Comparison of Relapsing Polychondritis Patients with and Without Respiratory Involvement Based on Chest Computed Tomography: A Retrospective Cohort Study

Background Relapsing polychondritis (RP) patients with tracheal cartilage involvement are different from other patients. The objectives of this study were to compare the clinical features and disease patterns between respiratory involvement subgroup and non-respiratory involvement subgroup according to chest computed tomography. Method We performed a retrospective cohort study collected RP patients hospitalized at the Beijing Chao-Yang Hospital between January 2012 - August 2021. Results The incident of costochondritis was more frequent in RP patients with respiratory involvement(p=0.03), the incidence of inflammatory eye disease(p=0.001) and auricular chondritis(p=0.001) was less frequent in RP respiratory involvement patients, compared with those of RP patients without respiratory involvement. Correlation analysis showed that a negative correlation between respiratory involvement and auricular chondritis (r=-0.58, p < 0.01), and between respiratory involvement and inflammatory eye disease (r=-0.45, P < 0.01). Auricular chondritis was positively correlated with inflammatory eye disease (r=0.49, P < 0.01). Compared with non-respiratory involvement subgroup, the incidence of pulmonary infection marginally increased in respiratory involvement subgroup(p=0.06). Inflammatory indexes except for CAR were significantly higher in respiratory involvement subgroup, subgroup analysis found that there was no significant relationship between inflammatory indexes and pulmonary infection. Conclusion RP patients with respiratory involvement was characterized by higher rate of costochondritis and pulmonary infection, fewer inflammatory eye disease and auricular chondritis compared to non-respiratory involvement. Increase inflammatory indexes may suggested that patients with respiratory involvement had a higher disease activity index of RP. The probability of survival was not significant between two subgroups.

Relapsing polychondritis: an unusual cause of sore throat, painful ear swelling and bilateral costal margin pain

A 54-year-old Chinese woman presented with a 3-month history of sore throat and dry cough, which was treated as chronic pharyngitis with minimal improvement. One month ago, she presented with painful right ear swelling without signs or symptoms of otitis media or otitis externa. She was treated with antibiotics and antiviral drugs without any improvement. Two weeks prior to her presentation to hospital, she developed bilateral costal margin pain with raised C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), mild chronic pharyngitis and painful right ear swelling. All other investigations including bloods and imaging were non-specific. Her painful right auricle swelling prompted the diagnosis of relapsing polychondritis (RP), which was supported by clinical improvement with high-dose corticosteroids. RP is a clinical diagnosis with non-specific inflammation of affected cartilage. It is a diagnosis of exclusion and early diagnosis can be made by the most common presenting feature of auricular chondritis.

Can Mechanical Injury Really Trigger Relapsing Polychondritis? The First Report From A Large Case Series

Objectives The triggers of relapsing polychondritis (RPC) are not fully characterized. This study was performed to explore the association between mechanical injuries and RPC.Methods The history of mechanical injuries of 127 RPC patients was reviewed and confirmed. The characteristics and survivals of patients with mechanical injuries were analyzed. Results Fifty-four patients (42.5%) had 63 mechanical injuries, among which 17 were cartilage-related. Thirty mechanical injuries in 28 patients occurred in the preceding one year before disease onset. Patients with mechanical injuries(n=54) had a higher proportion of female gender (59.3% vs 38.4%, P=0.002) and similar features compared to those without injuries(n=73), regarding clinical manifestations and mortality rate. Among 54 patients with mechanical injuries, patients with cartilage related injury(n=17) had a significantly higher rate of tracheobronchial chondritis (64.7% vs 27%, P= 0.008), a significantly lower rate of auricular chondritis (35.3% vs 67.6%, P=0.026) and a relatively higher mortality rate(29.4% vs 8.1%, P=0.041) compared with those with non-cartilage related injury(n=37).Conclusion Our findings suggest that both cartilage-related and non-cartilage related injury may be triggers of RPC and patients with cartilage related injury seem to be more severe than those without.

Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: analysis of 126 Chinese patients

Objective To explore the clinical patterns of relapsing polychondritis(RPC) to improve clinicians’ comprehensive understanding of the disease. Methods The electronic records of RPC patients that were hospitalized and followed-up by rheumatologists at our hospital between January 1, 2008 and August 31,2019 were retrospectively analyzed. Results Sixty-six males and 60 femaleswere included with onset age of 47.1±13.8 years and followed up for a median of 18 months. Eighty-seven patients (69.1%) did not met with the traditional criteria.Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r=-0.75，P<0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%) and patients with Ear pattern and Airway pattern were subdivided as limited and systemic form of RPC (27.8%with limited form of Ear pattern and 24.6% with limited form of Airway pattern). During follow-up, a small part of patients with Ear pattern and Airway pattern progressed into Overlap pattern andsome Airway-Ear negative patternpatients progressed into Ear pattern. Most of the limited RPC patientsremained limited form during follow-up whilesome patients with limited form progressed into systemic form. Patients with Ear pattern had the highest survival rate and relative lower inflammatory status. Conclusion RPC patients can be categorized as 4 differentclinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement.Limited form of RPC is not uncommon in Chinese patients and traditional criteria of RPC are not suitable for early diagnosis.

ANCA-associated life-threatening systemic vasculitis after alemtuzumab treatment for multiple sclerosis

Alemtuzumab is highly effective in relapsing remitting multiple sclerosis (RRMS), but autoimmune adverse events are of concern. In contrast to rare cases of immune-mediated cutaneous vasculitis, systemic vasculitis after alemtuzumab has not yet been described. We report the case of a 29-year-old man with RRMS who developed fever, auricular chondritis, cutaneous vasculitis and life-threatening diffuse alveolar haemorrhage, 12 months after alemtuzumab. Antibodies to myeloperoxidase appeared 9 months after alemtuzumab and were extremely high at the time of vasculitis. Outcome was favourable after glucocorticoids, plasma exchanges and rituximab. Thus, alemtuzumab may induce life-threatening vasculitis in patients treated for RRMS.

Limited auricular relapsing polychondritis in a child treated successfully with infliximab

Relapsing polychondritis (RP) is a rare progressive and destructive multisystem disorder characterised by recurrent inflammation of cartilaginous structures. It is a rare disease in paediatrics compared with adults. In children, the diagnosis is either delayed or overlooked due to low incidence. Auricular chondritis has been described in more than half of paediatric cases with RP. However, isolated auricular chondritis has not been reported as the only presentation of pediatric-onset RP. We described a lad who presented with isolated auricular chondritis, which is refractory to conventional treatment, including glucocorticoids and methotrexate as steroid-sparing agent. Remission of his disease’s relapses was sustained with infliximab. Limited auricular involvement as a presenting feature of RP in the absence of systemic association is very rare in children. We describe a case of successful use of infliximab on limited auricular chondritis disease.