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Inflammatory myopathies with anti-Ku antibodies: a prognosis dependent on associated lung disease.

Abstract

Anti-Ku antibodies have been reported in a wide spectrum of autoimmune diseases, sometimes in association with inflammatory myopathies (IM). We studied the clinical, laboratory, and muscle histologic features of all anti-Ku-positive patients detected in our hospital during the last 10 years, as well as their treatment and outcomes. Anti-Ku antibodies were found in 34 patients (0.46% of 20,600 sera positive for antinuclear antibodies), and complete data were available for 30 patients; 86.7% were female, mean age was 49 years (range, 20-73 yr). The most frequent clinical manifestations were arthralgia (77%) and Raynaud phenomenon (53%). Eleven (37%) patients had IM, 8 of them as part of an overlap syndrome defined as IM associated with connective autoimmune disease (5 systemic sclerosis [SSc], 2 Sjögren syndrome (SS), and 1 systemic lupus erythematosus [SLE]). Of 21 patients without IM, 19 had autoimmune diseases (including 6 SLE, 2 SSc, 2 SS, and 2 rheumatoid arthritis), 1 had bronchial neoplasia, and 1 had nephroangiosclerosis. Clinical features of the 9 patients with IM were myalgia (91%), proximal muscle weakness (89%), and dysphagia (36%). All had increased creatine kinase (median, 2210 U/L; range, 194-4073 U/L). Muscle biopsy showed necrosis, inflammation, and positive HLA class I immunostaining. Interstitial lung disease (ILD) was detected on computed tomography (CT) scan in 11 patients (37%) and was significantly more frequent in patients with IM (82% vs. 10.5%, p < 0.001). Fourteen (47%) patients required no immunosuppressive treatment or only a low corticosteroid dose (<15 mg/d, n = 3). A high dose of corticosteroids was more frequently administered in patients with IM (10/11 cases, 80% with associated ILD) than in patients without IM (4/19 cases, 0 with ILD). Complete muscle remission after steroids occurred in 73% of patients with IM. Lung disease was corticoresistant in 6 of 8 (75%) treated cases.Anti-Ku antibodies remain rarely detected, but their presence can be frequently associated with corticosensitive IM and severe, corticoresistant ILD.

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  • Authors

    Rigolet A, Musset L, Dubourg O, Maisonobe T, Grenier P, Charuel JL, Behin A, Herson S, Amoura Z, Benveniste O

    Institution

    Service de Médecine Interne 1, Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, Paris, France. aude.rigolet@psl.aphp.fr

    Source

    Medicine 91:2 2012 Mar pg 95-102

    MeSH

    Adrenal Cortex Hormones
    Adult
    Aged
    Antibodies, Antinuclear
    Antigens, Nuclear
    Arthralgia
    Autoantibodies
    Autoimmune Diseases
    Biopsy
    Creatine Kinase
    DNA-Binding Proteins
    Deglutition Disorders
    Drug Resistance
    Female
    Humans
    Hypertension, Pulmonary
    Immunosuppressive Agents
    Lung Diseases, Interstitial
    Male
    Middle Aged
    Muscle Weakness
    Muscle, Skeletal
    Myositis
    Necrosis
    Prognosis
    Raynaud Disease
    Retrospective Studies
    Young Adult

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    22391471