Idiopathic Inflammatory Myopathies: Skins Symptoms Overview
Idiopathic inflammatory myopathies (IIM) encompasses a group of muscle disorders with various skin symptoms like Gottron's sign, heliotrope rash, calcinosis cutis, and more. This condition includes polymyositis, dermatomyositis, and other subtypes. The presence of certain skin manifestations may provide valuable diagnostic clues for IIM.
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Presentation Transcript
Melinda Nagy-Vincze 1, Zoltn Griger1, Levente Bodoki1, Zsuzsa Szankai1, Zoe E. Betteridge2, Katalin Dank 1 1 University of Debrecen, Division of Clinical Immunology, Dept. of Internal Medicine, Debrecen, Hungary 2 University of Bath, Institute for Rheumatic Diseases, Bath, UK
IDIOPATHIC INFLAMMATORY MYOPATHIES Polymyositis (PM) Dermatomyositis (DM) Juvene PM/DM Inclusion body myositis (IBM) Overlap myositis (OM) Necrotizing autoimmun myopathy (NAM): Cancer associated myositis (CAM) Statin induced myopathy Infection induced myopathy
IDIOPATHICINFLAMMATORYMYOPATHIES SKINSYMPTOMS Gottron s sign and papule
IDIOPATHICINFLAMMATORYMYOPATHIES SKINSYMPTOMS Heliotrop rash Linear extensor erythema Periungual teleangiectasia
IDIOPATHICINFLAMMATORYMYOPATHIES SKINSYMPTOMS V-sign Facial erythema Shawl sign Periorbital oedema
IDIOPATHICINFLAMMATORYMYOPATHIES SKINSYMPTOMS Calcinosis cutis Poikiloderma athrophicans vasculare Alopecia Livedo reticularis
CAM (CANCER ASSOCIATED MYOSITIS) Frequency 7-66% Relative risk for malignancy 3x in DM 1,3x in PM-ben Tumor types: ovarium, breast, lung, colon, endometrium, nasopharyngeal, lymphoma, prostata) In time: Before myositis symptoms (> 1 years) Real paraneoplasia (- 1 +5 years) After myositis diagnosis (> 5 years) role of immunosuppressive therapy? R. Aggarwal, C.V. Oddis Paraneoplastic myalgias and myositis Rheum Dis Clin N Am 2011
CAM - ETIOLOGY Paraneoplasia Cytotoxic/immunesuppressive treatment (Methotrexat, cyclophosphamid) Common trigger (EBV?)
CROSSOVERIMMUNITYINCAM Cellular Immune response CD8+ T Ly CD4+ T Ly MSA B Ly MSA ? MSA Cross reactions MSA Stuart M. Levine Curr Opin Rheumatol 2006, 18:620-624
Anti-TIF1 o antigen: transcription intermedier factor 1 gamma 155/140kDa protein o 13 21% in adult and 23 29% in juvenile DM cases o severe skin symptoms, o high tumor risk in adults
OURSTUDY Autoantibody analysis from IIM patients serum (n=202) with ELISA and/or IPP Frequency of anti-TIF1 positivity Frequency of TIF1 negative CAM Clinical and lab findings associated with anti- TIF1 positivity
PARAMETERS Clinical symptoms Proximal muscle weakness Distal muscle weakness Skin rash Dysphagia Raynaud phenomen Arthralgia ILD Fever o Lab results: CK and LDH levels CRP ESR ANF positivity Tumor markers
TIF1POSITIVEPATIENTS (N=12) CAM n=3 Real paraneoplasia in DM (n=1) After myositis diagnosis in DM (n=1) and in PM (n=1) Subsets: DM n=7 JDM n=4 PM n=1 Gender: Female 75% (n= 9) Male 25 % (n=3)
TIF1POSITIVECAMPATIENTREAL PARANEOPLASIA 34 years old, women First symptoms in April 2007: Skin rash Muscle weakness Dysphagia Arthralgia In July 2007 ovarium tumor Histology: adenocarcinoma with peritoneal metastasis Operation and chemotherapy She died in November 2007 due to heart failure
TIF1NEGATIVECAMPATIENTS (N=51) Subsets: DM(n=33) PM (n=18) Gender: Female 68% (n= 35) Male 32 % (n=16) In time: real paraneoplasia (n=37) - 5 months before myositis (n=2) - 73,5 months After diagnosis (n=12) 181 months
TIF1NEGATIVECAM (N=51) breast lung gynecology colon brain skin epipharinx gastric hemat urinary salivary glands pancreas penis prostata 1 1 1 2 1 15 3 1 2 2 6 1 9 6
HISTOLOGY adenocc. duct. planocellulare lobular invasive microcellulare melanoma kaposi sc. B-cell lymphoma T-cell lymphoma ependymoma mucoepidermal cc. unknown 3 1 1 2 1 1 1 1 21 1 4 14
DIFFERENCESINCLINICALSYMPTOMS (%) 100 100 90 83 83 75 75 80 67 70 60 55 55 50 45 50 42 37 37 40 33 33 TIF1y negative 25 25 25 30 24 TIF1y positive 20 17 16 20 14 12 8 6 10 4 0 0
LABFINDINGS (%) 100 90 86 90 75 75 75 80 71 71 65 70 55 60 50 50 TIF1y negative 50 42 TIF1y positive 40 30 20 10 0 CK LHD GOT GPT CRP We o No differences in tumor markers o No differences in other antibodies (ANF, APA)
CONCLUSION TIF1 positivity is associated with several and severe skin rashes Tumor specificity did not confirmed Autoantibody tests help us in the diagnosis But tumor searching is necessary, specially in DM
THANKSTOMYCOLLEGUES Prof. Dr. Dank Katalin, Dr. Griger Zolt n, Dr. Bodoki Levente, Szankai Zsuzsa, Zoe E. Betteridge This research was organized within the following program: T MOP 4.2.4.A/2-11-1-2012-0001 National Excellence Program local convergence program providing personnel support in the development and operation for students and researchers. The project was funded by the EU and the European Social Fund. The autoantibody analysis was sponsored by the ESF EuMyoNet Research Networking Programme.
