneş³

et al. 2022

Int J of Rheum Dis

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Background Juvenile dermatomyositis (JDM) is the most common subtype of idiopathic inflammatory myopathies in childhood. Gottron’s papules, shawl sign, periorbital heliotrope rash, and periungual telengiectasis are characteristic skin findings of the disease. Besides characteristic skin involvement, some other skin findings, such as angioedema, may be seen prior or in the course of the disease. The presence of angioedema in JDM is emphasized in this report. Case presentations We present 2 unrelated girls, aged 2 (case 1) and 12 years (case 2), who had developed symmetrical weakness in the proximal muscles, muscle pain, elevated muscle enzymes and angioedema. Both cases had abnormal muscle magnetic resonance imaging findings, suggestive of inflammatory myositis. Muscle biopsy was performed only in case 1, and major histocompatibility complex‐1 expression on myofibers was shown consistent with JDM. Cases were diagnosed as probable and definite JDM, respectively. Angioedema was prominent, particularly in the lips and extremities of both cases, without laboratory evidence of C1 inhibitor deficiency or capillary leak syndrome, and absence of family history. Mast cell‐mediated, acquired angioedema was the most likely diagnosis. In both cases, skin and muscle findings improved significantly with steroid treatment. Conclusion We suggest that angioedema may be among the characteristic skin findings in JDM, and may be included in subsequent definitions.

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Life-threatening systemic capillary leak syndrome in juvenile dermatomyositis

Cited by 8 publications

References 7 publications

Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools

Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools

Polymyositis and dermatomyositis – challenges in diagnosis and management

Angioedema‐like presentation as the presenting finding of juvenile myositis and juvenile dermatomyositis in 2 patients

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