The changing incidence of rheumatoid arthritis over time in north-west Greece: data from a referral centre.

OBJECTIVE: Rheumatoid arthritis (RA) is a chronic inflammatory disease of unknown aetiology that affects approximately 1% of the population. The disease presents a temporal variability in different geographic areas. We investigated RA incidence over a 40-year-period in a defined area of north-west Greece, with a total population of about 400 000 inhabitants. METHOD: This incidence study was based on retrospective review of clinical records among adults with RA newly diagnosed from 1980 to 2019 at the referral university hospital of Ioannina. An incident case was defined as any patient diagnosed with RA based on the 1987 American College of Rheumatology criteria, over 16-years-old, and resident in the study area for at least 1 year before diagnosis. RESULTS: Out of 1411 cases diagnosed, women constituted a 2.65-fold higher number than men, with a lower mean age at diagnosis. The overall age-adjusted annual incidence rate (95% confidence interval) was 9.5 (8.5-10.5) for the total observation period, 11.7 (10.7-13.0) in 1980-1989, 10.4 (9.4-10.8) in 1990-1999, 9.8 (8.9-10.8) in 2000-2009, and 6.1 (5.3-6.9) in 2010-2019, presenting a statistically significant decline over time, along with a constant decrease in rheumatoid factor (RF)-positive incidence for both sexes. CONCLUSION: Our findings suggest a decrease in the incidence of RA over 40 years in a geographically defined Greek population. Also, the progressive decrease in the incidence of RF-positive disease may relate to less severe expression of RA in Greek patients. These trends could be explained by different clinical, serological, and genetic factors reported in Greece compared to northern European countries.

Fibroblastic rheumatism: an uncommon arthritis. A case-based review.

Fibroblastic rheumatism (FR) is an uncommon disease of the skin, characterized by the presence of non-tender cutaneous nodules accompanied often by other rheumatic manifestations. This condition shows male predominance, no age preference and unpredictable course, resulting frequently in permanent joint damage. A 60-year-old man came to our department with a 4-year history of multiple non-tender nodules and morning stiffness affecting mainly the upper extremities. Clinical examination revealed arthritis of the hands, confirmed by imaging tests. Laboratory exams were unremarkable. A skin nodule biopsy showed a dermal collagenous lesion with myxoid areas composed of spindle and stellate cells. Immunohistochemical staining demonstrated positivity for CD68 and negativity for CD34, S100, EMA and desmine. FR was diagnosed and the patient started methylprednisolone 16 mg/day. Hydroxychloroquine 400 mg/day and methotrexate 15 mg/weekly were further added as steroid-sparing agents with clinical benefit. Clinicians should be aware of this underreported entity, which can rapidly lead to irreversible deformities.

Cryoglobulinemic vasculitis in primary Sjögren's Syndrome: Clinical presentation, association with lymphoma and comparison with Hepatitis C-related disease.

OBJECTIVE: To describe the clinical spectrum of cryoglobulinemic vasculitis (CV) in primary Sjögren's syndrome (pSS), investigate its relation to lymphoma and identify the differences with hepatitis C virus (HCV) related CV. METHODS: From a multicentre study population of consecutive pSS patients, those who had been evaluated for cryoglobulins and fulfilled the 2011 classification criteria for CV were identified retrospectively. pSS-CV patients were matched with pSS patients without cryoglobulins (1:2) and HCV-CV patients (1:1). Clinical, laboratory and outcome features were analyzed. A data driven logistic regression model was applied for pSS-CV patients and their pSS cryoglobulin negative controls to identify independent features associated with lymphoma. RESULTS: 1083 pSS patients were tested for cryoglobulins. 115 (10.6%) had cryoglobulinemia and 71 (6.5%) fulfilled the classification criteria for CV. pSS-CV patients had higher frequency of extraglandular manifestations and lymphoma (OR=9.87, 95% CI: 4.7-20.9) compared to pSS patients without cryoglobulins. Purpura was the commonest vasculitic manifestation (90%), presenting at disease onset in 39% of patients. One third of pSS-CV patients developed B-cell lymphoma within the first 5 years of CV course, with cryoglobulinemia being the strongest independent lymphoma associated feature. Compared to HCV-CV patients, pSS-CV individuals displayed more frequently lymphadenopathy, type II IgMk cryoglobulins and lymphoma (OR = 6.12, 95% CI: 2.7-14.4) and less frequently C4 hypocomplementemia and peripheral neuropathy. CONCLUSION: pSS-CV has a severe clinical course, overshadowing the typical clinical manifestations of pSS and higher risk for early lymphoma development compared to HCV related CV. Though infrequent, pSS-CV constitutes a distinct severe clinical phenotype of pSS.

Efficacy and safety of switching from infliximab to adalimumab: a comparative controlled study.

OBJECTIVE: To describe the efficacy and safety of adalimumab in patients with rheumatoid arthritis (RA) who had previously discontinued infliximab treatment. METHODS: 24 patients with RA who discontinued treatment with infliximab (switchers) were treated with adalimumab (40 mg every 2 weeks, subcutaneously) for 12 months. The results were compared with those for 25 patients with RA receiving adalimumab who had not previously used an anti-tumour necrosis factor alpha inhibitor (controls). Disease activity was measured with the 28 joint count Disease Activity Score (DAS28), and clinical response with the American College of Rheumatology (ACR) 20% response criteria. RESULTS: At baseline there were no differences in demographic, clinical, and laboratory features between the two groups. After 12 months' adalimumab treatment, clinical improvement was similar in both groups. More specifically, ACR 20% response criteria were achieved by 18/24 (75%) switchers and by 19/25 (76%) subjects in the control group. Four switchers discontinued the study-two because of adverse events and two because of lack of efficacy, while three control patients discontinued the study-one because of lack of efficacy and two owing to side effects. CONCLUSION: Adalimumab is a well tolerated and effective treatment for patients with RA, even when infliximab has been discontinued.

Epidemiology of primary Sjögren's syndrome in north-west Greece, 1982-2003.

OBJECTIVES: To investigate the incidence and prevalence, as well as the mortality and survival rates, of primary Sjögren's syndrome (pSS) in a defined area of north-west Greece with a population of about 500 000 inhabitants. METHODS: Cases were recorded from the following sources: (i) in- and out-patients referred to the rheumatology clinics of the Ioannina University Hospital and the Ioannina General Hospital; and (ii) patients referred to private rheumatologists practising in the study area. All patients diagnosed between 1 January 1982 and 31 December 2003 who were resident in the study area were included as incident cases. Diagnosis was based on the American-European consensus criteria for SS. Incidence and prevalence rates were calculated as numbers of cases per 10(5) inhabitants. Population data were based on the National Censuses of 1981, 1991 and 2001. RESULTS: A total of 422 incident cases were identified for the study period 1982-2003. Age-adjusted mean annual incidence rate for this period was 5.3 (95% confidence interval [CI] 4.5-6.1) cases per 10(5) adult inhabitants. The female/male ratio of incident cases was about 20/1. The age-adjusted prevalence rate for the adult population was 92.8 (95% CI 83.7-101.9) cases per 10(5) inhabitants on 31 December 2003. The 5-yr survival rate in the incidence cohort was 96.6% and the 10-yr survival rate 92.8%. The standardized mortality ratio in comparison with the general population of the study area was 1.02 (95% CI 0.4-2.0). The main causes of death were cardiovascular diseases and cancer. The occurrence of the disease shows a slightly decreasing, but not statistically significant, trend with time. CONCLUSIONS: The estimated incidence and prevalence of pSS in this study were slightly higher in comparison with data from other studies based on physician-diagnosed cases. The prevalence was significantly lower when compared with the findings of studies based on the examination of a sample of the general population. Mortality rates did not differ significantly between pSS patients and the general population.