Background:Palindromic rheumatism (PR) has known to be three patterns of disease course: clinical remission of attacks, persistent attacks, and evolution to chronic arthritis or systemic disease. The spectrum in progression to chronic diseases of PR, however, is quite variable; rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjögren’s syndrome (SjS), ankylosing spondylitis (AS), relapsing polychondritis (RP), Behçet’s disease (BD), sarcoidosis, and psoriatic spondylitis and arthropathy. Because of the small numbers in case-control studies and too aged investigations, now we needs to shed new light on the fate of PR.Objectives:The aim was to investigate the epidemiology of PR and the risk of developing various rheumatic diseases compared with non-PR individuals, employing the National Health Insurance Service (NHIS) medical claims data, which covers all medical institutions of South Korea.Methods:The study used 2007-2018 claims data from the Korean Health Insurance Review and Assessment Service (HIRA). The identified 19,724 PR patients from 2010 to 2016 were assessed for the incidence rate (IR) compared with the population in the given year by 100,000 person-year (py). The date of diagnosis was the index date. After matching with non-PR individuals (1:10) for age, sex and the year of index date, we calculated the hazard ratios (HRs) with 95% confidence intervals (CIs). The risk of developing the various rheumatic diseases and adult immunodeficiency syndrome (AIDS) as the outcome diseases in PR cohort was estimated. This risk was compared with that of matched non-PR cohort.Results:Of 19,724 PR patients (8,665 males and 11,059 females), the mean age was 50.2 ± 14.9 years (47.7 ± 14.4 years in males and 52.6 ± 14.9 years in females,p< 0.001). The ratio of male to female patients with PR was approximately 1:1.28. The annual IR of PR was 7.02 (6.92-7.12) per 100,000 py (6.22 (6.09-6.35) and 7.80 (7.66-7.95) per 100,000 py in males and females, respectively). The mean duration to develop the outcome diseases was significantly shorter in PR cohort compared that of non-PR cohort (19.4 vs. 35.8 months,p< 0.001). The most common outcome disease was RA (7.34% of PR patients; 80.0% of total outcome diseases), followed by AS, SLE, BD, SjS, MCTD, DM/PM, SSc, RP, psoriatic arthropathy, and AIDS in PR cohort. The patients with PR had an increased risk of RA (HR 46.6, 95% CI [41.1-52.7]), psoriatic arthropathy (44.79 [15.2-132.4]), SLE (24.5 [16.2-37.2]), MCTD (22.0 [7.7-63.3]), BD (21.0 [13.8-32.1]), SjS (12.4 [8.5-17.9]), AS (9.0 [6.7-12.2]), DM/PM (6.1 [2.6-14.8]), and SSc (3.8 [1.5-9.6]) but not of AIDS. The risk of developing RA was greater in male patients (HR 58.9, 95% CI [45.6-76.2] vs. 43.2 [37.4-49.8],pfor interaction = 0.037) while female patients encountered a higher risk of developing AS (15.8 [8.9-28.1] vs. 7.2 [5.0-10.3],pfor interaction = 0.023). The risk of developing RA, SLE, SjS, and BD were significantly more highly affected in younger age (pfor interaction < 0.001, = 0.003, 0.002, and 0.017, at each).Conclusion:This nationwide, population-based cohort study demonstrated that patients with PR had an increased risk of developing various rheumatic diseases, not only RA but also psoriatic arthropathy. Therefore, patients with PR needs to be cautiously followed up for their potential of diverse outcome other than RA: RA, SLE, SjS, and BD in younger patients, RA in males, and AS in females, in particular.Disclosure of Interests:None declared
Epidemiology and Clinical Features of Birt-Hogg-Dubé Syndrome: A Nationwide Population-Based Study in South Korea
