Which autoimmune disorders are associated with relapsing polychondritis (RP)?

Updated: Dec 06, 2018
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

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

Ulcerative colitis

3 (2%) of 159

McAdam et al [16]

2 (3%) of 62

Zeuner et al [17]

5 (2%) of 221

Total

Crohn disease

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

Takayasu arteritis

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

Reactive arthritis/psoriatic arthritis

2 (1%) of 159

McAdam et al [16]

1 (< 1%) of 112

Michet et al [18]

3 (1%) of 271

Total

Systemic sclerosis

2 (1%) of 159

McAdam et al [16]

Raynaud syndrome

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]

Pernicious anemia

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]

Lichen planus

2 (1%) of 180

Piette et al [21]

Primary biliary cirrhosis

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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