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Results for “Polychondritis, Relapsing”

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PubMed2001

The occurrence of autoantibodies to matrilin 1 reflects a tissue-specific response to cartilage of the respiratory tract in patients with relapsing polychondritis.

OBJECTIVE: Relapsing polychondritis (RP) is an inflammatory disease that mainly affects cartilage tissue in the auricle, nose, and lower respiratory tract. When tracheolaryngeal cartilage is involved, the disease is occasionally fatal. Matrilin 1 is a cartilage-specific protein most prominently expressed in tracheal cartilage, but not in joint cartilage. Immunization with the protein in rats and mice induces respiratory distress and nasal destruction, as seen in RP. We investigated the response to matrilin 1 and other cartilage proteins in sera from patients with RP, 4 additional groups of patients with other major connective tissue diseases, and healthy control subjects. METHODS: Sera were analyzed by enzyme-linked immunosorbent assay (ELISA) for antibody responses to matrilin 1, types II, IX, and XI collagen, and cartilage oligomeric matrix protein (COMP). Titers above the mean + 3SD of controls were considered positive. Specificity of matrilin 1 recognition was further investigated by the capacity of high-titer sera to block the binding of a matrilin 1-specific monoclonal antibody in inhibition ELISAs. In vivo reactivity and specificity were tested by injecting sera into neonatal mice, and antibody binding was detected by immunohistochemical staining. RESULTS: Serum antibodies from RP patients bound tracheolaryngeal and nasal cartilage in vivo and inhibited the binding of anti-matrilin 1-specific monoclonal antibodies. Thirteen of the 97 RP patients had increased titers of matrilin 1 antibody. Positive titers correlated with respiratory symptoms in 69% of the cases. Significant responses to type II collagen and COMP were also detected. CONCLUSION: Antibodies to matrilin 1 bind tracheolaryngeal cartilage in vivo and are correlated with an inflammatory attack on tracheolaryngeal cartilage that is often seen in RP.

Adult↗

PubMed2004

Relapsing polychondritis.

SUMMARY: Relapsing polychondritis is a unique, rare autoimmune disorder in which the cartilaginous tissues are the primary targets of destruction but the immune damage can spread to involve noncartilaginous tissues like the kidney, blood vessels, and so forth. The manifestations of the disease can take many different forms and the pathogenesis is still unclear. It may occur in a primary form or it may be associated with other disease states. This article summarizes important aspects of the disease with a focus on recent information regarding clinical manifestations, disease associations, pathogenesis, and advances in therapeutics.

Antirheumatic Agents↗

PubMed2000

Relapsing polychondritis.

BACKGROUND: Relapsing polychondritis (RPC) is a rare, chronic, and potentially fatal multisystemic inflammatory disorder targeting cartilaginous structures. This disorder is frequently associated with rheumatoid arthritis, systemic vasculitis, connective tissue diseases, and/or hematologic disorders. RPC afflicts patients with recurrent and often progressive episodes of inflammation with the potential for destruction of the affected structures. Tissues involved include the ears, joints, nose, larynx, trachea, eyes, heart valves, kidneys,and skin. Ocular manifestations commonly include episcleritis, scleritis, conjunctivitis, iridocyclitis, chorioretinitis, and proptosis. Lid edema, orbital inflammation, muscle palsies, and corneal melting may also occur. CASE REPORT: An 83-year-old man previously diagnosed with RPC presented to our clinic with acute unilateral chemosis, conjunctivitis, lid edema, proptosis, and extraocular muscle restriction. After orbital cellulitis was ruled out, further evaluation revealed posterior scleritis with choroidal detachment OS. A course of oral indomethacin and topical antibiotic-steroid combination drops was implemented in the treatment of the ocular manifestations. The quick positive response to the anti-inflammatory agents confirmed the diagnosis of ocular complications secondary to RPC. DISCUSSION: The presenting ocular signs and symptoms of RPC often resemble other commonly encountered ocular conditions. It is important for the eye care practitioner to be familiar with the ocular manifestations of RPC because the eyes are sometimes the initial site of involvement and may be a marker of severity. Early diagnosis and intervention may significantly improve the patient's outcome. This case report with literature review will hopefully bring to light features of this disease which will help the eye care practitioner in the diagnosis and management of this condition.

Aged↗

PubMed2003

[Treatment of relapsing polychondritis].

INTRODUCTION: Relapsing polychondritis (RP) manifests as an inflammation of cartilaginous structures throughout the body. Since RP affects cartilage tissues and proteoglycan-rich structures, symptoms appear in the ear, nose, eye, joints, and respiratory system. Otolaryngologists are frequently involved in RP's initial diagnosis, since it is likely to affect the head and neck regions. The etiology of RP remains unknown and it is relatively uncommon, so appropriate treatment remains to be determined. We studied the clinical course and treatment response. METHODS: We studied clinical courses in 12 cases of RP treated at our hospital in the 11 years from 1991 to 2001. RESULTS: In 11 of the 12 cases, conventional steroid therapy was implemented. Five required steroid pulse therapy, and 7 immunosuppressive drugs to control the disease. Strong initial therapy such as steroid pulse therapy plus immunosuppressive drugs suppressed inflammation and decreased its relapse in severe RP patients. One patient could be controlled only with nonsteroidal antiinflammatory drugs. Side effects such as cataracts and diabetes from steroid therapy or pancytopenia from Dapson were found in some patients. All 12 are alive at present. Six were subsequently released from steroid therapy. Anti-type II collagen antibody was not useful as an indicator for treatment but helpful as one of a complementary factors for diagnosis. CONCLUSION: Manifestations of RP are sometimes self-limited, but in most cases, inflammation is recurrent, gradually worsening and finally becoming life-threatening. It is difficult to determine the severity of RP and choose appropriate treatment in its initial stage. Because severe RP cannot be controlled with conventional steroid therapy, strong therapy is indicated in initial treatment if patients have serious symptoms.

Adolescent↗

PubMed2004

Critical role of the major histocompatibility complex and IL-10 in matrilin-1-induced relapsing polychondritis in mice.

Relapsing polychondritis (RP) is an autoimmune disease that affects extra-articular cartilage. Matrilin-1-induced relapsing polychondritis (MIRP) is a model for RP and is useful for studies of the pathogenic mechanisms in this disease. There are indications that the major histocompatibility complex (MHC) class II plays a major role in RP, since DR4+ patients are more commonly affected than controls. We have now addressed the role of the MHC region, as well as the non-MHC contribution, using congenic mouse strains. Of the MHC congenic strains, B10.Q (H2q) was the most susceptible, the B10.P (H2p) and B10.R (H2r) strains developed mild disease, while B10 strains carrying the v, b, f, or u H2 haplotypes were resistant. A slight variation of susceptibility of H2q strains (B10.Q> C3H.Q> DBA/1) was observed and the (B10.Q x DBA/1)F1 was the most susceptible of all strains. Furthermore, macrophages and CD4+ T cells were the most prominent cell types in inflammatory infiltrates of the tracheal cartilage. Macrophages are the major source of many cytokines, such as interleukin-10 (IL-10), which is currently being tested as a therapeutic agent in several autoimmune diseases. We therefore investigated B10.Q mice devoid of IL-10 through gene deletion and found that they developed a significantly more severe disease, with an earlier onset, than their heterozygous littermates. In conclusion, MHC genes, as well as non-MHC genes, are important for MIRP induction, and IL-10 plays a major suppressive role in cartilage inflammation of the respiratory tract.

Animals↗

PubMed2007

Relapsing polychondritis and malignant lymphoma: is polychondritis paraneoplastic?

BACKGROUND: Relapsing polychondritis (RP) is associated with other rheumatic or autoimmune disease in about 30% of cases; however, an association with malignancy is rare with the exception of myelodysplastic syndrome (MDS). Observation Herein we report the first case, to our knowledge, of RP following splenic non-Hodgkin lymphoma (NHL), and we have reviewed all the previous well-documented reports that described the cases of RP associated with malignant lymphoma (ML). CONCLUSIONS: Our case and the review of reported cases showed that RP preceded ML in 2 cases, RP occurred after diagnosis and treatment of ML in 2 cases, and RP and ML occurred simultaneously in 1 case. The types of ML encountered were Hodgkin lymphoma, orbital mucosa associated lymphoid tissue type lymphoma, nodal NHL, and splenic NHL. From the frequent association of RP with MDS and, less frequently, with ML, we speculate that some RP cases may occur as a paraneoplastic condition of the concurrent hematological malignancies.

Diagnosis, Differential↗

PubMed2006

Meningeal plasma cell granuloma with relapsing polychondritis. Case report.

Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent inflammation of the cartilaginous structures and connective tissue. Central nervous system lesions in association with RP have occasionally been reported, but intracranial mass lesions have not been described. The authors report the first such case, in which a 51-year-old man presented with parasagittal meningeal plasma cell granuloma with RP. The mass was subtotally resected and adjuvant radiotherapy was administered. The patient did not experience any recurrence of the lesion during an 8-year follow-up period. In this case, the exact diagnosis of RP was made based on symptoms of respiratory tract chondritis, which was successfully treated by the placement of tracheobronchial stents.

Granuloma, Plasma Cell↗

PubMedDate not supplied

Successful treatment of relapsing polychondritis with infliximab.

Relapsing polychondritis (RP) is a rare and potentially fatal autoimmune disease in which an inappropriate immune response destroys the cartilage of the ears, larynx and nose. Many therapeutic approaches have been reported. We describe the results obtained with infliximab in a patient with RP unresponsive to conventional therapy. This therapy could be a new weapon to treat refractory RP.

Adult↗

PubMedDate not supplied

Cardiac manifestations of relapsing polychondritis--a case report.

Relapsing polychondritis is a systemic autoimmune disease that may be associated with inflammatory arthritis, vasculitis, aortitis, and inflammation of the aortic valve and ring. Information describing the cardiac manifestations of relapsing polychondritis is limited. The authors encountered a patient with relapsing polychondritis who had pericardial effusion and aortic valve regurgitation. The patient's history is reported and relevant literatures are reviewed.

Anti-Inflammatory Agents↗

PubMed2000

[Various clinical patterns of relapsing polychondritis in six cases].

Relapsing polychondritis is a relatively rare disease characterized by episodic inflammation and progressive destruction of cartilage involving ears, nasal and laryngotracheal cartilage, cardiovascular system and the eyes. The increasing awareness of its clinically distinct has resulted in recognition of at least 550 reported cases. Six cases are reported to demonstrate the wide variety of clinical pattern. The most common features of the disease are auricular and nasal cartilage inflammation and nondeforming arthritis. Ocular symptoms and vasculitis is relatively rare. Two cases of relapsing polychondritis with laryngotracheobronchial manifestations illustrate the severe clinical features of the disease. Relapsing polychondritis may associate with diverse forms of connective tissue disease, such as rheumatoid arthritis. It seems interesting to note the onset in childhood. Treatment has been primarily symptomatic. In situations of mild symptoms, initial treatment is with nonsteroidal antiinflammatory drugs. For cases with serious manifestation, corticosteroids and immunosuppressants are indicated.

Adult↗

PubMed1996

[Relapsing polychondritis with atypical presentation].

Relapsing polychondritis is an episodic systemic disorder, characterized by recurrent inflammation that affects cartilaginous structures, the cardiovascular system, eyes and ears. Although the etiology is unknown, experimental evidence strongly suggest that immunologically mediated mechanisms are implicated. Auricular, articular and nasal manifestations are the most frequent disturbances. In about 30% of cases, relapsing polychondritis is associated with other connective tissue diseases and vasculitis. Actually, the diagnosis is based on the criteria proposed by McAdam, and modified by Damiani. We present a case, whose first manifestations of relapsing polychondritis were an intermittent fever and transient arthralgias. Eight months later, auricular chondritis appeared, and gave us the key to diagnosis.

Aged↗

PubMed2001

Anesthetic management of a patient with relapsing polychondritis--a case report.

Relapsing polychondritis is a rare multisystem disorder of uncertain etiology. It is characterized by recurrent and progressive destruction of both elastic and hyaline cartilages as well as connective tissue. Respiratory tract involvement is associated with high mortality and morbidity. General anesthesia may be required in these patients for tracheostomy, bronchoscopy, nasal reconstruction, aortic valve replacement, and recent tracheobronchial stenting which they usually sustain. Tracheostomy was once the most likely surgical procedure in relapsing polychondritis. However, this procedure is only effective in patients with upper subglottic involvement. In cases of extensive tracheobronchial involvement, tracheostomy is ineffective because the distant tracheal collapse below the tracheostomy is still unresolved. We would like to report a case of relapsing polychondritis with tracheobronchial involvement, who underwent an emergent tracheostomy. Tracheostomy with continuous positive airway pressure (CPAP) effectively improved her airway collapse. Herein, we also discuss the anesthetic management after review of the current literature.

Anesthesia↗

PubMed1998

[Three cases of relapsing polychondritis--triggering factor and treatment of relapses].

We present three cases of relapsing polychondritis (RP), all of which were confirmed by biopsy. In the first case, where the symptoms appeared after treatment for kidney and lung cancers, it was thought that interferon alpha, radiation or photodynamic therapy had been the causative or triggering factor. In the second case, eye surgery may have triggered the recurrence of RP. The possibility that medical intervention might cause, promote or trigger RP by disturbing the immune system was discussed. In the third case, where the chief complaint was dyspnea due to severe bronchial constriction, the importance of assessing the state of the respiratory tract, for instance by spiral 3 D CT imaging of the chest, was suggested. The appropriate use of tracheobronchial stents for some cases associated with dyspnea was also discussed.

Aged↗

PubMed1988

Idiopathic CSF pleocytosis in relapsing polychondritis.

The association of relapsing polychondritis with CSF pleocytosis is reported for the first time. Three cases are described in which infectious etiologies of the pleocytosis were excluded by appropriate cultures and serologic studies. We suggest that the finding of CSF pleocytosis in relapsing polychondritis does not merit empiric antimicrobial therapy in the absence of demonstrated infection.

Adult↗

PubMedDate not supplied

Wegener's granulomatosis and relapsing polychondritis: a case report.

Relapsing polychondritis, a rare disorder characterized by inflammation of cartilaginous tissue, is often associated with vasculitic features. Wegener's granulomatosis is an uncommon form of vasculitis, usually responsive to treatment with cyclophosphamide. A 59-yr-old man with relapsing polychondritis, initially well controlled by corticosteroid therapy, developed pulmonary infiltrates and glomerulonephritis. After pathological confirmation of Wegener's granulomatosis, cyclophosphamide therapy was instituted and remission achieved.

Cyclophosphamide↗

PubMed1990

[Relapsing polychondritis. Apropos a case].

Relapsing polychondritis is a rare disease of undetermined cause. The most frequently seen symptom is redness and swelling of cartilaginous ear, followed by cartilage inflammation elsewhere in the body and inflammation of special sense organs. This report describes recurrent attacks of relapsing polychondritis in one patient, who responded to low doses of corticosteroid therapy. The great relevance of cutaneous vasculitis in our patient, lead us to suggest that PR may fall in to the spectrum of systemic vasculitis.

Aged↗

PubMed1988

Increased cartilagenous uptake on bone scintigraphy in a patient with relapsing polychondritis.

A patient with relapsing polychondritis and increased uptake on bone scintigraphy (using 99mTc-MDP) in the cartilagenous parts of the ribs, the sternum and in the larynx region is described. A biopsy of a costochondral junction (where uptake on the bone scan was increased) was compatible with relapsing polychondritis. After treatment with prednisolone and azathioprine the patient improved and a repeated bone scan (after six years) showed less intensive uptake.

Cartilage↗

PubMed1976

Multiple aortic aneurysms in relapsing polychondritis.

A patient with relapsing polychondritis and thoracic and abdominal aortic aneurysms is described. The aortic changes were due to aortitis, which primarily involved the media, with increased vascularization, perviascular infiltration of mononuclear cells, increased amounts of collagen and decreased amounts of elastic tissue and sulfated acid mucopolysaccharides. Aortic aneurysms frequently occur in relapsing polychondritis; they are usually in the ascending aorta but may be multiple and involve the abdominal aorta; involvement of the ascending aorta results in aortic regurgitation and left ventricular failure, and involvement of the abdominal aorta may be clinically silent and result in fatal rupture.

Adult↗