A nationwide study of the epidemiology of relapsing polychondritis

Abstract

ObjectiveRelapsing polychondritis (RP) is a rare autoimmune inflammatory disease that attacks mainly cartilaginous structures or causes serious damage in proteoglycan-rich structures (the eyes, heart, blood vessels, inner ear). This study shows results regarding the epidemiology, progression, and associations of this highly variable disease by collecting all cases from a 124-million-person-year Central European nationwide cohort.MethodsWe used the Hungarian Health Care Database to identify all persons with possible RP infection. We followed patients who had International Classification of Diseases 10th edition code M94.1 at least once in their inpatient or outpatient records between January 1, 2002 and December 31, 2013 in Hungary. We classified these patients into disease severity groups by their drug consumption patterns between January 1, 2010 and December 31, 2013. We analyzed the regional distribution of RP incidences as well. Overall maps of comorbidity are presented with network layouts.ResultsWe identified 256 patients with RP among cumulatively 11.5 million registered inhabitants. We classified these patients into four severity classes as “extremely mild” (n=144), “mild” (n=22), “moderate” (n=41), and “severe” (n=4). Two additional groups were defined for patients without available drug data as “suspected only” (n=23) and “confirmed but unknown treatment” (n=22). The age and sex distributions of patients were similar to worldwide statistics. Indeed, the overall survival was good (95% confidence interval for 5 years was 83.6%–92.9% and for 10 years was 75.0%–88.3% which corresponds to the overall survival of the general population in Hungary), and the associations with other autoimmune disorders were high (56%) in Hungary. Almost any disease can occur with RP; however, the symptoms of chromosomal abnormalities are only incidental. Spondylosis can be a sign of the activation of RP, while Sjögren syndrome is the most frequent autoimmune association. Regional distribution of incidences suggests arsenic drinking water and sunlight exposure as possible triggering factors.ConclusionThe good survival rate of RP in Hungary is probably associated with the early diagnosis of the disease.

Relapsing polychondritis, an underestimated dermatological urgency: case report and literature review

“…The main joint manifestations are arthralgias related to mono‐oligoarticular arthritis of large and small joints. The studies of synovial fluid are negative, and the disease is adequately controlled with NSAIDs, in cases with low activity . Our case presented asymmetric polyarthralgias, so it was necessary to rule out other differential diagnoses such as connective tissue disease (CTD) or a myelodysplastic syndrome (MDS) .…”

“…The studies of synovial fluid are negative, and the disease is adequately controlled with NSAIDs, in cases with low activity. 1,2,9,14,15,28,29 Our case presented asymmetric polyarthralgias, so it was necessary to rule out other differential diagnoses such as connective tissue disease (CTD) or a myelodysplastic syndrome (MDS). 14,28,29 The eye involvement is not a strange manifestation, being the scleritis and episcleritis are the earliest manifestations of RPC; however, posterior uveitis is the most severe ocular manifestation, as occurred in our patient, and these data are consistent with Dion J et al…”

“…Damiani and Levine proposed a modification of these criteria: for the diagnosis, (i) all patients had to have at least three or more McAdam diagnostic criteria, the histologic confirmation is not necessary; (ii) one or more of McAdam's signs with histologic confirmation; (iii) chondritis in two or more different anatomic locations with response to steroids and/or dapsone treatment. The epidemiological data show an incidence of approximately 0.71, 2.0, and 3.5 per million from population studies in the United Kingdom, Hungary, and Minnesota, USA, respectively, with a slight predominance in Caucasian women than in men (2:1 to 4:1) between the 4th to 5th decade of life, although it can also affect the pediatric population …”

“…Also, the treatment is not established, and there are no algorithms for the management of it, which causes confusion in the medical field. However, owing to the resistance to corticosteroids and therapeutic failure with conventional immunosuppressants, the advent of biological therapy represents a hopeful third line of management …”

Relapsing Polychondritis following Treatment with Secukinumab for Ankylosing Spondylitis: Case Report and Review of the Literature

“…The association between RP and AS has been reported in only few cases over the many decades of study. However, recent work estimates that as many of 12% of patients with AS have concomitant RP, with a risk of lifetime comorbidity risk of 67% to develop RP [4]. Historical treatment of AS focused on nonsteroidal anti-inflammatory drugs (NSAIDs), with more recent therapeutic regimens including TNFi.…”