Anasarca: not a nephrotic syndrome but dermatomyositis

Abstract

Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscle, connective tissue, skin, gastrointestinal tract, and small nerves. Periorbital and facial edema may also be associated. Although localized edema is a common feature of JDM, generalized edema has rarely been reported. Here, we report a 3.5-year-old boy with JDM presenting with generalized edema. The diagnostic criteria of JDM rely on typical clinical manifestations that include: severe symmetric weakness of the proximal musculature, characteristic cutaneous changes, elevated serum skeletal muscle enzymes, and myopathic electromyographic pattern. Our patient initially received methylprednisolone and intravenous immunoglobulin (IVIG) without significant improvement, so he was given azathioprine and a prolonged course of oral prednisolone. We conclude that JDM should be suspected in patients presenting with anasarca in the absence of laboratory parameters of other causes of generalized edema and an appearance of heliotrope rash with muscle weakness. Also, we suggest that muscle magnetic resonance imaging (MRI) should be considered among the diagnostic tools of JDM.

Polimiosite associada à síndrome nefrótica

“…On the other hand, there are reports on patients with idiopathic inflammatory myopathies (DM, PM) who developed anasarca not related to nephrotic syndrome. 13,14 Gorelik et al, 15 for example, found seven cases of DM and PM related to anasarca in adult males, as described in the episode, and they also reported dysphagia in four of these patients. However, none of these patients progressed to nephrotic syndrome.…”

Severe macrophage activation syndrome and central nervous system involvement in juvenile dermatomyositis

“…We read with great interest the article by Dong et al on 'Upregulation of 78-kDa glucose-regulated protein in macrophages in peripheral joints of active ankylosing spondylitis' (1). In this work, Dong et al found that the endoplasmic reticulum (ER) chaperone glucoseregulated protein 78 (GRP78) is highly expressed in macrophages in the affected peripheral joints of ankylosing spondylitis (AS) compared with osteoarthritis (OA) patients.…”

“…Periorbital oedema may be associated with this disease but generalized oedema is uncommon (1). Central nervous system (CNS) involvement and macrophage activation syndrome (MAS) are rarely reported in JDM (1,2). We present a case with both complications and an unusual presentation with severe generalized oedema.…”

Anasarca as the initial presentation of juvenile polymyositis: an uncommon occurrence

“…The striking feature in our case was anasarca. Such a presentation has been described with dermatomyositis before but not with polymyositis [4,5]. The weakness along with edema as well as the inability to get up from lying down position, indicating neck muscle involvement prompted us to suspect myositis.…”

“…It is suggested that as a sequel to diffuse and widespread capillary endothelial damage, there is increase in capillary permeability in muscles leading to generalized edema [6]. At the presentation, initially, these cases may be misdiagnosed as nephritic syndrome [4,5].…”