A descriptive and prognostic study of systemic sclerosis-associated myopathies

Ranque, Brigitte; Authier, François‐Jérôme; Le-Guern, Véronique; Pagnoux, Christian; Bérezné, Alice; Allanore, Yannick; Launay, David; Hachulla, É.; Kahan, André; Cabané, J.; Gherardi, Romain K.; Guillevin, Loı̈c; Mouthon, Luc

doi:10.1136/ard.2008.095919

Cited by 90 publications

(79 citation statements)

References 14 publications

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“…This treatment, which is usually well tolerated, may be prescribed intravenously at 0.6 gm/m 2 /month for 6 -12 months or orally at 1 mg/kg/day for 1 year. Again, among the many adverse events associated with cyclophosphamide therapy, the risk of infection is increased, particularly in (20,42) and/or skin involvement in patients with dcSSc (43), may result in mouth ulcers, although these ulcers usually occur only in the absence of folic acid treatment. These ulcers generally require local treatment, usually with antiseptic agents.…”

Section: Resultsmentioning

confidence: 99%

Recommendations for the care of oral involvement in patients with systemic sclerosis

Alantar¹,

Cabané²,

Hachulla³

et al. 2011

Arthritis Care & Research

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Objective. To review the literature and collect expert advice for proposing preventive and curative treatments of mouth and dental involvement in patients with systemic sclerosis (SSc; scleroderma). Methods. The literature pertaining to mouth and/or dental involvement related to SSc was reviewed, and recommendations were developed according to the suggestions of a French multidisciplinary working group of experts and validated by a lecture committee. Results. Dentists face 3 main issues in caring for SSc patients: oral mucosa involvement, manducatory apparatus and mouth involvement responsible for limitations in mouth opening, and treatment-related adverse events. An increased risk of tongue carcinoma has been noted. In patients with severe limitation in mouth opening (<30 mm), recommended treatments are a specific mouth-opening rehabilitation program, flexible sectional dentures, and splint therapy. Indications for dental implants depend on the severity of SSc, comorbidities, and/or ongoing bisphosphonate treatment. Prevention of mouth infections and caries implies patient education and teaching about mouth and dental hygiene, periodontal maintenance, and treatment of sicca syndrome. Cessation of tobacco use is mandatory. Patient-tailored rehabilitation may improve limitations in mouth opening. Systematic dental panoramic radiography allows for the early detection of dental caries. Conclusion. Prevention of oral and dental complications is a major issue in patients with SSc. Dental treatment should be tailored to limitations in mouth opening, disease severity, and ongoing treatments.

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Section: Resultsmentioning

confidence: 99%

Recommendations for the care of oral involvement in patients with systemic sclerosis

Alantar¹,

Cabané²,

Hachulla³

et al. 2011

Arthritis Care & Research

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“…Skeletal muscle disease in scleroderma is common, with a prevalence of 13-81% depending on the criteria used to define muscle involvement (1)(2)(3)(4)(5)(6)(7). Some studies have defined muscle disease by subjective proximal weakness only, while more rigorous investigations require electromyography and muscle biopsies to define muscle disease (8). One study reported that muscle involvement in scleroderma as a poor prognostic feature impacting survival, especially those of male gender, or early diffuse disease, or interstitial lung disease (9).…”

Section: Introductionmentioning

confidence: 99%

Independent Association of Severity of Muscle Weakness With Disability as Measured by the Health Assessment Questionnaire Disability Index in Scleroderma

Paik

Wigley

Mejia

et al. 2016

Arthritis Care & Research

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“…The main pathological features of systemic sclerosisassociated myopathies are mononuclear inflammation (63%), muscle atrophy (60%), necrosis (59%), regeneration (44%), fibrosis (24%), and microangiopathy (27%). The histological muscle inflammation seemed to be associated with good response to corticosteroids and better muscle prognosis, however myopathy without inflammation such as atrophy and fibrosis might have poor muscle prognosis [15]. Discrimination between myopathy and myositis with systemic sclerosis is clinically important to decide the therapeutic choice, because steroid therapy may be effective for myositis but ineffective for myopathy without inflammation.…”

Section: Discussionmentioning

confidence: 99%

“…The prevalence of muscle involvements in patients with systemic sclerosis varies from 14% to 81% [15]. The main pathological features of systemic sclerosisassociated myopathies are mononuclear inflammation (63%), muscle atrophy (60%), necrosis (59%), regeneration (44%), fibrosis (24%), and microangiopathy (27%).…”

Section: Discussionmentioning

confidence: 99%

A case of sarcoidosis developing as sarcoid myopathy concomitant with systemic sclerosis and review of the literature

et al. 2011

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A 65-year-old man was diagnosed with systemic sclerosis on the basis of skin thickening and positivity of anti-Scl-70 antibodies. Because myogenic enzymes, such as creatinine phosphokinase and aldorase, were also elevated, myopathy or myositis associated with systemic sclerosis was considered. Muscle magnetic resonance imaging and gallium scintigraphy did not show abnormalities. Findings of muscle biopsy demonstrated presence of noncaseating granulomas with multinucleated giant cells. In addition, serum angiotensin-converting enzyme and lysozyme were elevated, and therefore a diagnosis of sarcoid myopathy was made. Further, renal sarcoidosis was revealed with renal biopsy. Prednisolone (40 mg/day) improved both the myopathy and nephritis. Sarcoid myopathy is a rare condition, but it should be considered when myogenic enzymes are elevated in the patient with systemic sclerosis. Further, muscle biopsy may be essential to make an accurate diagnosis in such condition.

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A descriptive and prognostic study of systemic sclerosis-associated myopathies

Abstract: Muscle histopathology is critical in the therapeutic management of SSc-associated myopathy.

Cited by 90 publications

References 14 publications

Recommendations for the care of oral involvement in patients with systemic sclerosis

Recommendations for the care of oral involvement in patients with systemic sclerosis

Independent Association of Severity of Muscle Weakness With Disability as Measured by the Health Assessment Questionnaire Disability Index in Scleroderma

A case of sarcoidosis developing as sarcoid myopathy concomitant with systemic sclerosis and review of the literature

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