The hypothesis of an autoimmune etiology for relapsing polychondritis is also supported by the high prevalence of other autoimmune disorders found in patients with relapsing polychondritis. McAdam et al reported that 25%-35% of patients with relapsing polychondritis had a concurrent autoimmune disease. [16]
Table. Autoimmune Conditions Reported in Patients With Relapsing Polychondritis (Open Table in a new window)
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 |
In addition, several reports have linked relapsing polychondritis with internal malignancy. It is thought to be paraneoplastic in these cases. The underlying malignancy is most often hematological in nature, but solid tumors have also been described. [23]
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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.