Original paper Reumatologia 2019; 57, 1: 8–18 DOI: https://doi.org/10.5114/reum.2019.83234 Relapsing polychondritis – analysis of symptoms and criteria Beata Maciążek-Chyra1, Magdalena Szmyrka1,2, Marta Skoczyńska1,2, Renata Sokolik1,2, Joanna Lasocka2, Piotr Wiland1,2 1Clinic of Rheumatology and Internal Medicine, Wrocław University Hospital, Poland 2Department and Clinic of Rheumatology and Internal Medicine, Wrocław Medical University, Poland Abstract Objectives: Relapsing polychondritis (RP) is a rare disease characterised by recurrent inflammation of the cartilaginous structures and proteoglycan-rich organs. The aim of this case series study is to share the 10-year clinical experience of our department in diagnosing RP patients in the context of data from available published studies. Material and methods: A retrospective case analysis of 10 patients with symptoms of RP, hospi- talised at the Department of Rheumatology and Internal Diseases of Wrocław University Hospital between January 2008 and December 2018. Results: Nine out of 10 patients fulfilled at least one of the three sets of the diagnostic criteria. The mean age (±standard deviation) at diagnosis was 54.4 ±13.3 years and ranged from 32 to 73 years. The symptoms suggestive of the RP diagnosis were mainly inflammation of the pinna (in 80% of patients) and laryngeal stenosis (in 20% of patients). The mean age at which initial symptoms were observed was 52.3 ±12.0 years and ranged from 31 to 69 years. Auricular chondritis was the first mani- festation of the disease in 40% of cases (two women and two men) laryngeal chondritis in 20%, nasal chondritis in 10%, and bronchial stenosis in 10%. Other initial symptoms were polyarthritis, which was present in 10% of cases (male) and general symptoms observed in 10%. Conclusions: A thorough analysis of the entire medical history with specific questions about the occurrence of the manifestations of the disease in the past leads to the diagnosis of RP. The RP also should be considered in differential diagnosis of respiratory track narrowings. It is very useful to apply the three sets of criteria simultaneously in the diagnostic process. Key words: symptoms, diagnostic criteria, relapsing polychondritis. Introduction the most recognised symptom of RP, while other clini- cal manifestations are not as typical. The course of the Relapsing polychondritis (RP) is a rare, clinically disease consists of recurrent acute and painful inflam- heterogeneous, multi-systemic inflammatory disease mation in different organs followed by asymptomatic characterised by recurrent inflammation of the cartilag- periods with the silently ongoing inflammatory process. inous structures and proteoglycan-rich organs. It affects Relapsing polychondritis leads to progressive degener- primarily the cartilages of the ear, nose, larynx, tracheo- ation of cartilaginous structure and connective tissue, bronchial tree, and ribs, but the inflammatory process which may cause serious complications such as airway may damage also connective tissue components of collapse or obstruction, deafness, loss of vision, aortic the heart, large vessels (especially the aorta), eyes, in- and other large vessel aneurysms, cardiac arrhythmia, ner ear, skin, joints, kidneys, and other organs. Clinical heart failure, or renal insufficiency [1–3]. manifestations vary from isolated auricular chondritis to Relapsing polychondritis occurs most frequently life-threatening systemic features. Red swollen pinna is between the ages of 40 and 60 years. Data on RP inci- Address for correspondence: Beata Maciążek-Chyra, Department and Clinic of Rheumatology and Internal Diseases, University Hospital in Wrocław, 213 Borowska St., 50-556 Wrocław, Poland, e-mail: [email protected] Submitted: 11.02.2019; Accepted: 19.02.2019 Reumatologia 2019; 57/1 Relapsing polychondritis 9 dence and prevalence comes mainly from national pop- cartilages’ inflammation, and audio-vestibular dysfunc- ulation-based studies. The incidence of RP estimated in tion had to be present. a study performed recently in Hungary was about two In 1979, Damiani et Levine [8] introduced the mod- per million person-years, which is comparable to the ified McAdam’s criteria, which included histological incidence in the US [4]. In Hungary, the overall 10-year changes and response to treatment. The diagnosis could survival was good (75–88.3%) and comparable to that of be made three-way: either based on McAdam’s criteria the general population, which the authors attribute to with no histological tests necessary; in the presence of at early diagnosis. The distribution of incidences across re- least one McAdam’s sign accompanied by typical histo- gions may be suggestive of sunlight exposure and arse- logic changes; or in the presence of chondritis in at least nic drinking water pollution as possible environmental two different sites and a good treatment response [8]. triggers of RP. A high prevalence (56%) of other autoim- In 1986, Michet et al. [9] proposed the most recent mune conditions was found among the Hungarian RP set of criteria, once more based solely on symptoms. In patients, with Sjögren syndrome as the most common order to meet the criteria, a patient has to present either concomitant autoimmune disease [4, 5]. with cartilage inflammation in at least two locations, or Although, comparing to Hungary, the incidence of with a singular-site chondritis accompanied by at least the disease in UK was found to be much lower (0.71 per two of the following symptoms: seronegative arthritis, million person-years), the standardised mortality ratio ocular inflammation, hearing loss, and/or vestibular was over twice as high as in the UK general population dysfunction [9]. Three sets of diagnostic criteria for RP (2.16, 95% CI: 1.24–3.51, p < 0.01), with respiratory tract are presented in Table I. disease, heart disease, and cancer as the most fre- Relapsing polychondritis poses a diagnostic chal- lenge due to its rarity of occurrence, wide variety of quent causes of death. The mean age at diagnosis was clinical manifestations, and lack of validated criteria. 55 years for men and 51 years for women, while the peri- The aim of this study is to compare the clinical picture in od from first symptoms to the diagnosis was on average patients with RP based on the experience of one centre, 1.9 years [6]. At present, the population-based studies as well as reference to the available literature. concerning RP are scarce. No such study has been per- formed among the Polish population as of today. In 1976, McAdam et al. [7] published a prospective Material and methods study of 23 patients and created the first set of diagnos- A retrospective case analysis of 10 patients (six wom- tic criteria. In order to establish the diagnosis, at least en and four men) with symptoms of RP, hospitalised at three symptoms such as bilateral auricular chondritis, the Department of Rheumatology and Internal Diseases nasal chondritis, nonerosive seronegative inflammatory of Wrocław University Hospital between January 2008 polyarthritis, ocular inflammation, tracheal or laryngeal and December 2018, was carried out. Table I. Three sets of diagnostic criteria for relapsing polychondritis McAdam et al. (1976) Damiani et al. (1979) Michet et al. (1986) Symptoms Bilateral auricular A criteria: A criteria: chondritis Bilateral auricular chondritis Auricular cartilage inflammation Nasal chondritis Nasal cartilage inflammation Nasal cartilage inflammation Respiratory tract Respiratory tract chondritis Laryngotracheal cartilage chondritis Non-erosive seronegative polyarthritis inflammation Non-erosive seronegative Ocular inflammation B criteria: polyarthritis Audiovestibular damage Seronegative arthritis Ocular inflammation Ocular inflammation Audiovestibular damage Hearing loss Vestibular dysfunction B criterion: Histological confirmation C criterion: Positive response to corticosteroids or dapsone Required criteria ≥ 3 ≥ 3 (A) or ≥ 1 (A) and (B) ≥ 2 (A) or ≥ 1 (A) and 2 (B) or ≥ 2 (A) criteria and (C) Reumatologia 2019; 57/1 10 Beata Maciążek-Chyra, Magdalena Szmyrka, Marta Skoczyńska, et al. Table II. Case analysis of diagnostic criteria fulfilment Case I II III IV V VI VII VIII IX X Fulfilment of criteria according to McAdam et al. Yes No Yes No Yes No Yes No No Yes The number of present criteria according to McAdam et al. 3 2 4 2 5 2 6 1 1 4 Fulfilment of criteria according to Michet et al. Yes Yes Yes Yes Yes Yes Yes No No Yes The number of present A criteria 2 2 2 2 3 2 3 1 1 1 The number of present B criteria 0 0 2 0 1 0 4 0 0 2 Fulfilment of criteria according to Damiani et al. Yes Yes Yes No Yes Yes Yes Yes No Yes 3 McAdam’s criteria present Yes No Yes No Yes No Yes No No Yes 1 McAdam’s criterion and positive histological biopsy NA Yes Yes NA NA Yes NA Yes No NA 2 McAdam’s criteria and positive response to Yes Yes Yes No Yes Yes NA No NA Yes corticosteroids or dapsone NA – not applicable All of them were of Caucasian origin, n = 9 were Pol- polyarthritis that was present in 10% of cases and gen- ish and n = 1 was French. The average age at the onset eral symptoms observed in 10%. The latter are common of the first symptoms in our group was 53.2 years. The for many different disorders and were established as first female to male ratio was 3 : 2. The clinical features, in- signs of RP only on retrospective analysis. In two cases cluding first symptoms, age of onset, time from onset polyarthritis and general symptoms emerged as the first to diagnosis, and laboratory findings at first hospitalisa- manifestation, and the suggestion of the diagnosis of RP tion at our Department were analysed. Diagnoses were was made after the occurrence of redness and oedema verified according to the three sets of criteria (charac- of the pinna. terised by Damiani and Levine, Michet, and McAdam, Comparative characteristics of the examined pa- respectively) [7–9].