Clinical, serologic, and immunogenetic features in polish patients with idiopathic inflammatory myopathies

Interstitial lung disease in polymyositis and dermatomyositis

“…The first group, the most common, comprises autoantibodies against one of the five aminoacyl-transfer-RNA synthetases which catalyse the binding of each amino acid to its tRNA (Love LA 1991). The presence of these antibodies is strongly associated with an acute disease onset and the so-called anti-synthetase syndrome comprising in myositis of, arthritis, mechanic hands, Raynaud's phenomenon and interstitial lung disease (Love LA 1991, Hausmanowa-Petrusewicz I 1997. Patients with antiaminoacy-tRNA syntheatasis have a moderate response to therapy.…”

Long-term outcome of patients with polymyositis/ dermatomyositis and anti-PM-Scl antibody

“…Further investigators have also reported that anti‐PM‐Scl antibodies are more often found in patients with overlap syndrome of systemic sclerosis and PM or DM (scleromyositis and sclerodermatomyositis, respectively), being encountered in 24% of these patients 9,12–29 . In contrast, anti‐PM‐Scl antibodies are more infrequently found in patients with isolated PM/DM (5–8% of cases) 11,13,16,17,22,23,28 . Indeed, in the series of Raijmakers et al.…”

“… 8 Anti‐PM‐Scl antibodies are directed against a nucleolar macromolecular complex of peptides of 75 kDa (PM‐Scl 75 protein) and 100 kDa (PM‐Scl 100 protein), with PM‐Scl 75 being considered the main autoantigen; 9 this autoantigenic complex is the human homologue of the yeast exosome, which is an RNA‐processing complex 10,11 . Anti‐PM‐Scl antibodies have been found in 24% of patients with PM/scleroderma overlap syndrome, compared with only 2–3% of patients with scleroderma alone and 5–8% of patients with PM/DM alone 9,11–29 . Further investigators have also reported that anti‐PM‐Scl antibodies are more often found in patients with overlap syndrome of systemic sclerosis and PM or DM (scleromyositis and sclerodermatomyositis, respectively), being encountered in 24% of these patients 9,12–29 .…”

“…Anti‐PM‐Scl antibodies have been found in 24% of patients with PM/scleroderma overlap syndrome, compared with only 2–3% of patients with scleroderma alone and 5–8% of patients with PM/DM alone 9,11–29 . Further investigators have also reported that anti‐PM‐Scl antibodies are more often found in patients with overlap syndrome of systemic sclerosis and PM or DM (scleromyositis and sclerodermatomyositis, respectively), being encountered in 24% of these patients 9,12–29 . In contrast, anti‐PM‐Scl antibodies are more infrequently found in patients with isolated PM/DM (5–8% of cases) 11,13,16,17,22,23,28 .…”

Dermatomyositis: cutaneous manifestations of its variants

“…All patients with PM-Scl antibodies have HLA-DQA1*0501 and 94% HLA-DRB1*0301 suballeles. 48 Patients with MCTD and dermatomyositis rarely develop internal neoplasms. Patients with overlap syndrome tend to respond better to systemic corticosteroids than do patients with other types of primary inflammatory myositis.…”