Does Ocular Pemphigoid Cause Elevated ESR?
Ocular cicatricial pemphigoid (ocular mucous membrane pemphigoid) does not typically cause elevated ESR, as it is primarily a localized autoimmune disease affecting the conjunctival basement membrane without significant systemic inflammation. 1
Understanding Ocular Pemphigoid and Inflammatory Markers
Ocular mucous membrane pemphigoid (OMMP) is a progressive, immune-mediated process that specifically targets the conjunctival basement membrane, presenting with nonspecific ocular surface complaints such as redness, foreign body sensation, dryness, tearing, and discharge. 1 The disease is characterized by subepithelial fibrosis with frequent remissions and exacerbations, leading to progressive conjunctival scarring, symblephara, and potentially ankyloblepharon in later stages. 1
Key Clinical Considerations
ESR is not a diagnostic feature of isolated ocular pemphigoid. The disease process is localized to the ocular surface and does not generate the systemic inflammatory response that would elevate acute phase reactants like ESR. 1
When ESR is elevated in patients with ocular pemphigoid, consider associated autoimmune diseases. A recent multicenter study found that 27.3% of ocular cicatricial pemphigoid patients had coexisting autoimmune diseases, with Sjögren's syndrome being the most prevalent. 2 In this context, hypergammaglobulinemia was significantly associated with the presence of both OCP and other autoimmune diseases (OR 8.7; 95%CI 1.6-46.8). 2
ESR elevation should prompt investigation for systemic conditions. In patients with uveitis (a different ocular inflammatory condition), the majority had ESR and CRP within normal limits, but when ESR was ≥60 mm/h and/or CRP ≥60 mg/L, systemic immune-mediated diseases or systemic infectious causes were identified in most cases. 3 This principle applies to ocular pemphigoid patients as well—elevated ESR suggests looking beyond the eye.
Clinical Pitfalls to Avoid
Do not assume elevated ESR is due to ocular pemphigoid itself. If you encounter a patient with ocular pemphigoid and elevated ESR, systematically evaluate for:
Coexisting autoimmune diseases (particularly Sjögren's syndrome, rheumatoid arthritis, or systemic lupus erythematosus), as more than one-quarter of OCP patients have associated autoimmune conditions. 2
Systemic infections or malignancies, as ESR ≥70 mm/h typically indicates significant underlying pathology (infection in 42%, malignant disease in 27%, or other inflammatory conditions in 20% of cases). 4
Giant cell arteritis in elderly patients presenting with new ocular symptoms, as ESR >40 mm/h has 93.2% sensitivity for GCA, and this represents an ophthalmologic emergency requiring immediate corticosteroid therapy. 1, 5
Diagnostic Approach When ESR is Elevated
When encountering elevated ESR in a patient with ocular pemphigoid:
Obtain complete blood count with differential to assess for anemia, leukocytosis, or other hematologic abnormalities that might explain the elevation. 5
Measure C-reactive protein (CRP) concurrently, as it often correlates with ESR but rises and falls more rapidly with changes in inflammation. 5
Screen for extraocular involvement, as 11.5% of ocular pemphigoid patients have extraocular manifestations that might generate systemic inflammation. 2
Consider serum protein electrophoresis if hypergammaglobulinemia is suspected, as this finding strongly suggests coexisting autoimmune disease in OCP patients. 2
The diagnosis of ocular pemphigoid remains one of exclusion, confirmed by conjunctival biopsy for immunopathology (though false negatives are frequent), and elevated ESR should redirect your investigation toward systemic causes rather than being attributed to the ocular disease itself. 1