[PM-Scl antibody positive systemic sclerosis associated with inclusion-body myositis].
We describe a 72- year-old patient with a ten year history of anti-PM-Scl positive systemic sclerosis associated with inclusion-body myositis. While the association of dermatomyositis and polymyositis with anti-PM-Scl positive systemic sclerosis is frequently reported, inclusion-body myositis was, to the best of our knowledge, only previously described once in association with anti-PM-Scl-positive systemic sclerosis. The distinction between inclusion-body myositis and other forms of inflammatory myopathy, like the histopathologically well distinguishable polymyositis or dermatomyositis, is relevant because of the poor response of inclusion- body myositis to immunosuppressive treatment. Our case underlines that in patients with anti-PM-Scl-positive systemic sclerosis and treatment resistant progressive myopathy the diagnosis of inclusion body myositis should be considered.
Kim
S
S
Klinik und Poliklinik für Dermatologie und Venerologie der Universität Köln, Joseph-Stelzmann-Strasse 9, 50937 Köln, Germany.
Genth
E
E
Krieg
T
T
Hunzelmann
N
N
D000368:Aged; D001324:Autoantigens / Q000097:blood* / Q000276:immunology; D015415:Biomarkers / Q000097:blood; D003937:Diagnosis, Differential; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; D005260:Female; D006801:Humans; D018979:Myositis, Inclusion Body / Q000097:blood* / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D012595:Scleroderma, Systemic / Q000097:blood* / Q000150:complications / Q000175:diagnosis* / Q000276:immunology
D001324:Autoantigens; D015415:Biomarkers; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; C077044:EXOSC10 protein, human