O97 Polymyositis and dermatomyositis: short-term and long-term outcome, and predictive factors of prognosis

Marie, I.; Hachulla, É.; Chérìn, P.; Mf, Hellot; Hatron, P.Y.; Herson, S.; Lévesque, H.; Courtois, H

doi:10.1016/s0953-6205(03)91246-5

Cited by 132 publications

(264 citation statements)

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“…PM/DM has been reported to be associated with increased mortality, with mortality rates ranging from 4% to 50% (1)(2)(3)(4)(5). Malignancy, pulmonary and cardiac involvement, and infections are generally cited as the most common causes of death in PM/DM patients (1)(2)(3)(4)(5).…”

Section: Marie Et Almentioning

confidence: 99%

“…Polymyositis (PM) and dermatomyositis (DM) are systemic inflammatory disorders affecting skeletal muscles and other organs (1)(2)(3)(4)(5). PM and DM are considered to be associated with high morbidity and mortality rates, in some cases as high as 50%, primarily related to life-threatening muscle weakness, cardiac and lung impairment, as well as infectious manifestations (1)(2)(3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

“…PM and DM are considered to be associated with high morbidity and mortality rates, in some cases as high as 50%, primarily related to life-threatening muscle weakness, cardiac and lung impairment, as well as infectious manifestations (1)(2)(3)(4)(5). In clinical series of patients with PM/DM, infectious complications have been described in up to 26% of patients (1,2,4,6). Several factors may be implicated in this apparent increased frequency of infections in PM/DM patients, particularly immunosuppressive medications.…”

Section: Introductionmentioning

confidence: 99%

“…Several factors may be implicated in this apparent increased frequency of infections in PM/DM patients, particularly immunosuppressive medications. In addition, immune system dysfunction, due to PM/DM itself, may lead to elevated susceptibility to opportunistic infections (1,2,4,5).…”

Section: Introductionmentioning

confidence: 99%

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Opportunistic infections in polymyositis and dermatomyositis

Marie¹,

Hachulla²,

Chérìn³

et al. 2005

Arthritis & Rheumatism

166

126

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Objective. To assess prevalence and characteristics of opportunistic infections in patients with polymyositis/dermatomyositis (PM/DM). To determine the predictive values for opportunistic infections on clinical presentation, biochemical findings, and paraclinical features of PM/DM to detect patients at risk of opportunistic infections. Methods. The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. Results. Eighteen PM/DM patients (11.5%) developed opportunistic infections. The majority of patients exhibited an opportunistic infection after the onset of PM/DM (89% of cases). Opportunistic infections occurred most frequently during the first year following PM/DM diagnosis (62.5%). The pathogen microorganisms responsible for opportunistic infections were various, i.e., Candida albicans, Pneumocystis carinii, Aspergillus fumigatus, Geotrichum capitatum, Mycobacterium avium-intracellulare complex, M. xenopi, M. marinum, M. tuberculosis, Helicobacter heilmanii, cytomegalovirus, and herpes simplex virus. Mortality rates were as high as 27.7% in these PM/DM patients. Higher mean daily doses of steroids, lymphopenia, and lower serum total protein levels were significantly more frequent in the group of PM/DM patients with opportunistic infections. Conclusion. Our study underscores the high frequency of opportunistic infections in PM/DM, resulting in an increased mortality rate. It also indicates that a great variety of microorganisms are responsible for opportunistic infections, although they were more often due to fungi (>50% of cases). Our series highlights a predominance of both lung and digestive opportunistic infections (89% of cases). In addition, our results suggest that PM/DM patients presenting with factors predictive of opportunistic infection may require closer monitoring.

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Section: Marie Et Almentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Opportunistic infections in polymyositis and dermatomyositis

Marie¹,

Hachulla²,

Chérìn³

et al. 2005

Arthritis & Rheumatism

166

126

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“…If this treatment fails (because of too small an effect, repeated relapses, or unacceptable side effects) various second line treatments are in use, but most of these have not been appropriately investigated [2][3][4]. Despite current therapies, outcome is poor in many patients [5,6]. New drugs are emerging, but improvements in treatment are hampered by difficulties in the design of trials and the low incidence and prevalence of patients.…”

Section: Introductionmentioning

confidence: 99%

119th ENMC international workshop: Trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10–12 October 2003, Naarden, The Netherlands

Hoogendijk

Amato

Lecky

et al. 2004

Neuromuscular Disorders

806

602

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Intravenous immunoglobulins for steroid‐refractory esophageal involvement related to polymyositis and dermatomyositis: A series of 73 patients

Marie¹,

Ménard²,

Hatron³

et al. 2010

Arthritis Care & Research

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132

108

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Objective. To assess the long-term outcome of esophageal complications in the group of patients receiving intravenous immunoglobulins (IVIG) for the treatment of severe steroid-refractory esophageal involvement related to polymyositis/ dermatomyositis (PM/DM). Methods. We retrospectively reviewed the medical records of 73 patients (39 with PM, 34 with DM) with steroid-resistant esophageal involvement. Esophageal involvement was evaluated by clinical and manometric investigations. Results. Seventy-three patients with steroid-refractory esophageal involvement related to PM/DM received IVIG therapy (2 gm/kg monthly). The median interval between PM/DM diagnosis and the onset of esophageal complications was 6 months. The most common clinical manifestations revealing esophageal dysfunction were dysphagia (69.9%), coughing while eating (61.6%), and gastroesophageal reflux into the pharynx and/or mouth (34.2%). Twenty-five patients exhibited life-threatening esophageal complications requiring exclusive enteral feeding; 33 patients (45.2%) with esophageal impairment developed aspiration pneumonia. Sixty patients (82.2%) exhibited resolution of esophageal clinical manifestations, leading to a return to normal oral feeding and ablation of feeding enteral tubes. Four other patients (5.5%) improved, although they still experienced mild dysphagia intermittently. Because of impaired cricopharyngeal muscle relaxation, another patient successfully underwent cricopharyngeal myotomy. Eight patients died from aspiration pneumonia (n ‫؍‬ 6) and cancer (n ‫؍‬ 2). Muscle weakness, thoracic myopathy, and aspiration pneumonia were independent predictive factors of IVIG-treated esophageal complications in PM/DM patients. Conclusion. Our findings indicate that IVIG should be considered in life-threatening esophageal impairment complicating steroid-resistant PM/DM. We also suggest that combined therapy of IVIG and high-dose steroids may be the first-line therapy in PM/DM patients with life-threatening esophageal manifestations.

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O97 Polymyositis and dermatomyositis: short-term and long-term outcome, and predictive factors of prognosis

Cited by 132 publications

References 0 publications

Opportunistic infections in polymyositis and dermatomyositis

Opportunistic infections in polymyositis and dermatomyositis

119th ENMC international workshop: Trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10–12 October 2003, Naarden, The Netherlands

Intravenous immunoglobulins for steroid‐refractory esophageal involvement related to polymyositis and dermatomyositis: A series of 73 patients

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