AB0706 Demographic, clinical and serological characteristics in Idiopathic Inflammatory Myopathies. The role of specific antibodies

Autor: X. E. Larco Rojas, S. Sáez-Álvarez, P. Pérez-García, C. Sieiro Santos, I. González Fernández, C. Moriano, A. López Robles, M. Martín Martín, C. Álvarez Castro, E. Diez Alvarez
Rok vydání: 2022
Předmět:
Zdroj: Annals of the Rheumatic Diseases. 81:1479.3-1480
ISSN: 1468-2060
0003-4967
Popis: BackgroundIdiopathic Inflammatory myopathies are a heterogenous group of diseases which main common characteristic is muscle injury. Specific autoantibodies related with different phenotypes have become an important diagnostic and prognostic tool. These antibodies may be associated with different histopathological features in muscle biopsies.ObjectivesTo describe the demographic, clinical and serological characteristics of Idiopathic Inflammatory Myopathies (IIM); the most frequent corticoid sparing agents used and the frequency of associated neoplasms in a sample of patients with a compatible biopsy result. To compare these characteristics in patients with positive and negative ANA and the association of different biopsy patterns with specific myositis antibodies.MethodsMuscle biopsies results that were compatible with inflammatory myopathy were collected from the Pathology Unit database of Leon´s hospital between January 2010 and March 2021.Demographic, clinical and serological characteristics, associated malignancies and treatments used were collected from clinical records.ResultsWe included 30 patients in the study. Patient`s characteristics are shown in Table 1. Median age of diagnosis was 59 years. Arthritis and lung disease were found in 20% of patients. The patterns of lung involvement found in HRCT were NSIP in 2, UIP in 1, and bronchiectasis in 3 patients. Associated collagenopathies were found in 26,7%, being vasculitis the most frequent (2 patients). Associated Malignancies were found in 13,3%: lung carcinoma in 1, Urothelial carcinoma in 1, cervix carcinoma in 1 and prostate carcinoma 1 patient. Specific myositis antibodies were negative in 56,7%; positive ANA was found in 60%.Table 1.Demographic, clinical, serological characteristics and treatment in the sample studiedCharacteristicsPatients (n=30)Median; IQR or n(%)Age at diagnosis (years)59; 18,5Sex F/M17(56,7)/13(43,3)Artralgias11(36,7)Arthritis6(20)Raynaud3(10)Lung disease6 (20)Skin disease7 (23,3)Autoimmunity biomarkers (+/-/ not done)ACPA2(6,7) /3 (10) /25(83,3)Positive Rheumatoid Factor4(13,3) /13 (43,3) /13(43,3)ANA18 (60%) /12(40%)Specific Myositis AntibodiesAnti SRP1(3,3)Anti-HMG CoA reductase5 (16,7)Anti Jo12(6,7)Negative17 (56,7)Not done5 (16,4)Biopsy patternnecrotizing8 (26,7)inflammatory15(50)Inclusion Bodies4 (13,3)dermatomyositis3 (10)DiagnosisDermatomyositis3 (10)Statin Myopathy5(16,7)Inclusion body myopathy3(10)Polymyositis8(26,7)Immune-mediated necrotizing myopathy2 (6,7)Antisynthetase syndrome1(3,3)Other collagenopathies8(26,7)TreatmentMethotrexate11(36,7)Azathioprine3(10)immunoglobulins5(16,7)Rituximab5(16,7)Mycophenolate4(13,3)Cyclophosphamide2 (6,7)None11 (36,7)2 or more treatments8(26,7)Associated malignancies4 (13,3)The median of levels at diagnosis of CK was: 1181; IQR:6252,5; aldolase 14,4; IQR: 34,65; AST 62; IQR:181,25; ALT 55,5; IQR:119,25; CRP 7,6; IQR 13,55.When compared, CK and ALT levels in patients with negative and positive ANA, this were higher in negative patients (6365; IQR:9592vs888;3045; P=0.044) and (182; IQR:287vs43; 85; P=0,035) respectively. Anti HMGCoA reductase antibodies were more frequently found in patients with necrotizing pattern in biopsies 5/8 patients; (62,5%); p=0.002.Methotrexate was the corticoid sparing agent most frequently used (36,7% of patients), 36,7% of patients were treated only with corticoids and 26,7% needed more than one treatment.ConclusionSpecific myositis antibodies are helpful tools in the diagnosis when present, meanwhile biopsy can be an important tool when antibodies are negative. ANA positivity seems to be associated with milder disease at the muscular domain.References[1]Zanframundo G,Tripoli A, Cometi L, et al. One year in review 2020: idiopathic inflammatory myopathies. Clin Exp Rheumatol 2021; 39: 1-12.Disclosure of InterestsNone declared
Databáze: OpenAIRE