Relapsing Polychondritis: A Rare Case Report.

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology characterized by recurrent episodes of inflammation and the destruction of cartilaginous tissues, primarily involving the ear, nose, and the respiratory tract. Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the diseases progress. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in the saddle nose deformity. In patients with RP, surgical management for saddle nose is carefully decided due to the disease relapsing characteristics. We present a RP patient with a saddle nose deformity who underwent reconstruction rhinoplasty with autologous costal cartilage grafting. At 6-month follow-up, the patient retained good esthetic results and showed neither complication nor relapse of RP.

Relapsing polychondritis is characterized by recurrent episodes of painful inflammation and destructive change of cartilage of auricle, nose and trachea, often accompanied by systemic vasculitis with inner ear, cardiovascular, neurological, cutaneous and renal involvement. This disease is very rare and probably caused by autoimmune meshanism. The authors experienced 37 years old female patient of relapsing polychondritis with recurrent perichondritis of both auricles, saddle nose deformity, trachitis, and polyarthritis. This case had been performed tracheal fenestration to maintain the airway, and had successfully delivered full-term baby.

Relapsing polychondritis (RP) is a rare progressive inflammatory condition involving cartilaginous structures, predominantly those of the ears, nose and laryngotracheobronchial tree. The McAdam’s diagnostic criteria require meeting three out of...

Three cases of relapsing polychondritis are reported. They all complained of dyspnea, which derived from tracheal involvement. Other clinical manifestations were saddle nose (3 cases), painful swelling of ear (2 cases), arthralgia (1 case) ophthalmodynia (1 case). The pathological examination of biopsied cartilage of the pinnae showed loss of basophils with cellular infiltration, fibrosis and breakdown of the matrix. We also performed PAP (Peroxidase-Anti-Peroxidase) staining of biopsied cartilage, which showed the deposition of IgG, C3, C4 and fibrinogen. The results suggested that the pathogenesis of RP might be related to the autoimmune mechanism.

Relapsing polychondritis (RP) is a very rare autoimmune disorder characterized by recurrent episodes of inflammation and destruction of cartilaginous tissues. We examined the clinical characteristics, management, and outcomes of Japanese RP patients. We identified 8 RP cases in our department between 2003 and 2017. Detailed clinical features, testing, treatment, and outcomes were recorded. The mean time from symptom onset to diagnosis was 9 months. Four cases presented with auricular chondritis and laryngotracheal involvement and 3 cases presented with a saddle nose deformity. Anti-type II collagen antibody was positive in 5 of 6 cases. Of 3 cases with associated diseases (rheumatoid arthritis, ulcerative colitis, and Sjögren's syndrome), 2 died of respiratory failure. When RP is diagnosed, early computed tomography or pulmonary function testing is essential to enable early treatment. Undiagnosed airway involvement can cause tracheobronchial wall fibrosis, leading to fixed stenosis.

Relapsing polychondritis in an adolescent

Subclinical aortitis in a case of polychondritis

Relapsing polychondritis is a rare disease of uncertain etiology, characterized by inflammation of cartilage. Cartilage of the respiratory tract is engaged in more than half of all cases. Hydralazine-induced systemic lupus erythematosis (SLE) is a well known disease. Females who are slow acetylators and are HLA-DR4 positive when treated with hydralazine are at serious risk of contracting SLE syndrome. We describe here a woman, treated for 10 years with hydralazine, who was a slow acetylator and was HLA-DR4 positive and who presented with a relapsing polychondritis and ultimately required a permanent tracheostoma. To our knowledge, this is the first published case of relapsing polychondritis induced by hydralazine.

Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant –SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP – including the inner ear dysfunction – were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.

Pediatric-Onset Relapsing Polychondritis: Case Series and Systematic Review

Background:This pilot study aims to evaluate the effectiveness and safety of baricitinib in relapsing polychondritis (RP) patients.Objectives:Aim of this case report was to show efficacy of baricitinib in a patients...

Relapsing polychondritis (RP) is a rare multisystem disease characterised by progressive inflammation and destruction of cartilaginous structures. Airway involvement in RP occurs in approximately 50% of cases. We present a 70-year-old woman with a diagnosis of early adult onset asthma. She required multiple hospital admissions for recurrent episodes of acute severe dyspnoea for 1 year. These were treated as asthma. Months later she developed saddle nose deformity and hoarseness of voice. CT revealed tracheal/bronchial wall thickening and luminal narrowing. Based on these findings, RP was diagnosed. Insertion of a tracheobronchial stent was necessary due to severe tracheobronchomalacia. This intervention improved the patient9s dyspnoea immediately. This case is reported to raise awareness of airway involvement in RP and discuss its current management. Early diagnosis of RP is essential to allow prompt treatment and to decrease the risk of life-threatening airway collapse.

Relapsing polychondritis (RP) evolves with variable and intermittent involvement of cartilage and proteoglycan-rich structures. Ocular manifestations are present in up to two-thirds of RP patients. Necrotising scleritis (NS) and peripheral ulcerative keratitis (PUK) may be inaugural and may lead to eye perforation and vision loss. We aimed to review NS and PUK in RP, in order to characterise them, to identify successful treatment options and unmet needs. A systematic review of the currently available evidence in PubMed, EMBASE and Scopus was performed according to PRISMA, including observational studies, single case reports and case series of NS/PUK in RP. Study design, number of patients, age, gender, treatment and outcome, were extracted. Two RP patients also provided their opinion. Five case reports and two case series were eligible for inclusion. We identified 10 RP patients with eye-threatening complications (NS and/or PUK), 9 adults (2 males, 7 females, aged 35-72, median age 57.6 years) and one paediatric patient (F, 11 years). Apart from glucocorticoids, cyclophosphamide was effective in 4 patients; infliximab, high-dose immunoglobulins, dapsone, or cyclosporine were also successfully employed in a case each. Surgical repair was reported in 2 cases. Ocular inflammation is often bilateral and recurring in RP; NS/PUK are rare complications. All patients who develop NS/PUK should be specifically questioned for RP signs and symptoms. Early institution of immunosuppressive therapies is mandatory. Increasing awareness, physicians' and patients' education and a multidisciplinary approach may help improve the prognosis of these serious complications of RP.

Relapsing polychondritis is a rare autoimmune disease associated with inflammation and destruction of cartilage and connective tissue.We report on a patient with a severe form of this disease that had a progressive and complicated course despite administration of a number of disease-modifying anti-rheumatic drugs.Finally, therapy with the TNF-alpha-antagonist etanercept was initiated, which led to a considerable decrease in disease activity. This case is further evidence for the efficiency of TNF-alpha-antagonists in relapsing polychondritis.

BackgroundTo report rapid corneal thinning and perforation in a case with relapsing polychondritis.Case presentationA 43 year-old male diagnosed with relapsing polychondritis suffered from bilateral scleritis, bilateral swelling of pinna, saddle nose and tracheal stenosis. The patient presented with right eye pain and redness for one month. Slit lamp examination of the right eye showed 80% peripheral corneal thinning between 3 and 7 o’clock. The best-corrected visual acuity (BCVA) was 1.0 bilaterally. The degree of corneal thinning worsened to 90% after one week of oral corticosteroid use. Subsequently, topical cyclosporine 2% eye drops four times a day, oral doxycycline 100 mg twice a day and oral vitamin C 2 g daily were added. The corneal thinning gradually improved to about 60%. However, the patient rapidly tapered oral prednisolone against medical advice and returned with an acute drop in vision in his right eye. Slit lamp examination of the right eye showed peripheral corneal perforation with iris prolapse. An emergency repair with cyanoacrylate glue was performed. Intravenous methylprednisolone 1 mg/kg body weight was administered for three days and 1 g/day intravenous immunoglobulin was administered every four weeks. At 3 months postoperatively, BCVA in the right eye was 0.6. Slit lamp examination showed a well-formed anterior chamber with glue in situ.ConclusionsRelapsing polychondritis may be associated with rapid corneal thinning. The clinicians should be aware of the possibility of corneal perforation in these cases. Cyanoacrylate glue is a viable temporary management option in such scenarios.