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Results for “Polychondritis, Relapsing”

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PubMed1979

Relapsing polychondritis: unusual neurological findings and therapeutic efficacy of dapsone.

Relapsing polychondritis is a rare disease of undetermined cause. The most frequently seen symptom is redness and swelling of the cartilaginous ear, followed by cartilage inflammation elsewhere in the body. Our five cases had neurologic symptoms that posed diagnostic difficulties. Because vasculitis is not uncommon in relapsing polychondritis, it was difficult to determine whether these neurologic symptoms were attributable to relapsing polychondritis or to other unrelated causes. In our cases, specific neurologic complaints were caused by a concomitant but unrelated disease, rather than by relapsing polychondritis. We add five additional documented cases to the medical literature and confirm the therapeutic efficacy of dapsone in the treatment of this unusual disease.

Adult↗

PubMed2005

Relapsing polychondritis with aortitis without valvular involvement.

Relapsing polychondritis is a multisystemic disease of unknown etiology that mainly involves the cartilaginous portions of the ear, nose, and trachea. Occasionally, there is involvement of the cardiovascular system, which usually results in severe morbidity and mortality. The most common manifestation of cardiovascular involvement is aortic root dilation resulting in aortic regurgitation. We describe the first case in Korea, a 51-year-old woman with relapsing polychondritis with aortitis, but without aortic valve involvement. She presented only with complaints of abdominal pain, and was successfully treated with corticosteroids and methotrexate.

Abdominal Pain↗

PubMed2004

Sweet's syndrome revealing relapsing polychondritis.

Sweet's syndrome is extremely rare in relapsing polychondritis. We report a rare case of Sweet's syndrome revealing relapsing polychondritis. A 77-year-old man presented with fever, associated with respiratory symptoms and non pruritic, painful, erythematous, papules and plaques mainly on the arms and legs. Skin biopsy of the arm found a neutrophilic dermal infiltrate consistent with Sweet's syndrome. A month and a half after admission, the patient presented with ear chondritis and dysphonia. Biopsy of the ear cartilage found a significant perichondral inflammatory cells infiltrate. The diagnosis of relapsing polychondritis was established and a steroid treatment was instituted with a complete regression of the cutaneous signs and chondritis.

Aged↗

PubMed1997

Isolated mitral regurgitation complicating relapsing polychondritis.

The reported incidence of mitral regurgitation in relapsing polychondritis ranges from 2 to 3% and is associated with aortic regurgitation. There are no reports that mitral regurgitation can be an isolated cardiac complication of relapsing polychondritis. This case report demonstrates that partial chordal rupture and consequent severe mitral regurgitation can be the only features of cardiac involvement in relapsing polychondritis.

Adult↗

PubMed2006

[Acute mesenteric ischemia in a patient with relapsing polychondritis].

We report about a 43-year old woman with relapsing polychondritis, admitted with progressive malaise, fatigue, anorexia and profound weight loss. Two years ago a nasal chondritis with characteristic changes of the nasal profil, scleritis, laryngitis and unspecific general symptoms (fever, fatigue, signs of a systemic inflammation) developed and relapsing polychondritis was diagnosed. The patient was treated initially with azathioprine followed by methotrexate in combination with ciclosporine and glucocorticoids. During the hospitalization her condition slowly worsened, and crampy abdominal pain developed subsequently with massive tenderness, rebound and guarding. The clinically presumed diagnosis of ileus and peritonitis was radiomorphologically confirmed with signs of enteric perforation and a laparatomy was performed. A complete occlusion of the A. mesenterica superior and stenosis of the truncus coeliacus were diagnosed followed by a revascularization with an aorto-mesenteric bypass and subsequent resection of the necrotic ischemic ileum. An association with various autoimmune disorders including vasculitic syndromes is well known for relapsing polychondritis. The case demonstrated a progressive mesenteric ischemia with the acute exacerbation caused by a vasculitic mesenterial occlusion. The unusual presentation should be considered in the differential diagnosis of uncommon abdominal symptoms during the course of relapsing polychondritis.

Acute Disease↗

PubMed1999

Relapsing polychondritis diagnosed by Tc-99m MDP bone scintigraphy.

PURPOSE: Relapsing polychondritis is a generalized recurring disease of cartilage that involves joints, trachea, bronchi, laryngeal cartilages, costal cartilages, and cartilages of the ear and nose. It is associated with autoimmune diseases, including Hashimoto disease in some cases. METHODS: The authors evaluated a 29-year-old man with relapsing polychondritis who had symptoms and signs of a common cold for 2 months and anterior chest pain near the sternum for 1 month. RESULTS: After the diagnosis, the authors found that the patient had a history of thyroid therapy for hyperthyroidism 15 years before. Tc-99m MDP bone scintigraphy performed to evaluate anterior chest pain showed diffusely increased accumulation of radioactivity in all costocartilages and sternoclavicular joints. Based on that information, relapsing polychondritis was diagnosed. Ga-67 citrate scintigraphy was preformed to determine the optimum biopsy site of the cartilage. The diagnosis was histologically supported by the results of the open biopsy. CONCLUSIONS: In this case, Tc-99m MDP bone scintigraphy was useful for diagnosing relapsing polychondritis, and Ga-67 citrate scintigraphy was helpful in determining the biopsy site.

Adult↗

PubMed2004

Hypopyon uveitis and relapsing polychondritis: a report of 2 patients and review of autoimmune hypopyon uveitis.

OBJECTIVE: To report hypopyon uveitis as an ophthalmic finding associated with relapsing polychondritis. DESIGN: Interventional case reports. PARTICIPANTS: Two patients with hypopyon uveitis as a manifestation of relapsing polychondritis. METHODS: Complete ocular and systemic examinations as well as chart reviews were performed for 2 patients with hypopyon uveitis as a manifestation of relapsing polychondritis at presentation. MAIN OUTCOME MEASUREMENTS: Clinical observation and response to treatment in 2 patients with hypopyon uveitis secondary to relapsing polychondritis. RESULTS: A 70-year-old white male had unilateral hypopyon uveitis accompanied by a migratory polyarthralgia at presentation. Fifteen months later, he sought treatment for chondritis of the right auricle and was diagnosed with relapsing polychondritis. A 42-year-old white female with known relapsing polychondritis had bilateral hypopyon uveitis at presentation during a systemic relapse. Both patients had resolution of the hypopyon with topical and systemic steroids. CONCLUSIONS: Relapsing polychondritis should be considered in the differential diagnosis of sterile hypopyon uveitis. Steroids and systemic immunosuppression can be used to treat the uveitis.

Adult↗

PubMed2001

Relapsing polychondritis associated with monoclonal gammopathy in a patient with myelodysplastic syndrome.

Relapsing polychondritis is a rare condition characterized by inflammation and subsequent degeneration of cartilages. Deformity of the pinna, nasal saddling and stridor due to involvement of the cartilages of the respiratory tract may lead to patients being referred to the otolaryngologist for initial assessment and further management. Recent observations have suggested that relapsing polychondritis may occur as a paraneoplastic phenomenon in cases of myelodysplasia. The case of a patient with relapsing polychondritis, myelodysplastic syndrome and a monoclonal gammopathy is presented. The authors highlight the apparent existence of this association and encourage otolaryngologists to consider such possible links when cases of relapsing polychondritis present to the outpatients department.

Aged↗

PubMedDate not supplied

[Polymorphic ocular involvement in relapsing polychondritis: an early, not always recognized symptom].

Relapsing polychondritis is a rare disorder of unknown etiology. It is characterized by arthropathy, inflammation of auricular and nasal cartilage, and various ocular complications. Potentially destructive inflammatory lesions involve cartilaginous structures and the cardiovascular system. In three reported cases multiple ocular complications and arthropathy were early symptoms of relapsing polychondritis.

Adult↗

PubMed1983

Relapsing polychondritis. A case report and literature review.

Relapsing polychondritis is a systemic disorder characterized by recurrent inflammation and degeneration of cartilaginous tissue. The eyes, ears, nose, larynx, trachea, and articular areas are commonly involved. While ocular involvement, hearing, and vestibular dysfunction are frequently present, aortic valve and root disease as well as vasculitis also may occur. While the cause remains unknown, an autoimmune pathogenesis appears likely. Thus, dapsone and/or systemic corticosteroids remain the predominant effective therapies. Another case of relapsing polychondritis with an associated vasculitis syndrome is presented as well as a review of the current literature.

Arthritis, Rheumatoid↗

PubMed2004

Relapsing polychondritis, induced in mice with matrilin 1, is an antibody- and complement-dependent disease.

Relapsing polychondritis is an autoimmune disease that affects cartilage in the ear, nose, and respiratory tract. A pathogenic immune response has been proposed and antibodies to several cartilage proteins are detected in sera from these patients. To investigate the role of the humoral immune response in relapsing polychondritis, we used the matrilin-1-induced relapsing polychondritis model. Mice deficient of B cells (muMT) and mice congenic at the complement factor 5, were immunized with matrilin-1, a cartilage-specific protein mainly detected in the tracheal cartilage. To investigate the binding properties and tissue selection of matrilin-1-specific antibodies we produced matrilin-1-specific B-cell hybridomas. Although 83% of the micro MT heterozygous mice developed respiratory distress and erosive chondritis in the respiratory tract, none of the B-cell-deficient mice were susceptible to disease. In addition, we show that complement factor 5 is important for the induction of matrilin-1-induced relapsing polychondritis. Monoclonal matrilin-1-specific antibodies injected into neonatal mice bound specifically to cartilage of the respiratory tract and adult B-cell-deficient mice injected with the same antibodies developed erosive chondritis in the respiratory tract. We conclude that relapsing polychondritis can be mediated by a pathway involving tissue-specific antibodies and complement activation.

Animals↗

PubMed1990

Relapsing polychondritis--a report on two Chinese patients with severe costal chondritis.

Relapsing polychondritis is a rare multisystemic connective tissue disorder found mainly in Caucasians. Oriental patients with relapsing polychondritis are uncommon. We report 2 Chinese patients with relapsing polychondritis who had severe laryngotracheal narrowing requiring tracheostomy. Unlike most patients, there was marked costal chondritis resulting in depression of the anterior chest wall with minimal involvement of other joints. One of the patients had treatment with prednisolone, dapsone, azathioprine, cyclophosphamide and cyclosporin A at various stages of the disease without significant remission of the disease.

Adult↗

PubMed1995

The role of MRI when relapsing polychondritis is suspected but not proven.

Relapsing polychondritis (RP), while relatively rare, presents a characteristic clinical picture. Based upon a symptom complex of auricular, nasal, and respiratory chondritis associated with ocular and otic complaints, diagnosis can frequently be made with confidence in the absence of histologic confirmation. We present a case where a therapeutic intervention was required without sufficient criteria for diagnosis. Magnetic resonance imaging (MRI) proved useful in initial evaluation and allowed follow-up imaging demonstrating a gratifying response to steroid treatment. We believe that MRI has an important role in the diagnosis and subsequent evaluation of patients with proven or suspected RP, particularly those with tracheal involvement.

Airway Obstruction↗

PubMed1998

Migratory leptomeningeal inflammation with relapsing polychondritis.

We report a case of relapsing polychondritis with focal sensorimotor seizures, aseptic meningitis, and migratory leptomeningeal enhancement on contrast MRI. These abnormalities on imaging studies correlated accurately with laterality of the patient's seizures, facilitating early aggressive management of his neurologic symptoms.

Aged↗

PubMed1997

Treatment of diffuse tracheomalacia secondary to relapsing polychondritis with continuous positive airway pressure.

Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation and destruction of the cartilaginous structures. Tracheobronchial chondritis is a dreaded complication of RP. We wish to report a case of RP of the trachea and bronchi which was treated with nasal continuous positive airway pressure.

Fatal Outcome↗

PubMed2003

Relapsing polychondritis--an Oriental case series.

INTRODUCTION: Relapsing polychondritis (RPC) has been described mainly in Caucasian populations. Reports from other ethnic groups are few. OBJECTIVES: To describe the clinical characteristics, management and outcome of RPC patients seen in an Oriental population in Singapore. METHODS: The case records of RPC patients treated in our department from 1989 to 2001 were reviewed. Only 12 fulfilled the McAdam-Michet-Damiani-Levine diagnostic criteria and these were studied. RESULTS: The female-to-male ratio in our series was 3:1. There were 10 ethnic Chinese and two Malay patients. The age of onset of symptoms ranges from three to 65 years, with a mean of 34 years. A diagnosis was made from two weeks to three years after onset, with a median of 4.5 months. There were 10 patients with pinna, nine articular, eight ocular, six laryngotracheal, five inner ear, four nasal and one cardiac involvement. Five presented with fever. None of them had cutaneous, renal or central nervous system involvement. Ten had raised ESR at presentation. One patient developed discoid lupus erythematosus two years later. All 12 patients received prednisolone with eight of them requiring additional immunosuppressants. Two patients had resistant disease failing to respond adequately to various immunosuppressants together with prednisolone. There was no mortality amongst the nine patients who had remained on follow-up at the time of this report. Five of the six patients with laryngotracheal involvement had tracheostomy and one of them had airway stenting as well. CONCLUSION: Our series suggests that although the clinical manifestations of RPC are similar in the Oriental and the Caucasian populations, Oriental patients may have less cutaneous, renal or nervous system involvement and more serious airway complications.

Adolescent↗

PubMed1977

Vascular involvement in relapsing polychondritis.

Review of four cases of relapsing polychondritis (RP) seen at one hospital in the 12-year period 1963 to 1974 revealed that one patient had aortic insufficiency with large artery involvement, two others had involvement of medium and large arteries and the fourth may have had mucocutaneous vasculitis. Valvular disease has occurred in 9% of all cases of RP reported in the literature and, if vasculitis beyong the aortic root is included, 25% of cases of RP manifested inflammatory vascular disease. The frequency of pseudotumour of the orbit and cochlear-labyrinthine dysfunction is also high and may be a manifestation of vasculitis.

Aged↗

PubMed1989

[Relapsing polychondritis: report of 4 cases].

Four cases of Relapsing Polychondritis followed in the Hospital do Servidor Publico Estadual de Sao Paulo, since 1985, are reported and discussed. Relapsing Polychondritis, an auto-immune disease that destroys the pinnal and nasal cartilage has been observed occasionally around the world. Since it was first defined and described, in 1923, about 250 cases have been reported.

Adult↗