Systemic Diseases Associated with Scleritis
What systemic autoimmune/autoinflammatory diseases are associated with scleritis?
Association in up to 50% of patients (see the following table). RA is the most common systemic disease, usually occurring in the setting of severely disabling and well-established disease. Joint inflammation may be “burnt out” and patients often have other extraarticular manifestations (e.g., systemic vasculitis). When associated with peripheral ulcerative keratitis, RA, GPA, relapsing polychondritis, and SLE are most common. Necrotizing scleritis without inflammation (scleromalacia perforans) is seen almost exclusively in RA.
| Systemic Diseases Associated with Scleritis | |
|---|---|
| Associated Condition | Cases of Scleritis (%) |
| RA | 10.3–18.6 |
| GPA | 3.8–8.1 |
| Relapsing polychondritis | 1.6–6.4 |
| SLE | 1.0–4.1 |
| IBD | 2.1–4.1 |
| Seronegative spondyloarthropathies | 0.3–3.5 |
| Other a | ≤1% |
a Other: Behçet’s, Takayasu’s, GCA, hypocomplementemic urticarial vasculitis, cutaneous vasculitis, hepatitis C virus-associated vasculitis, undifferentiated connective tissue disease, polymyalgia rheumatica, pyoderma gangrenosum, sarcoidosis, and Cogan’s syndrome.
