Scleritis in Relapsing Polychondritis

Hoang–Xuan, T.; Foster, C. Stephen; Rice, Beverly A.

doi:10.1016/s0161-6420(90)32485-5

Cited by 94 publications

(45 citation statements)

References 20 publications

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“…Airway management along with early pharmacotherapy can lead to relief of symptoms. 2,7,9 Glucocorticoids, other immunosuppressive agents (such as methotrexate, cyclophosphamide, colchicine, and azathioprine), and anti-tumor necrosis factor agents have been used with varying outcomes [10][11][12][13][14][15][16] Relapse of the disease is common when therapy is tapered off.…”

Section: Discussionmentioning

confidence: 99%

Respiratory Failure Secondary to Relapsing Polychondritis

2014

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Section: Discussionmentioning

confidence: 99%

Respiratory Failure Secondary to Relapsing Polychondritis

2014

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“…However, there can be areas of active inflammation that are difficult to detect, including at the trachea, cardiac valves, and large arteries. Cyclophosphamide and azathioprine are reported to be effective in treating renal and ophthalmic involvement, but their use has been limited to organ-threatening, steroid-resistant disease activity (13,14).…”

Section: Discussionmentioning

confidence: 99%

Progressive aortic valve inflammation occurring despite apparent remission of relapsing polychondritis

Buckley

Ades

1992

Arthritis & Rheumatism

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We describe a patient with relapsing polgchondritis in whom aortic valve inflammation developed 3 years after diagnosis, when the polychondritis had been in apparent remission for an extended period of time. Infection and cardiac involvement can be significant complications of relapsing polychondritis. Recommendations for monitoring and treatment of patients with this disease are discussed.Relapsing polychondritis is a systemic autoimmune disease characterized by episodic inflammation of cartilaginous structures (nose, ear, and trachea). It is associated with inflammatory arthritis and occasionally with vasculitis, aortitis, and inflammation of the aortic valve and ring. We report a case of relapsing polychondritis in which aortic valve involvement developed 3 years after diagnosis, when the patient was otherwise asymptomatic, had not been receiving therapy for 7 months, and had a normal erythrocyte sedimentation rate (ESR). CASE REPORTThe patient was well until April 1985 when, at the age of 35, he developed chronic otitis media which did not respond to antibiotic treatment. Over the ensuing 4 months he developed iridocyclitis, cough and hemoptysis, fatigue, myalgias, and arthralgias. In October he noted the onset of erythema and tenderness of the bridge of the nose and external otitis media, which resolved without treatment.On presentation to us 4 weeks later, his physical examination findings were notable only for costochondritis and swelling of the second proximal interphalangeal joint of the left hand. Cardiac examination revealed the absence of murmurs. Urinalysis results, creatinine levels, and chest radiography findings were within normal limits. The ESR (Westergren) was 94 mm/hour, and antinuclear antibodies were present at a titer of 1 : 160. Assays for anticollagen antibodies, rheumatoid factor, and complement levels all yie!ded normal results.Relapsing polychondritis was diagnosed and the patient was begun on a regimen of prednisone, 50 mg/day. He responded well with resolution of symptoms, and the prednisone was tapered over 2 years, and then discontinued. During this time he developed occasional worsening of the iridocyclitis and joint pain, but had no further episodes of inflammation of the nasal or ear cartilage.The patient had been asymptomatic and not receiving treatment for 7 months when he returned for followup. Examination findings at that time were remarkable for a III/VI systolic and diastolic murmur at the left sternal border (second intercostal space), which radiated to the carotid artery. Echocardiography revealed aortic regurgitation. The ESR was 16 mmhour. He felt well and continued to participate in competitive soccer.However, a followup echocardiogram 6 months later showed progressive aortic regurgitation and left

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“…Since tracheal stenosis based on this cartilage inflammation can be life-threatening when the initiation of treatment is not prompt, the selection of immunosuppressive therapy for RP is crucial. When steroid therapy fails to reduce inflammation or airway involvement in RP, its combination with other immunosuppressive agents including MTX, cyclophosphamide (CY), azathioprine, or cyclosporin A can be considered [4][5][6]. Yamaoka et al [10] previously reported the efficacy of a combination therapy of glucocorticoids, MTX and CY.…”

Section: Discussionmentioning

confidence: 99%

“…Immunosuppressive therapy is used to treat RP, for which the use of glucocorticoids and other immunosuppressive agents, including cyclosporin A and cyclophosphamide, has been reported [4][5][6]. The recent development of biological agents, such as tumor necrosis factor (TNF) inhibitors, has drastically changed the treatment of autoimmune disorders, especially rheumatoid arthritis (RA) [7].…”

Section: Introductionmentioning

confidence: 99%

Efficacy of adalimumab for a refractory case of relapsing polychondritis with reduction of pro-inflammatory cytokines

et al. 2011

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A 68-year-old man with a fever of unknown origin was admitted to our hospital. Bilateral auricularis and nasal root inflammation, with auricular perichondritis positive for anti-type II collagen (CII) antibody, led to a diagnosis of relapsing polychondritis (RP). Because the patient was refractory for high-dose glucocorticoid therapy, adalimumab was used. After the initiation of 40 mg of adalimumab, a rapid improvement of clinical manifestations, with a reduction in tumor necrosis factor-alpha (TNF-α) and interleukin 6 (IL-6), together with a titer of anti-CII antibody was observed.

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Scleritis in Relapsing Polychondritis

Cited by 94 publications

References 20 publications

Respiratory Failure Secondary to Relapsing Polychondritis

Respiratory Failure Secondary to Relapsing Polychondritis

Progressive aortic valve inflammation occurring despite apparent remission of relapsing polychondritis

Efficacy of adalimumab for a refractory case of relapsing polychondritis with reduction of pro-inflammatory cytokines

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