Distinctive features of idiopathic inflammatory myopathies inFrench Canadians

doi:10.1016/S0049-0172(96)80025-4

Seminars in Arthritis and Rheumatism

Volume 26, Issue 1, August 1996, Pages 447-458

https://doi.org/10.1016/S0049-0172(96)80025-4 Get rights and content

Abstract

This is the first report on idiopathic inflammatory myopathies (IIM) in FrenchCanadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4,13%), anti-U1RNP (n = 3,10%), and anti-Ro (n = 2,7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1, autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.

References (41)

SenécalJ.L. et al.
Autoantibodies to major and minor nuclear lamins are not restricted to autoimmune diseases
Clin Immunol Immunopathol
(1992)
HildebrandtS. et al.
Autoantibodies to topoisomerase I (Scl-70): Analysis by gel diffusion, immunoblot and enzyme-linked immunosorbent assay
Clin Immunol Immunopathol
(1990)
von MühlenC.A. et al.
Autoantibodies in the diagnosis of systemic rheumatic diseases
Semin Arthritis Rheum
(1995)
CroninM.E. et al.
Idiopathic inflammatory myopathies
Rheum Dis Clin North Am
(1990)
TargoffI.N.
Autoantibodies in polymyositis
Rheum Dis Clin North Am
(1992)
TargoffI.N.
Immune manifestations of inflammatory muscle disease
Rheum Dis Clin North Am
(1994)
RamirezG. et al.
Adult-onset polymyositis-dermatomyositis: Description of 25 patients with emphasis on treatment
Semin Arthritis Rheum
(1990)
HochbergM.C. et al.
Adult onset polymyositis/dermatomyositis: An analysis of clinical and laboratory features and survival in 76 patients with a review of the literature
Semin Arthritis Rheum
(1986)
LundbergI. et al.
A clinical, serological, and histopathological study of myositis patients with and without anti-RNP antibodies
Semin Arthritis Rheum
(1992)
MedsgerT.A. et al.
The epidemiology of polymyositis
Am J Med
(1970)

LakhanpalS. et al.

Polymyositis-dermatomyositis and malignant lesions: Does an association exist?

JoffeM.M. et al.

Drug therapy of the idiopathic inflammatory myopathies: Predictors of response to prednisone, azathioprine, and methotrexate and a comparison of their efficacy

Am J Med

(1993)

DalakasM.C.

Polymyositis, dermatomyositis, and inclusion-body myositis

N Engl J Med

(1991)

BohanA. et al.

Polymyositis and dermatomyositis

N Engl J Med

(1975)

BohanA. et al.

Polymyositis and dermatomyositis

N Engl J Med

(1975)

Senécal, JL, Chartier S, Rothfield N: Hypergamma-globulinemic purpura in systemic autoimmune rheumatic diseases:...

JoyalF. et al.

Evaluation of the severity of systemic sclerosis by nailfold capillary microscopy in 112 patients

Angiology

(1992)

DagenaisA. et al.

A novel autoantibody causing a peripheral fluorescent antinuclear antibody pattern is specific for nuclear pore complexes

Arthritis Rheum

(1988)

DagenaisA. et al.

Detection of a myosin heavy chain-like polypeptide at the nuclear pore complex

DabauvalleM.C. et al.

Nuclear pore complex structure analyzed by immunogold EM with human autoantibodies

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Polyarthritis is commonly reported in idiopathic inflammatory myositis patients, but few studies have focused on the overlap of myositis with rheumatoid arthritis which is a difficult diagnosis in the absence of well-defined diagnostic criteria. The primary objective of this scoping review was to map the field of research to explore the potential diagnoses in patients presenting with both myositis and polyarthritis.
Two electronic databases (MEDLINE/PubMed® and Web of Science®) were systematically searched using the terms (myositis OR ‘inflammatory idiopathic myopathies’) AND (polyarthritis OR ‘rheumatoid arthritis’) without any publication date limit.
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The spectrum of joint and muscle inflammatory diseases encompasses many diagnoses including primitive and secondary myositis associated with RA or arthritis mimicking RA. This review highlights the need for a consensual definition of OM with RA to better individualise this entity from the numerous differential diagnoses.
Clinical manifestations
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Las miopatías inflamatorias idiopáticas son un grupo de enfermedades de origen autoinmune que afectan al músculo estriado. Sin embargo, la gama de manifestaciones es múltiple, no sólo muscular. Este capítulo describe tanto las manifestaciones musculares como las características extramusculares de estos padecimientos.
Idiopathic inflammatory myopathies are a group of autoimmune diseases that characteristically affect striated muscle. Nevertheless their spectrum of manifestations is very broad, not only muscular. This chapter describes both muscular and extramuscular manifestations of inflammatory myopathies.
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Our study suggests that the prevalence of dysphagia is approximately 10% in inflammatory myopathy, although its exact prevalence could not be determined because not all the records of the 783 patients with inflammatory myopathy were reviewed in detail. The 10% frequency of dysphagia in our study patients is at the low end of the 10% to 73% range reported by several studies that ranged in size from 18 to 153 patients and included differing subsets of inflammatory myopathy.2–8,10 Similarly, our finding that dysphagia is a presenting symptom in 21% of the patients who experienced dysphagia is, again, at the low end of the range (25%, 32%, and 69%, respectively) reported by 3 smaller reports.6,9,13
To assess the clinical characteristics, treatment, and outcome of patients with inflammatory myopathy-associated dysphagia.
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A total of 783 patients were diagnosed as having inflammatory myopathy during the 5-year study period. Of these, 62 patients (41 women and 21 men; mean age, 68.6 years) had inflammatory myopathy-associated dysphagia: 26 with inclusion body myositis (IBM), 18 with dermatomyositis, 9 with polymyositis, and 9 with overlap syndrome. Dysphagia was a presenting symptom in 13 patients (21%), with the highest incidence in the IBM group. Videofluoroscopic examinations revealed pharyngeal pooling and impaired oropharyngeal and cricopharyngeal function. The benefits of swallowing compensation techniques and exercises were difficult to establish. Interventional procedures were performed in 24 patients (39%) and most frequently (62%) in patients with IBM, with cricopharyngeal myotomy being most beneficial. Patients with IBM had the least symptomatic improvement. Overall, 11 patients died during the median follow-up of 38 months, with respiratory failure due to aspiration pneumonia as the most common cause. Mortality was high in patients who required percutaneous endoscopic gastrostomy (7/11, 64%), and 1-year mortality was highest (31%) in those with dermatomyositis.
Dysphagia is a serious and at times presenting problem in patients with inflammatory myopathy. It occurs most frequently and appears to be most refractory in patients with IBM. The mortality rate was high in patients who required percutaneous endoscopic gastrostomy, and the 1-year mortality rate was the highest in patients with dermatomyositis.
Antisynthetase syndrome
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Se conoce como síndrome antisintetasa (SA) la asociación de miopatía inflamatoria idiopática, afección intersticial pulmonar, manos de mecánico, artritis no erosiva y la presencia de anticuerpos antisintetasas en suero. La etiología y la patogenia son desconocidas, y se ha implicado a agentes infecciosos, químicos, drogas y mecanismos autoinmunitarios. Se caracteriza también por una respuesta variable al tratamiento. Los anticuerpos antisintetasas son anticuerpos IgG dirigidos contra la enzima citoplásmica aminoacidil-ARNt sintetasa, que media la unión del ARN con un determinado aminoácido para formar el ARN transferente (ARNt). Se encuentran en el 25-35% de los pacientes con polimiositis/dermatomiositis y el más común es el antihistidil-ARNt (Jo-1), que está en el 20-30% de los casos.
La determinación de los anticuerpos antisintetasas permite definir un subgrupo dentro de las miopatías inflamatorias idiopáticas en que la afección pulmonar es un factor pronóstico determinante. Por tanto, en el abordaje diagnóstico de las miopatías inflamatorias y de las neumopatías intersticiales idiopáticas es necesario sistematizar la determinación de los anticuerpos antisintetasas para establecer un diagnóstico y un tratamiento precoces y mejorar así el pronóstico de estas enfermedades.
Antisynthetase syndrome is characterized by the association of idiopathic inflammatory myositis, pulmonary disease, mechanic's hands, nonerosive artrhitis and detection of antisynthetase antibodies. The etiology and pathogenesis are unknown, but chemical agents, drugs, bacterial and autoimmune mechanisms have been implied. The response to the treatment is variable. The target antigen of antisynthetase antibodies (IgG) are cytoplasmic RNA-t synthetases, enzyme that catalyse the covalent union between the RNA-t and its corresponding aminoacid. It is found in 25-35% of PM/DM patients. The anti-histidyl-RNAt-synthetase (anti-Jo-1) antibody is the most frecuently found myositis specific atibody, representing 20-30% of patients.
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^*: Supported in part by grants from The Arthritis Society ofCanada, Société de sclérose systémique du Québec and Lupus Québec. Dr Uthman is recipient of the Metro Ogryzlo International Fellowship Award.

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Distinctive features of idiopathic inflammatory myopathies inFrench Canadians*

Abstract

Clin Immunol Immunopathol

Clin Immunol Immunopathol

Semin Arthritis Rheum

Rheum Dis Clin North Am

Rheum Dis Clin North Am

Rheum Dis Clin North Am

Semin Arthritis Rheum

Semin Arthritis Rheum

Semin Arthritis Rheum

Am J Med

Am J Med

Polymyositis, dermatomyositis, and inclusion-body myositis

N Engl J Med

Polymyositis and dermatomyositis

N Engl J Med

Polymyositis and dermatomyositis

N Engl J Med

Evaluation of the severity of systemic sclerosis by nailfold capillary microscopy in 112 patients

Angiology

A novel autoantibody causing a peripheral fluorescent antinuclear antibody pattern is specific for nuclear pore complexes

Arthritis Rheum

Detection of a myosin heavy chain-like polypeptide at the nuclear pore complex

Nuclear pore complex structure analyzed by immunogold EM with human autoantibodies