Overlap between dermatomyositis and ANCA vasculitides

Yuste, Claudia; Rapalai, M.; Pritchard, Benjamin A.; Jones, Terence J.; Amoasii, Constanza; Alansari, A.; Ramakrishna, Satish

doi:10.1093/ckj/sft142

Cited by 10 publications

(6 citation statements)

References 19 publications

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“…The mechanism of peripheral nervous system involvement in patients with DM is assumed to involve vascular endothelial injury in the peripheral nerves and overproduction of vascular endothelial growth factor . The occurrence of systemic vasculitis in other organs in patients with DM supports such a mechanism . Our literature survey of the nerve conduction and sural nerve biopsy studies in patients with DM‐related neuropathy mostly suggested axonal neuropathy, which is also consistent with a vascular mechanism .…”

Section: Discussionsupporting

confidence: 61%

Dermatomyositis complicated with asymmetric peripheral neuritis on exacerbation: A case report and literature review

Irie

Sekiya

Koge

et al. 2016

Clinical & Exp Neuroim

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We describe a 69-year-old woman with dermatomyositis who presented with asymmetric peripheral neuritis. The patient first developed dermatomyositis at 39 years-of-age; her symptoms improved several years later with oral prednisolone treatment. A year and a half before admission to our hospital, she developed a sensory disturbance in her feet that gradually expanded to both legs with left dominance. Six months later, bilateral leg muscle weakness and proximal muscle tenderness appeared. Neurological examination at admission revealed proximal muscle weakness and atrophy of all four limbs. Ankle jerk was absent bilaterally. There was sensory impairment in the left hand and both lower limbs, with more severe involvement of the left side and the medial side. Electrophysiological tests showed decreased sensory nerve action potentials in the left superficial fibular nerve and bilateral sural nerves, and decreased motor action potentials in the bilateral fibular nerves. We diagnosed mononeuritis multiplex as a complication of dermatomyositis. Her symptoms of mononeuritis multiplex and dermatomyositis were successfully treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone therapy. Although our literature search found nine cases of dermatomyositis with polyneuropathy/ polyradiculoneuropathy, most of which involved axonal neuropathy, this is the first case report of dermatomyositis presenting with mononeuritis multiplex.

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Section: Discussionsupporting

confidence: 61%

Dermatomyositis complicated with asymmetric peripheral neuritis on exacerbation: A case report and literature review

Irie

Sekiya

Koge

et al. 2016

Clinical & Exp Neuroim

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“…We conducted an additional literature search and retrieved case reports describing the association of PM or DM with a variety of pathological glomerular lesions, including mesangial proliferative glomerulonephritis [21][22][23][24][25], membranous nephropathy [26][27][28][29][30][31], membranoproliferative glomerulonephritis [32], IgA nephropathy [33][34][35][36][37], diffuse proliferative glomerulonephritis [38], immune complex-type glomerulonephritis [39][40][41][42], acute interstitial nephritis [43], lipoid nephrosis and focal glomerulosclerosis [44], focal segmental glomerulosclerosis with evidence of acute tubular injury [45], sclerotic GN with fibrocellular crescent [46], and crescentic glomerulonephritis associated with malignancy [47]. Cases presenting with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in the context of DM have also been reported [48][49][50]. As pointed out in previous studies [6,25], there is a tendency for mesangial proliferative glomerulonephritis to be more frequently observed in PM, while membranous nephropathy is more frequently observed in the context of DM.…”

Section: Discussionmentioning

confidence: 99%

Nephrotic Syndrome as an Extramuscular Manifestation of Anti-EJ Antibody-Positive Dermatomyositis: A Case Report and Review of the Literature

Tsubouchi

Mizuuchi

Yamamoto

et al. 2022

Case Reports in Rheumatology

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Renal involvement is underestimated as an extramuscular manifestation of dermatomyositis (DM). Here, we describe a 67-year-old woman with anti-glycyl-transfer ribonucleic acid synthetase (anti-EJ) antibody and anti-ribonucleoprotein antibody-positive DM complicated by systemic sclerosis, who developed nephrotic syndrome concurrently with the exacerbation of DM, as indicated by incremental serum creatine kinase levels, high-intensity lesions on muscle magnetic resonance imaging, and active interstitial pneumonitis on chest computed tomography. Renal biopsy revealed the presence of immune-deposition in the glomerulus by immunofluorescence. To our knowledge, this is the first report describing the coexistence of anti-EJ antibody-positive DM and nephrotic syndrome. More reports of similar cases are warranted to substantiate the association.

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“…Both prior patients also initially presented with dermatomyositis and minimal, if any, overt inflammatory muscle pathology. The patient described by Kawai et al (1), in 2011, had a 10-year history of dermatomyositis, while the patient described by Yuste et al (2), in 2014, developed both diseases within months of each other. As in the case presented here, both patients were p-AN-CA/MPO-positive with confirmatory renal biopsies and without documentation of pulmonary involvement.…”

Section: Discussionmentioning

confidence: 99%

“…To our knowledge, there have been only two other reported cases of patients with dermatomyositis who subsequently developed an AN-CA-associated vasculitis (AAV) (1,2). Both prior patients also initially presented with dermatomyositis and minimal, if any, overt inflammatory muscle pathology.…”

Section: Discussionmentioning

confidence: 99%

Microscopic polyangiitis with dermatomyositis

Bauer

Brahn

2017

Eur J Rheumatol

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Dermatomyositis is a rare autoimmune disease with a heterogeneous presentation that often has multiple extramuscular manifestations, although it does not typically involve the renal function. A 62-year-old female presented with proximal muscle weakness and rashes, which are classic symptoms of dermatomyositis without creatine kinase (CK) elevation. Initial serologic evaluation revealed a positive p-ANCA, although she did not develop renal failure for several months, at which point renal biopsy findings were consistent with microscopic polyangiitis. The patient was initially treated with cyclophosphamide, maintained with rituximab, and has been in remission for more than 2 years. Dermatomyositis and microscopic polyangiitis are both uncommon diseases, but are concomitantly present in this patient. A positive p-ANCA and development of renal insufficiency should be promptly evaluated in dermatomyositis patients. more than 2.5 years after onset, with a normal p-ANCA and myeloperoxidase antibody.Written informed consent was obtained from the patient. DiscussionTo our knowledge, there have been only two other reported cases of patients with dermatomyositis who subsequently developed an AN-CA-associated vasculitis (AAV) (1, 2). Both prior patients also initially presented with dermatomyositis and minimal, if any, overt inflammatory muscle pathology. The patient described by Kawai et al. (1), in 2011, had a 10-year history of dermatomyositis, while the patient described by Yuste et al. (2), in 2014, developed both diseases within months of each other. As in the case presented here, both patients were p-AN-CA/MPO-positive with confirmatory renal biopsies and without documentation of pulmonary involvement. Table 2 summarizes disease courses and management. Dermatomyositis is a rare (incidence, 1.16-19 per million) and frequently heterogeneous disease characterized by proximal greater than distal muscle weakness, violaceous poikiloderma, typically involving the eyelids and malar area, anterior upper chest and back, extensor surfaces, and sun exposed areas (3). Many, but not all patients, will have elevation of muscle enzymes. Common extramuscular manifestations include polyarthritis, neuropathy, Raynaud's phenomenon, and interstitial lung disease (4). While AAV appears to be extremely rare in dermatomyositis patients, vasculitis of other organs is a known complication, especially in juvenile dermatomyositis patients. Vasculitis of the skin, lungs, gastrointestinal tract, testicles, muscle, cardiac, and central nervous systems has been reported (5-7).Microscopic polyangiitis is a necrotizing, pauci-immune, small-vessel vasculitis, commonly resulting in glomerulonephritis and pulmonary capillaritis in the absence of granulomatous inflammation (8). The incidence is estimated at 5.9 per million individuals (9). The majority of MPA patients are positive for ANCA (95%) and predominantly directed to MPO (70%) (10). Evidence increasingly suggests that ANCA/MPO may directly contribute to the pathogenesis of MPA (11, 12).ANC...

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Overlap between dermatomyositis and ANCA vasculitides

Cited by 10 publications

References 19 publications

Dermatomyositis complicated with asymmetric peripheral neuritis on exacerbation: A case report and literature review

Dermatomyositis complicated with asymmetric peripheral neuritis on exacerbation: A case report and literature review

Nephrotic Syndrome as an Extramuscular Manifestation of Anti-EJ Antibody-Positive Dermatomyositis: A Case Report and Review of the Literature

Microscopic polyangiitis with dermatomyositis

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