Medical Care
No controlled trials of therapy for relapsing polychondritis (RP) have been published. The goal of treatment is to abate current symptoms and to preserve the integrity of cartilaginous structures.
Mild cases of RP are usually treated with non-steroidal anti-inflammatory drugs (NSAIDs) or low doses of systemic corticosteroids. Severe RP may require high-dose systemic corticosteroids, perhaps along with disease-modifying antirheumatic drugs (DMARDs) as steroid-sparing agents or for more severe disease. [52]
Prednisone (20-60 mg/d) is administered in the acute phase and is tapered to 5-25 mg/d for maintenance. Severe flares may require 80-100 mg/d. Most patients require a low daily dose of prednisone for maintenance; however, intermittent administration of high doses during only flares of the condition is successful in rare cases. McAdam et al found that continuous prednisone decreased the severity, frequency, and duration of relapses. [16] See the images below.
Bilateral inflammation and structural collapse of the auricles in a patient found to have aortic dissection. Courtesy of the University of Washington, Division of Dermatology.
Same patient as in Image 5 after 4-6 weeks of steroid treatment. Note resolution of auricular inflammation with nodularity and forward listing of the ears. Courtesy of the University of Washington, Division of Dermatology.
Close-up view of same patient as in Image 6. Forward flopping of ear with nodularity after steroid treatment. Courtesy of the University of Washington, Division of Dermatology.
Other medications reported to control symptoms and, perhaps, progression of the disease, include azathioprine, methotrexate (MTX; 7.5-22.5 mg/wk), cyclophosphamide, and cyclosporine. MTX has been dosed beginning at 7.5 mg/wk, increasing up to 22.5 mg/wk in conjunction with steroid administration and has been found to significantly decrease corticosteroid requirements while controlling symptoms. Dapsone (25-200 mg/d) has been beneficial in some patients with mild relapsing polychondritis, although more current clinical experience has found dapsone to be less useful.
Case reports have described successful treatment with the following:
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Leflunomide, which inhibits pyrimidine synthesis [59]
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Rituximab, an anti-CD20 chimeric antibody [60]
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Tocilizumab, a humanized monoclonal antibody against the interleukin-6 receptor (IL-6R) [61]
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Abatacept, a chimeric protein that inhibits T-lymphocyte activation64
In a French multicenter retrospective cohort study that included 41 patients with RP treated with biologics (105 instances; TNF inhibitors, n=60; tocilizumab, n=17; anakinra, n=15; rituximab, n=7; abatacept, n=6), the overall response rate during the first 6 months of treatment was 62.9%; however the complete response rate was 19.0%. Reduction in corticosteroid doses was highly variable. [62]
Differences in clinical response rates varied with organ involvement. There were trends toward a lower response rate in patients with associated myelodysplastic syndrome and a higher response rate for nasal/auricular chondritis, sternal chondritis, and concomitant exposure to non-biologic disease-modifying antirheumatic drugs. [62]
Medical care must include assessment for and treatment of other confounding or concurrent autoimmune disorders.
Surgical Care
Surgical procedures that may be used in the care of patients with relapsing polychondritis include the following:
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Tracheostomy
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Permanent tracheotomy placement
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Tracheal stent placement
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Aortic aneurysm repair
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Cardiac valve replacement
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Saddle-nose deformity repair
Subglottic stenosis can be treated with submucosal corticosteroid injection followed by serial dilation. Wierzbicka et al reported good airway patency for more than 24 months in eight of 12 patients with relapsing polychondritis or other autoimmune disorders treated with this approach. [63]
The benefits of any proposed surgery must be weighed adequately against the patient's risk for infection, especially in the event of acute relapse, since patients are at an increased risk of infection whether or not they are using corticosteroids.
Additionally, patients with relapsing polychondritis and tracheal disease may be at particular risk regarding complications resulting from tracheal intubation and extubation.
Consultations
Relapsing polychondritis is a complex condition that requires a team approach for patient care, as follows:
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Dermatologists or specialists in infectious diseases are often involved early in the course of the disease to evaluate the patient for infectious causes of cellulitis or perichondritis.
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Rheumatologists usually become the primary care provider and should be involved early in patient care.
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Ophthalmologists should also be involved early to diagnose, monitor, and treat the potentially devastating ocular complications.
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Cardiologists, neurologists, nephrologists, and otolaryngologists may be asked to manage other aspects of relapsing polychondritis.
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Plastic surgeons can aid in nasal reconstruction if saddle-nose deformity is present.
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Auricular edema and erythema sparing the lobule. Courtesy of Gregory J. Raugi, MD, PhD.
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Severe auricular edema and inflammation. Courtesy of the University of Washington, Division of Dermatology.
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Forward listing ear. Courtesy of the University of Washington, Division of Dermatology.
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Floppy ear. Courtesy of the University of Washington, Division of Dermatology.
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Bilateral inflammation and structural collapse of the auricles in a patient found to have aortic dissection. Courtesy of the University of Washington, Division of Dermatology.
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Same patient as in Image 5 after 4-6 weeks of steroid treatment. Note resolution of auricular inflammation with nodularity and forward listing of the ears. Courtesy of the University of Washington, Division of Dermatology.
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Close-up view of same patient as in Image 6. Forward flopping of ear with nodularity after steroid treatment. Courtesy of the University of Washington, Division of Dermatology.
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Unilateral episcleritis. Courtesy of Gregory J. Raugi, MD, PhD.
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Saddle-nose deformity. Courtesy of the University of Washington, Division of Dermatology.
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Tracheal stenosis on chest x-ray film. Courtesy of Julie E. Takasugi, MD.
Tables
Disease |
Patients With Condition/Total Patients |
References |
Systemic vasculitis |
3 (5%) of 62 |
Zeuner et al [17] |
11 (10%) of 112 |
Michet et al [18] |
|
8 (12%) of 66 |
Trentham and Le [19] |
|
28 (18%) of 159 |
McAdam et al [16] |
|
50 (13%) of 399 |
Total |
|
Cutaneous leukocytoclastic vasculitis |
2 (33%) of 6 |
Priori et al [20] |
6 (5%) of 112 |
Michet et al [18] |
|
8 (7%) of 118 |
Total |
|
Thyroid disease |
8 (5%) of 159 |
McAdam et al [16] |
10 (15%) of 66 |
Trentham and Le [19] |
|
2 (33%) of 6 |
Priori et al [20] |
|
4 (4%) of 112 |
Michet et al [18] |
|
2 (3%) of 62 |
Zeuner et al [17] |
|
26 (6%) of 405 |
Total |
|
Rheumatoid arthritis* |
8 (5%) of 159 |
McAdam et al [16] |
3 (2%) of 180 |
Piette et al [21] |
|
8 (7%) of 112 |
Michet et al [18] |
|
7 (11%) of 62 |
Zeuner et al [17] |
|
26 (5%) of 513 |
Total |
|
Systemic lupus erythematosus† |
2 (1%) of 159 |
McAdam et al [16] |
9 (5%) of 180 |
Piette et al [21] |
|
1 (17%) of 6 |
Priori et al [20] |
|
6 (5%) of 112 |
Michet et al [18] |
|
3 (5%) of 62 |
Zeuner et al [17] |
|
21 (4%) of 519 |
Total |
|
Sjögren syndrome (possible) |
5 (3%) of 159 |
McAdam et al [16] |
5 (5%) of 111 |
Piette et al [21] |
|
10 (4%) of 270 |
Total |
|
3 (2%) of 159 |
McAdam et al [16] |
|
2 (3%) of 62 |
Zeuner et al [17] |
|
5 (2%) of 221 |
Total |
|
2 (1%) of 180 |
Piette et al [21] |
|
1 (2%) 62 |
Zeuner et al [17] |
|
1 (100%) of 1 |
Haigh et al [22] |
|
4 (2%) of 243 |
Total |
|
Mixed connective-tissue disease |
5 (3%) of 180 |
Piette et al [21] |
2 (2%) of 112 |
Michet et al [18] |
|
7 (2%) of 292 |
Total |
|
3 (2%) of 180 |
Piette et al [21] |
|
Mesenteric panniculitis |
3 (2%) of 180 |
Piette et al [21] |
Spondyloarthropathy |
2 (1%) of 180 |
Piette et al [21] |
3 (3%) of 112 |
Michet et al [18] |
|
2 (3%) of 62 |
Zeuner et al [17] |
|
7 (2%) of 354 |
Total |
|
Diabetes mellitus |
1 (2%) of 62 |
Zeuner et al [17] |
3 (2%) of 159 |
McAdam et al [16] |
|
4 (2%) of 221 |
Total |
|
2 (1%) of 159 |
McAdam et al [16] |
|
1 (< 1%) of 112 |
Michet et al [18] |
|
3 (1%) of 271 |
Total |
|
2 (1%) of 159 |
McAdam et al [16] |
|
2 (1%) of 159 |
McAdam et al [16] |
|
Glomerulonephritis |
2 (1%) of 159 |
McAdam et al [16] |
Dysgammaglobulinemia |
2 (1%)of 159 |
McAdam et al [16] |
1 (1%) of 159 |
McAdam et al [16] |
|
Behçet disease* |
1 (< 1%) of 112 |
Michet et al [18] |
Psoriasis |
2 (1%) of 180 |
Piette et al [21] |
2 (1%) of 180 |
Piette et al [21] |
|
1 (< 1%) of 112 |
Michet et al [18] |
|
*Individual patients may carry more than one autoimmune diagnosis. †Reported as 13 (20%) of 66 prevalence by Trentham and Le without division by disease |
||
