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PubMed2002

Relapsing polychondritis: a clinical review.

OBJECTIVE: This study comprehensively reviews the literature related to relapsing polychondritis (RP). METHODS: A detailed search via MEDLINE (PubMed) was performed using relapsing polychondritis as the key term. Relevant articles were analyzed with a focus on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP. RESULTS: RP is a rare episodic and progressive inflammatory disease of presumed autoimmune etiology first described in 1923. RP affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. The diagnosis of RP is based on the presence of clinical criteria. A standardized therapeutic protocol for RP has not been established. Nonsteroidal anti-inflammatory drugs, dapsone and/or colchicine, may control disease activity in some patients. In other patients, immunosuppressive drugs and prednisone have been effective. RP is a potentially lethal disease; pulmonary infection, systemic vasculitis, airway collapse, and renal failure are the most common causes of death. Earlier studies indicate survival rates between 70% at 4 years and 55% at 10 years. In a recent study, a survival rate of 94% at 8 years may be due to improved medical and surgical management. CONCLUSIONS: RP is a rare, multisystemic, and potentially fatal disease. The pathogenesis and optimal therapeutic approach to patients with RP is poorly understood.

Cartilage↗

PubMed1980

Relapsing polychondritis. A feature of systemic lupus erythematosus.

In summary, we have presented two well documented cases of SLE with relapsing polychondritis. The chondritis was not a presenting feature and had no obvious prognostic significance. Neither patient has developed any further manifestations of relapsing polychondritis. A local immune complex etiology may be suggested by the laboratory findings. Treatment with corticosteroids was associated with the resolution of inflammation within a few days. Relapsing polychondritis should be recognized as an uncommon feature of SLE.

Adult↗

PubMed1993

Relapsing polychondritis, another cause for a "salmon patch".

We present the case of a patient with long-standing relapsing polychondritis and, first, an orbital mass and, then, a "salmon patch" conjunctival mass. The histologic pathologic findings were similar on both occasions, showing an inflammatory process with reactive lymphoid hyperplasia. In both situations, the masses responded to a short course of systemic corticosteroids. Although ocular inflammatory changes from relapsing polychondritis have been well described, to our knowledge, there have been no previous reports of conjunctival changes in the form of a salmon patch lesion, as described here. Relapsing polychondritis may be added to the differential diagnosis of a conjunctival salmon patch lesion.

Conjunctival Diseases↗

PubMed2006

Relapsing polychondritis: prevalence of expiratory CT airway abnormalities.

PURPOSE: To retrospectively determine the prevalence of expiratory computed tomographic (CT) abnormalities, including malacia and air trapping, in patients with relapsing polychondritis and to retrospectively determine the frequency with which expiratory abnormalities are accompanied by inspiratory abnormalities on CT scans. MATERIALS AND METHODS: Institutional review board approval was obtained, and informed consent was not required for this retrospective HIPAA-compliant study. A computerized hospital information system was used to identify all patients with clinically diagnosed or biopsy-proved relapsing polychondritis who were referred for CT airway imaging during a 17-month period. The study cohort comprised 18 patients (15 women, three men; mean age, 47 years; age range, 20-71 years). Multidetector helical CT was performed in all patients by using a standard protocol, which included end-inspiratory and dynamic expiratory volumetric imaging. Two observers who were blinded to the original scan interpretations simultaneously reviewed CT scans. Findings were recorded in consensus. Dynamic expiratory CT scans were assessed for malacia that involved the trachea and main bronchi (reduction in cross-sectional area of more than 50%) and for air trapping (failure of lung parenchyma to increase in attenuation during expiration). Air trapping was visually classified according to pattern and extent (lobular, segmental, lobar, or whole lung). Inspiratory CT scans were evaluated for tracheal and bronchial stenosis (>25% luminal diameter narrowing compared with a corresponding uninvolved segment), wall thickening (>2 mm), and calcification. RESULTS: Expiratory CT abnormalities were present in 17 (94%) of 18 patients and included malacia in 13 patients (72%) and air trapping in 17 patients (94%). Inspiratory CT abnormalities were found in eight (47%) of 17 patients who had expiratory CT abnormalities. Calcification of the airway walls was present in seven (39%) of 18 patients. All patients who had inspiratory CT abnormalities demonstrated expiratory CT abnormalities. CONCLUSION: Expiratory CT abnormalities were present in the majority of patients with relapsing polychondritis who were referred for airway imaging, yet only half of these patients demonstrated abnormalities on routine inspiratory CT scans. Thus, dynamic expiratory CT should be a standard component of imaging assessment in patients with relapsing polychondritis.

Adult↗

PubMedDate not supplied

[Relapsing polychondritis. A case report of a patient treated with methotrexate and celecoxib].

Relapsing polychondritis is a rare illness in which the cartilaginous tissues such as auricles, nose, laryngotracheal structures, joints and others, are affected. Customary treatment is based on corticosteroids and traditional antiinflammatory agents including aspirin and indomethacin. We describe a case of relapsing polychondritis in an 82-year-old man with associated diabetes mellitus and special features treated successfully with alternative therapy based on methotrexate and celecoxib.

Aged↗

PubMed1996

Relapsing polychondritis presented as inner ear involvement.

We report a rare case of relapsing polychondritis with an initial symptom of inner ear involvement. This 53-year-old Japanese man experienced a hearing difficulty, tinnitus in both ears, and dizziness of sudden onset, but lacked auricular chondritis at that time, which is the most frequent finding in relapsing polychondritis. Thus it was difficult to reach a correct diagnosis. Steroid therapy, with oral prednisolone 15 mg daily, was effective. Almost two months after we began the steroid therapy, the patient complained of losing interest in his work and reported a hallucination vision on the TV screen, so the dose of prednisolone was decreased to 10 mg. The hallucinations then disappeared, but the serum level of C-reactive protein increased highly. To reduce the dose of prednisolone, we tried low-dose oral methotrexate. However, we had to discontinue it when the patient experienced severe vomiting and diarrhoea. As adjuvant therapy, we then administered Sho-saiko-to, Chinese herbal medicines with few side effects. Symptoms and laboratory abnormalities then improved markedly.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed1997

A case of relapsing polychondritis presenting as mediastinal syndrome, diagnosed by CT scans of the trachea and head.

OBJECTIVE: The aim of this study is to evaluate the significance of CT scans of the trachea and head in the diagnosis of Relapsing Polychondritis (RP). DESIGN: Relapsing polychondritis is a disease involving cartilaginous structures, particularly those of the ears, nose and trachea. Diagnosis is based on specific clinical features and immuno-histopathological evaluation of the cartilages involved. SETTING AND PATIENTS: We describe a case of RP in which the most evident clinical signs (cough, dyspnoea, vertigo, tinnitus, headache, oedema of the face and shoulders and fever), led us first to suspect a mediastinal compression syndrome. INTERVENTION: A CT scan of the trachea and head revealed details which established the correct diagnosis, supported by other typical RP symptoms and by histopathological examination of the cartilage. MAIN OUTCOME MEASURES: Evaluation by CT scan of the chest, the mediastinum, the head and the pinnae. RESULTS: CT scanning revealed thickening and calcification of the anterolateral tracheal wall and main bronchi besides marked narrowing of the trachea. CT of the head showed calcification also of the external auditory meatus and part of the pinnae. CONCLUSION: We consider that CT scan of the trachea and head is helpful in evaluating the bronchial tree, the auditory meatus and pinnae as well as being a valid tool for the final diagnosis and in following the course of the disease.

Diagnosis, Differential↗

PubMedDate not supplied

Vasculitis with mesangial IgA deposits complicating relapsing polychondritis.

The authors report the case of a patient presenting with cutaneous, renal and neurologic vasculitis in the course of relapsing polychondritis (RPC). A 78-year-old man presented with a palpable purpura of the lower limbs, high fever arthralgias, delirium, and nephrotic syndrome. He had a history of relapsing polychondritis treated by corticosteroids. Renal biopsy showed diffuse endo- and extracapillary proliferative glomerulonephritis with mesangial IgA deposits. A spectacular regression of the symptoms was observed in response to pulse intravenous methylprednisolone. Relapsing polychondritis is complicated by vasculitis in 25% of the cases. This vasculitis is characterized by cutaneous, neurologic and renal manifestations, usually occurring in elderly patients. Renal involvement is characterized by segmental and focal or diffuse necrotizing glomerulonephritis. The mesangial IgA deposits observed in our patient are rarely present in the course of RPC. Renal manifestations identify severe forms of RPC, justifying systematic screening for renal complications.

Aged↗

PubMed1979

Relapsing polychondritis in a Latin American man.

The first known reported case of relapsing polychondritis in a Latin American man is presented. The 35-year-old man, demonstrating auricular chondritis, arthritis, nasal cartilage involvement, episcleritis and respiratory tract chondritis, was admitted to the hospital and treated with 60 mg of prednisone daily, prednisolone acetate 1% ophthalmic drops every waking hour and homatropine hydrobromide 5% ophthalmic drops twice daily. After discharge from the hospital, he received 30 to 60 mg of prednisone daily and prednisolone acetate 1% ophthalmic drops twic daily for about 18 months. A permanent trachestomy was placed, azathioprine, 150 mg daily, was given and prednisone dosage was tapered to 25 mg daily when the patient subsequently was hospitalized for colapsed airway and Cushing's syndrome. The classic symptoms, pathogenesis and treatment of the disease are reviewed. Systemic corticosteroids are the drugs of choice in relapsing polychondritis, with immunosuppressive drugs, azathioprine in particular, being used as adjuvants to lower steroid dosage requirements and to achieve greater control of symptoms in patients with the severe progressive disease. Treatment of the disease with dapsone alone is promising and may offer an alternative to high-dose steroids and immunosuppressive therapy.

Adrenal Cortex Hormones↗

PubMed2000

Relapsing polychondritis: a course over 20 years with cerebral involvement.

Relapsing polychondritis is a chronic inflammatory disease associated with an autoimmune disorder in cartilaginous tissue, eyes, labyrinth, blood vessels, and central nervous system. We describe a 75-year-old woman who presented with a 20-year history of dyspnea, inspiratory stridor, and polyarthritis. She developed dysmorphism of both ears and a saddle nose approximately 10 years earlier. Subsequently, she suffered from hearing loss and a tremor. A T2-weighted magnetic resonance imaging scan of the brain revealed multiple, spotted signal intensities. Immunohistochemical analysis of a serum sample showed antibodies to cartilaginous tissue, which were further identified on immunoblotting as antibodies to type II collagen. The extremely prolonged course of disease (>20 years) until a correct diagnosis was made is remarkable. Also, cerebral involvement, which was most likely caused by cerebral angiitis, and which, to our knowledge, has never previously been reported in this form, was detected. Arch Otolaryngol Head Neck Surg. 2000;126:1495-1498

Aged↗

PubMed1992

Corneal autoimmunity in a patient with relapsing polychondritis.

We report our experiences with a patient with relapsing polychondritis, in whom circulating antibodies against corneal epithelium were determined by immunofluorescence before and after medical treatment. The results provide further evidence for an autoimmune pathogenesis of the disease.

Audiometry, Pure-Tone↗

PubMed1997

Relapsing polychondritis with Castleman-like lymphadenopathy: a case report.

Relapsing Polychondritis (RP) is a systemic disorder characterized by an inflammatory process involving predominantly cartilaginous structures and the cardiovascular system. Lymphadenopathy is a very uncommon finding of RP. We report on a patient affected by RP presenting with lymphadenopathy of Castleman-like type quickly responsive to corticosteroids. The bronchial involvement and the evolution of the inflammatory process in a 3-year follow-up has been documented by computed tomography of the chest.

Adrenal Cortex Hormones↗

PubMed1991

Airway complications in relapsing polychondritis.

To analyze the importance of airway involvement in relapsing polychondritis, an illustrative case report is presented and 62 patients reported in the literature with serious airway complications are reviewed. There were 47 female and 17 male patients, with an average age of 40.3 years (range, 2 to 73 years). Patients were seen with hoarseness, breathlessness, cough, stridor, wheezes, and tenderness over laryngotracheal cartilages. Respiratory tract involvement was confirmed by conventional radiography, tomography, computed tomography, dynamic pulmonary function tests, and bronchoscopy. Corticosteroids and antiinflammatory and immunosuppressive agents were used in these patients. Tracheostomy was performed in 18 patients. Death occurred in 13 patients despite tracheostomy or corticosteroid therapy, or both. A detailed analysis of the clinical, radiological, and pulmonary function studies is presented, with emphasis on upper airway mechanics. The medical and surgical management options are reviewed, including the use of endotracheal prosthesis and extraluminal splinting in dynamic airway collapse.

Adolescent↗

PubMedDate not supplied

Anesthesia in a patient with acute respiratory insufficiency due to relapsing polychondritis.

We report on a male patient with advanced relapsing polychondritis (RP), who was hospitalized for an ophthalmologic intervention. He had to undergo a tracheostomy with general anesthesia due to respiratory decompensation. RP is a rare systemic, inflammatory, and destructive disease of the cartilaginous structures leading to multiple functional disorders in the affected organs. Involvement of the tracheobronchial tract may cause severe respiratory problems and even life-threatening respiratory insufficiency. Treatment consists of corticoids, chemotherapy, antirheumatics, and surgical intervention. Anesthesiologic management requires careful preoperative evaluation of vital organ functions, in particular respiration, so that the anesthetic approach can be tailored to the individual needs of each patient.

Acute Disease↗

PubMed1984

Relapsing polychondritis. Pathological and immunological findings in an autopsy case.

Relapsing polychondritis (RP) is a disorder of unknown etiology characterized by the destruction of cartilage. A case of RP in a 59-year-old male was autopsied, and systemic inflammation of various cartilages was confirmed. We demonstrated the circulating antibodies to Type II (cartilage) collagen. No antibodies to other collagen types were demonstrated. The presence of granular deposits of immunoglobulins, fibrinogen, and the C3 component of complement at the chondrofibrous junction was also demonstrated. From these findings, this case suggested that the pathogenesis of RP is related to an immune mechanism.

Adult↗

PubMed1988

Dermatitis herpetiformis associated with relapsing polychondritis.

A 35-year-old woman developed typical relapsing polychondritis within a year following the onset of dermatitis herpetiformis. This occurred during a reduction in the dapsone dose regimen. To our knowledge, the coexistence of these two conditions in a single patient has not yet been reported in the literature. Both the parallel course and the good response to dapsone suggest a pathogenic link between these two diseases.

Adult↗

PubMed1995

Optic neuropathy, headache, and diplopia with MRI suggestive of cerebral arteritis in relapsing polychondritis.

The pathogenesis of central nervous system disease in relapsing polychondritis (RPC) is unknown but may be related to cerebral arteritis. Previous reports have described clinical and histopathologic evidence of cerebral vasculitis in RPC; however, a neuroimaging correlate has not been reported. We present a 36-year-old man with neuro-ophthalmic features of RPC whose magnetic resonance imaging revealed multifocal gray- and white-matter high intensities. This pattern is consistent with cerebral arteritis as described in other systemic vasculitides.

Adult↗

PubMed1977

[Relapsing polychondritis (author's transl)].

Report about a case of relapsing polychondritis in a woman aged 60 years, which was proofed through autopsy. There was involvement of the trachea, the bronchial system and the cartilages of the ribs. The cartilages of nose and ear did not show any typical changes; therefore the diagnosis could not be established during life. The pecularities of the clinical picture and the pathohistological findings are reported in detail. It seems that the polychondritis is related in it's pathogenesis to the rheumatic disorders. tthe etiology, however, remains obscure.

Autopsy↗