Deciphering the Clinical Presentations, Pathogenesis, and Treatment of the Idiopathic Inflammatory Myopathies

Rider, Lisa G.; Miller, Frederick W.

doi:10.1001/jama.2010.1977

Cited by 126 publications

(99 citation statements)

References 46 publications

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“…Muscle atrophy is detected early in inclusion-body myositis, with selective atrophy of the quadriceps and forearm muscles, but it develops in all subtypes if the weakness is severe and chronic. Myalgia and muscle tenderness may occur, especially in patients with the antisynthetase syndrome (see the Glossary), 6,7 but if pain is severe and the weakness follows a "breakaway" pattern, in which the patient has difficulty sustaining effort, fasciitis or fibromyalgia should be ruled out.…”

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

“…Extramuscular manifestations may occur in all inflammatory myopathies, although they occur in inclusion-body myositis only in rare cases; these manifestations include systemic symptoms, such as fever, arthralgia, and Raynaud's phenomenon, as seen in the antisynthetase syndrome 4,6,7 ; cardiac arrhythmias or ventricular dysfunction, in relatively uncommon cases in which the affected cardiac muscle is clinically symptomatic; and pulmonary complications, due primarily to interstitial lung disease, which are reported in 10 to 40% of patients. 8 The prevalence of interstitial lung disease, a condition that is best detected with highresolution computed tomography, is as high as 70% among patients with anti-histidyl-transfer RNA (tRNA) synthetase (anti-Jo-1) or anti-melanoma differentiation-…”

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

“…Dermatomyositis is seen in both children and adults, and the early symptoms include distinct skin manifestations accompanying or preceding muscle weakness; the skin manifestations include periorbital heliotrope (blue-purple) rash with edema; erythematous rash on the face, knees, elbows, malleoli, neck, anterior chest (in a V-sign), and back and shoulders (in a shawl sign); and a violaceous eruption (Gottron's rash) on the knuckles, which may evolve into a scaling discoloration. [1][2][3][4][5][6][7]9 The lesions are photosensitive and may be aggravated by ultraviolet radiation. 6,7,9 Dilated capillary loops at the base of the fingernails, irregular and thickened cuticles, and cracked palmar fingertips ("mechanic's hands") are characteristic of dermatomyositis.…”

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

“…[1][2][3][4][5][6][7]9 The lesions are photosensitive and may be aggravated by ultraviolet radiation. 6,7,9 Dilated capillary loops at the base of the fingernails, irregular and thickened cuticles, and cracked palmar fingertips ("mechanic's hands") are characteristic of dermatomyositis. [1][2][3] Subcutaneous calcifications, sometimes extruding to the surface of the skin and causing ulcerations and infections, may occur and are especially common among children.…”

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

“…[1][2][3] In children, an early symptom is "misery," defined as irritability combined with a red flush on the face, fatigue, and a reluctance to socialize. 2,3 The symptoms of dermatomyositis may overlap with those of systemic sclerosis and mixed connective-tissue disease [1][2][3][4][5][6][7] ; in such cases, the typical skin rash is transient or faint. Overlap myositis is now starting to be recognized as a distinct entity; it manifests without the rash that is typical of dermatomyositis, with prominent pathologic changes in the perifascicular, interfascicular, and perimysial regions, and is frequently associated with antisynthetase antibodies.…”

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

See 4 more Smart Citations

Inflammatory Muscle Diseases

Young¹,

Finn²,

Reisin³

2015

N Engl J Med

140

View full text Add to dashboard Cite

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

Section: Gener a L Clinic A L Fe At Ur Esmentioning

confidence: 99%

See 3 more Smart Citations

Inflammatory Muscle Diseases

Young¹,

Finn²,

Reisin³

2015

N Engl J Med

140

View full text Add to dashboard Cite

Cytosolic 5′‐Nucleotidase 1A As a Target of Circulating Autoantibodies in Autoimmune Diseases

Lloyd

Christopher‐Stine

Pinal‐Fernandez

et al. 2015

Arthritis Care & Research

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Objective Prior investigations demonstrated that autoantibodies recognizing cytosolic 5′-nucleotidase 1A (NT5C1A) are found in 33–76% of patients with inclusion body myositis (IBM) but are observed only rarely in patients with polymyositis (PM). Thus, anti-NT5C1A may help distinguish IBM from PM. Although 4–21% of patients with dermatomyositis (DM) were shown to be anti-NT5C1A antibody positive, the clinical features of anti-NT5C1A–positive patients with DM have not been described. Furthermore, the prevalence of anti-NT5C1A antibodies in other rheumatic conditions has not been reported. This study was undertaken to define the prevalence and clinical features of anti-NT5C1A–positive patients with DM, PM, IBM, or other systemic autoimmune diseases. Methods We screened for anti-NT5C1A autoantibodies in patients with IBM, DM, PM, Sjögren’s syndrome (SS), or systemic lupus erythematosus (SLE) and in healthy volunteers. Clinical characteristics were compared between patients who were anti-NT5C1A positive and those who were anti-NT5C1A negative. Results Anti-NT5C1A autoantibodies were detected in 71 (61%) of 117 patients with IBM, 2 (5%) of 42 patients with PM, 2 (5%) of 42 healthy volunteers, 24 (15%) of 159 patients with DM, 10 (23%) of 44 patients with SS, and 13 (14%) of 96 patients with SLE. No anti-NT5C1A antibody–positive patients with SS or SLE had muscle involvement. Anti-NT5C1A–positive patients with IBM had a lower prevalence of rimmed vacuoles (62% versus 83% of antibody-negative patients; P = 0.02). No differences in the clinical characteristics of antibody-positive and antibody-negative patients with DM, SS, or SLE were observed. Conclusion Anti-NT5C1A is a common target of circulating autoantibodies, especially in IBM but also in several different autoimmune diseases. In SLE and SS, anti-NT5C1A autoreactivity is not associated with muscle disease.

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Association of Anti–3‐Hydroxy‐3‐Methylglutaryl‐Coenzyme A Reductase Autoantibodies With DRB1*07:01 and Severe Myositis in Juvenile Myositis Patients

Kishi

Rider

Pak

et al. 2017

Arthritis Care & Research

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Objective Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of pediatric anti-HMGCR-positive myositis patients. Methods The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti-HMGCR-positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis-specific autoantibodies (MSAs). Results Five (1.1%) of 440 patients were anti-HMGCR-positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and two patients had immune-mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti-HMGCR-positive subjects was 17,000 IU/L. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR-positive subjects compared to MSA-negative patients or those with other MSAs. Anti-HMGCR-positive patients had only partial responses to multiple immunosuppressive medications and often a chronic course. The DRB1*07:01allele was present in all 5 patients compared to 26.25% of healthy controls (Pcorrected=0.01); none of the 5 pediatric patients had DRB1*11:01. Conclusions Compared to children with other MSAs, muscle disease appeared to be more severe in those with anti-HMGCR autoantibodies. Like adults, children with anti-HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, anti-HMGCR-positive children have a strong association with HLA DRB1*07:01.

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Deciphering the Clinical Presentations, Pathogenesis, and Treatment of the Idiopathic Inflammatory Myopathies

Cited by 126 publications

References 46 publications

Inflammatory Muscle Diseases

Inflammatory Muscle Diseases

Cytosolic 5′‐Nucleotidase 1A As a Target of Circulating Autoantibodies in Autoimmune Diseases

Association of Anti–3‐Hydroxy‐3‐Methylglutaryl‐Coenzyme A Reductase Autoantibodies With DRB1*07:01 and Severe Myositis in Juvenile Myositis Patients

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