Scleromyositis: a scleroderma/polymyositis overlap syndrome

Jabłońska, S; Błaszczyk, M

doi:10.1007/bf01451281

Cited by 38 publications

(25 citation statements)

References 22 publications

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“…Scleromyositis is an overlapping syndrome clinically characterized by scleroderma, Raynaud's disease, arthralgia/arthritis, myositis or myalgia, interstitial lung disease, and hyperkeratotic rhagadiform eczema of the fingers ("mechanic's hands") [5][6][7]. Immunological findings involve PM-Scl autoantibody positivity [5,[8][9][10].…”

Section: Discussionmentioning

confidence: 98%

Dropped head syndrome as a presenting sign of scleromyositis

Garcin

Lenglet

Dubourg

et al. 2010

Journal of the Neurological Sciences

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Section: Discussionmentioning

confidence: 98%

Dropped head syndrome as a presenting sign of scleromyositis

Garcin

Lenglet

Dubourg

et al. 2010

Journal of the Neurological Sciences

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“…4,5 In fact, this particular overlap syndrome is considered a distinct clinical entity, scleromyositis. 6 Criteria for each autoimmune disease must be fulfilled. The diagnosis of polymyositis is based on the Bohan and Peter 7 criteria: (1) symmetric proximal muscle weakness, (2) myositis on biopsy, (3) increased serum muscle enzymes, and (4) characteristic EMG pattern.…”

Section: Discussionmentioning

confidence: 99%

Clinical Reasoning: A 49-year-old woman with progressive motor deficit

Monteiro

Mendes

Silveira³

et al. 2014

Neurology

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A previously healthy 49-year-old woman presented with progressive motor deficit. The complaints started the year before with weakness of the right arm. Over the subsequent months, she developed weakness in the left arm, followed by both legs, and, finally, difficulty speaking, with nasal voice, and swallowing. It was increasingly difficult to attend to her chores, and, by the time she sought medical attention, she needed help with all daily activities. In the last few weeks, she also complained of diffuse joint and muscle pain. Medical and family history were unremarkable.Neurologic examination showed bilateral facial weakness, severe dysarthria, dysphonia and dysphagia (nauseous reflex preserved), decreased shoulder elevation strength, and difficulty protruding the tongue, without fasciculations or atrophy. Symmetrical tetraparesis (proximal-greater-than-distal weakness) and increased tone were noted, with severe pain upon mobilization and palpation of joints and muscles. Deep tendon reflexes were brisk and symmetric, with bilateral flexor plantar responses. There was atrophy of the interosseous muscles of the hands and shoulder girdle muscle wasting.Questions for consideration:

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“…This antibody correlates strongly with the syndrome 8,9 and results in a nucleolar antinuclear antibody pattern 8,10 . Although it can be observed in isolated DM/JDM or SSc, 80% of patients with anti-PM/Scl antibodies will have overlap SSc/myositis syndrome 4 . PM/Scl antibodies persist regardless of disease activity, but do not appear to correlate with disease activity 2 .…”

Section: Four Cases Of Anti-pm/scl Antibody-positive Juvenile Overlapmentioning

confidence: 99%

“…Relatively few cases of childhood SSc/myositis overlap have been reported 1,2,3,4,5,6,7 . In our case series, 4 patients diagnosed with juvenile overlap syndrome of myositis with SSc are described ( Table 1).…”

Section: Four Cases Of Anti-pm/scl Antibody-positive Juvenile Overlapmentioning

confidence: 99%