30 research outputs found
Coexistence of Becker muscular dystrophy and HLA-B27 positive spondyloarthritis
No full text[No abstract available
POS0016 COMMON VARIABLE IMMUNODEFICIENCY DISEASE FROM THE PERSPECTIVE OF RHEUMATOLOGY
No full textBackgroundCommon variable immunodeficiency (CVID) is a primary immunodeficiency characterized by impaired B cell differentiation and immunoglobulin production. In addition to increased susceptibility to infection, patients also have an increased tendency to autoimmune disease. Since rheumatological findings usually start earlier than other autoimmunities, it is very important to increase the awareness of rheumatologists about this disease, for accurate diagnosis and to prevent delay in treatment.ObjectivesTo increase the awareness of rheumatologists about the main symptoms and findings of CVID.MethodsAdult patients referred to the rheumatology department since January 2015 were included in the study. Demographic and clinical characteristics (infections, pulmonary and extrapulmonary granulomatous involvement, autoimmune manifestations), laboratory and imaging findings and treatments of the patients were analyzed.ResultsTen adult patients with CVID were included in the study. The gender distribution of the patients was similar and the median age was 38±10.0 years. The mean duration of diagnosis was 123.5±89.3 months. At least one autoimmune manifestation was observed in 80% of the patients. In the follow-up period, 40% of the patients developed arthritis. Involvement of lower extremity joints such as knee and ankle was more prominent. While all patients were given 0.8 g/kg/3 weeks of intravenous immunoglobulin, 80% required immunosuppressive therapy for autoimmune manifestations. The demographic and clinical characteristics of the patients are summarized in Table 1.Table 1.Autoimmune manifestations of common variable immunodeficiency patientsCase 1Case 2Case 3Case 4Case 5Case 6Case 7Case 8Case 9Case 10Age/Sex36/F43/F40/M25/M23/M36/M41/M57/F35/F48/FSymptom duration (years)121111417121113127Diagnosis duration (years)61183171091112Sinopulmonary infection++++++++++GI infection-+-+-++---Other infections++-++-++-+Pulmonary Involvementlymphocytic ILDnodule*bronchiectasis--bronchiectasisbronchiectasiscavitationnodulenodule-Extrapulmonary Granulomabrain parenchyma, bone marrow, liver, spleen, skinliver, spleen-renal°, liver-**bone-ᵜlymph node--Autoimmune manifestations-monoarthritis,chronic ileitisoligoarthritis,atrophic gastritis,chronic ileitisalopecia, thyroiditis,bilateral anterior uveitis-oligoarthritis,autoimmune neutropenia,chronic ileitis,kounis syndromeoligoarthritis,autoimmune neutropeniaoligoarthritis, thyroiditis,sicca syndrome,recurrent scleritiscolitis-Cirrhosis of the liver++---+----Lymphadenopathy++++-+++++Splenomegaly++++-++-+-Immunosuppressive TreatmentSteroid, AZA, CSA, AdalimumabSteroidSteroid, SSZSteroid, MMF, CYC-Steroid, SSZ, MTX, LEF, CSASteroidSteroid, MTX, AZA, CSASteroid, AZA-Abbreviations: AZA-azathioprine, CSA-cyclosporine, CYC-cyclophosphamide, F-female, GI-gastrointestinal, ILD-interstitial lung disease, LEF-leflunomide, M-male, MTX-methotrexate, SSZ-sulfasalazine*right lower lobectomy, ** right foot 5th toe enucleation, ᵜ mediastinoscopic lymph node biopsy ° interstitial granulomatous nephritisConclusionAutoimmune diseases can be seen in patients with CVID, and sometimes this may be the first presentation of CVID. Heterogeneous clinical findings of the disease may lead to delay in diagnosis. Clinicians should be more careful about the different manifestations of CVID to avoid delay in diagnosis.Disclosure of InterestsNone declared</jats:sec
Rheumatic diseases detected in patients presenting with uveitis
No full textOBJECTIVE: Uveitis may occur in the course of systemic inflammatory rheumatic diseases (IRD), or it may be the first clinical manifestation of these diseases. The aim of this study was to determine the incidence of IRD in patients whose initial clinical manifestation was non-infectious uveitis. METHODS: The study included adult patients diagnosed as having noninfectious uveitis in the department of ophthalmology and referred to rheumatology for further investigation of potential rheumatic diseases as underlying etiology of uveitis. The patients’ demographic and clinical features, laboratory, and imaging findings were examined. RESULTS: One hundred six patients who were diagnosed as having uveitis (42.4% anterior, 2.8% intermediate, 19.8% posterior, and 34.9% panuveitis) were included. Just over half (52.8%) of the patients were male and the mean age was 40.1±14.8 years. The mean age at the uveitis attack was 38.7±15 years. One-third (33%) of the patients were diagnosed as having rheumatologic disease (spondyloarthritis (SpA) n=10, Behcet disease (BD) n=17, vasculitides n=2, sarcoidosis n=2, undifferentiated connective tissue diseases n=3, rheumatoid arthritis n=1). SpA was diagnosed in 20% of patients presenting with anterior uveitis. BD was detected in 27% of patients referred with panuveitis and in 33.3% of patients whose first clinical finding was posterior segment involvement. Bilateral uveitis was detected in two-thirds of patients with posterior uveitis and tended to recur more frequently (p=0.014). CONCLUSION: Rheumatic diseases have been identified in approximately one-third of patients presenting with different types of uveitis. Investigations addressing systemic rheumatic diseases are of paramount importance in patients with uveitis because they may change diagnosis and treatment processes
The effect of COVID-19 pandemic in a large series of patients with Takayasu arteritis
No full textBackground/aim: Patients with inflammatory rheumatic diseases faced several challenges during the COVID-19 pandemic. Uncertainties such as the lack of evidence regarding the use of immunosuppressive (IS) therapies and deferred patient care because of limited health resources affected negatively on many aspects of treatment decisions and routine follow-up of the patients. In this study, we aimed to investigate the prevalence and severity of SARS-CoV-2 infection, the impact of the pandemic on delays in routine clinical follow-up, changes in IS treatment, and COVID-19 vaccination status of patients with Takayasu arteritis (TAK). Materials and methods: The study was performed between July and September 2021. TAK patients who registered in our database were investigated with regards to the COVID-19 infection and vaccination status, delays in routine clinical visits, changes in their IS treatments, and flares during the pandemic. Physical examination, laboratory tests, and imaging of the patients were performed and ITAS2010 scores were calculated. Results: There were 56 adult TAK patients (87.5% female and median age 47 years). A total of 44 (78.6%) patients experienced a delay with routine follow-up visits to their physicians and about 20% of patients stopped their antirheumatic treatments without consulting their physicians. Compared to the pre-COVID-19 pandemic, 16 (28.5%) patients flared. In total group, 13 (23.2%) patients had a mild COVID-19 infection and about 90% of the patients had received the COVID-19 vaccine. Conclusion: Deferred patient care and disease flares are the most significant problems in TAK patients during the pandemic. The risk of TAK flares may outweigh the risk of COVID-19 infection
Hematologic Involvement in Systemic Lupus Erythematosus: Clinical Features and Prognostic Implications in a Hematology-Referred Cohort
No full textBackground/Objectives: Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease frequently complicated by hematologic abnormalities, which may reflect disease activity or treatment effects. To characterize the clinical, laboratory, and immunological features of adult SLE patients referred to hematology during routine rheumatology follow-up. Methods: We retrospectively analyzed 84 adult SLE patients who fulfilled the 2012 SLICC or 2019 EULAR/ACR criteria and were referred to hematology during follow-up. Clinical, laboratory, and immunological data were collected. Associations between hematologic manifestations, organ involvement, autoantibodies, and complement levels were evaluated. Results: The cohort included 92.6% females with a median age of 46 (IQR 36–62). Hematologic abnormalities commonly appeared within three years of disease onset. Lymphadenopathy was more frequent in patients with cutaneous vasculitis and lupus nephritis (p = 0.046 and p = 0.045). Splenomegaly was associated with serositis, anti-β2 glycoprotein I IgG, and lupus anticoagulant (LA) positivity; anti-β2GPI IgG independently predicted splenomegaly (OR 26.02, p = 0.006). Low C4 was associated with increased autoimmune hemolytic anemia risk (OR 5.88, p = 0.009), while low C3 was linked to lupus nephritis (p = 0.017). Antiphospholipid antibodies were significantly associated with venous thrombosis, with anti-cardiolipin IgG as an independent predictor (OR 7.43, p = 0.007). Stroke history, anti-histone antibodies, and higher steroid doses were associated with mortality. Remission was linked to fewer comorbidities (p = 0.008). Conclusions: Hematologic complications in SLE arise early and carry prognostic significance, with splenomegaly associated with lupus anticoagulant and anti-β2GPI IgG, and mortality linked to anti-histone antibodies
