Cutaneous manifestations of patients with relapsing polychondritis: an association with extracutaneous complications

Clinical Rheumatology - The aim of this study was to determine the prognostic characteristics of patients with relapsing polychondritis (RP) accompanying cutaneous manifestations in Japan. We...     

Vasculitis and relapsing polychondritis

Relapsing polychondritis is a systemic disease of unknown etiology with predominate manifestations of multiorgan cartilaginous inflammation. Although relapsing polychondritis occurs predominantly as a separately defined clinical complex, a significant number of patients may suffer from another rheumatic disease. Vasculitic syndromes are the most commonly observed disorders associated with relapsing polychondritis.     

Relapsing polychondritis, interstitial granulomatous dermatitis and antiphospholipid syndrome: an unusual clinical association

The authors describe the case of a 49 year-old male patient with a 3-year history of antiphospholipid syndrome, admitted after presenting in the emergency room with erythematous nodular skin lesions, affecting the face and neck, with a week's duration. Local biopsies were suggestive of interstitial granulomatous dermatitis. The patient described lesions compatible with bilateral auricular chondritis, two weeks prior to the appearance of the nodules, which resolved spontaneously after 3 days. There was a previous episode of nasal chondritis, two years previously, and another episode starting at the 7th day of hospitalization. These findings, taken together with a diagnosis of seronegative polyarthritis established 5 years before the current events, lead to a diagnosis of relapsing polychondritis.     

Hodgkin's disease presenting as relapsing polychondritis a previously undescribed association

Relapsing polychondritis is a rare syndrome thought to be autoimmune in nature and characterized by inflammation and degeneration of cartilage. The association with a reticuloendothelial malignancy has not previously been reported. A 46-year-old man presented typical clinical manifestations of relapsing polychondritis requiring 30 mg of prednisone daily for suppression. However within 6 months he developed chronic fatigue, fevers, night sweats, adenopathy, and splenomegaly leading to the diagnosis of Hodgkin's disease.     

The arthropathy of relapsing polychondritis

Twenty-three adults (11 males and 12 females) with well-defined relapsing polychondritis (RP) are studied in order to characterize the arthropathy of RP. Arthritis was found in 19 patients-as the presenting feature in 8 and as a significant symptom in 11 others. The usual pattern of involvement was mitratory, asymmetric, nonnodular, nonerosive, and seronegative, and affected large and small joints as well as parasternal articulations. In addition RP was seen in 3 patients with preexisting chronic polyarthritis or associated rheumatic disease.     

Tracheobronchial involvement in relapsing polychondritis

Relapsing polychondritis (RPC) is a multisystem disorder of chondromalacia involving any cartilage. Respiratory tract involvement is the greatest threat to life. We report a patient with stenosis of the subglottic trachea and left main bronchus who suddenly ceased breathing. As this patient did not have any other clinical features of RPC, the diagnosis was difficult. CT showed circumferential worm-eaten-like thickening suggesting a deformity and edema of the tracheal mucosa. Biopsy of the tracheal and thyroid cartilage revealed mild cartilage degeneration and infiltration with inflammatory cells. Therefore, the patient was diagnosed as having RPC. She is currently well 24 months after Montgomery T tube intubation with systemic steroids. Narrowing of the left main bronchus has not worsened.     

Cardiac involvement in relapsing polychondritis

Relapsing polychondritis is a rare disease characterized by recurrent inflammatory lesions involving cartilaginous structures, the eyes, the ears and the cardiovascular system. Valvar insufficiency represents the most common of the cardiac abnormalities and appears in 8 percent of patients. We describe two patients having relapsing polychondritis with cardiac involvement. One of them required valvar substitution and died 6 months after surgery.     

Cardiovascular involvement in relapsing polychondritis

Relapsing polychondritis is an inflammatory disease that characteristically involves cartilagenous tissues. Cardiovascular involvement is a fairly common complication and the second most frequent cause of mortality in this disease. The case of a man with a progressive cardiac involvement, aortic incompetence, mitral regurgitation, and finally complete atrioventricular block offered the opportunity of reviewing the cardiovascular complications in relapsing polychondritis. The most frequent abnormalities are aortic regurgitation and aortic aneurysm. Furthermore, several cases of atrioventricular block, mitral regurgitation, and acute pericarditis have been reported. For early diagnosis and treatment of these severe complications, periodic cardiovascular examination is mandatory in these patients.     

Facial diplegia revealing relapsing polychondritis

SIR, Relapsing polychondritis (RP) is a systemic disorder, whichis characterized by recurrent inflammation and destruction ofcartilage structure [1, 2]. Auricular, nasal, ocular, tracheobronchialand articular impairment are well-recognized manifestationsof RP [1, 2]. Neurological involvement is considered to be rarein RP, and usually occurs during the course of the disease [1–7].We recently observed a case which is of particular interest,since the patient developed facial diplegia revealing RP. A 63-yr-old woman presented with     

Renal involvement in relapsing polychondritis

Twenty-nine of the 129 patients with RP seen at the Mayo Clinic between 1943 and 1984 had renal involvement. These patients were older, had arthritis and extrarenal vasculitis more frequently, and had a significantly worse survival rate than those without renal involvement. Renal biopsies were obtained in 11 of these 29 patients. The predominant lesions were mild mesangial expansion and cell proliferation, and segmental necrotizing glomerulonephritis with crescents. Small amounts of electron-dense deposits, predominantly mesangial, were noted on electron microscopy. Immunofluorescence revealed faint deposition of C3 and/or IgG or IgM, predominantly in the mesangium. Autopsies were obtained in 13 of the 47 patients who had died. Information regarding the renal pathology was available in 10 of these 13 autopsies. At the time of the initial evaluation at the Mayo Clinic, 6 of these 10 patients had evidence of renal involvement. At autopsy, none of these 10 patients had evidence of active renal vasculitis or segmental necrotizing glomerulonephritis, but 8 of the 10 patients exhibited variable degrees of vascular and glomerular sclerosis, segmental mesangial proliferation, tubular loss, and interstitial lymphocytic infiltrates. These observations expand the limited information available in the literature, which is based on 11 previously published case reports of renal involvement in RP. In only a few of our patients and previously reported patients were the manifestations of the disease limited to the systems characteristically involved in pure RP. The frequent coexistence of other autoimmune and connective tissue diseases supports the role of immune mechanisms in the pathogenesis of this syndrome. Deposition of immune complexes is likely to play a role in the pathogenesis of the glomerular lesions associated with RP. Administration of corticosteroids alone is sufficient to induce a complete remission in some cases, while in others the addition of a cytotoxic agent is necessary to control the activity of the disease or to spare corticosteroid side effects and maintain a remission. Immunosuppression-related infectious complications and undetected relapses after discontinuation of immunosuppressive therapy are largely responsible for the morbidity and mortality observed in these patients.     

Urticarial vasculitis revealing relapsing polychondritis

We report a case of hypocomplementemic urticarial vasculitis heralding a relapsing polychondritis in a 63-year-old woman. The patient, who had suffered in the past from polymyalgia rheumatica, suddenly experienced a generalized urticarial eruption with a dramatic decrease in C4 complement fraction and the presence of anti-C1q antibodies. Two months later, an ear chondritis occurred and the patient rapidly responded to steroids and dapsone. To our knowledge, this association has never been reported.