Sarcoidosis with Ocular Manifestations
This presentation of conjunctivitis with calcified granulomas on chest X-ray is highly suggestive of sarcoidosis, and you should immediately obtain serum ACE levels, serum lysozyme, and consider chest CT or gallium scintigraphy to confirm systemic involvement. 1
Diagnostic Approach
Mandatory Initial Workup
- Chest imaging interpretation: Calcified granulomas on chest X-ray indicate previous granulomatous disease, with sarcoidosis being the leading differential diagnosis in a patient presenting with conjunctivitis 1
- Serum markers: Obtain serum angiotensin-converting enzyme (ACE) levels, serum lysozyme, and liver function tests, as these are frequently elevated in sarcoidosis 1
- Advanced imaging: Chest CT scan and/or gallium scintigraphy should be performed to better characterize the extent of pulmonary involvement and aid in confirming sarcoidosis 1
Ophthalmic Evaluation
- Slit-lamp biomicroscopy: Document the pattern of conjunctival inflammation, presence of nodules, and assess for concurrent anterior chamber inflammation or uveitis 2
- Visual acuity measurement: Essential to establish baseline and detect any vision-threatening complications 2
- Corneal assessment: Evaluate for keratitis, peripheral ulcerative keratitis, or other corneal involvement that would indicate more severe disease 2
Critical Differential Diagnoses to Exclude
Rule out vasculitis-associated conditions first, as these may indicate life-threatening systemic disease requiring urgent immunosuppression, particularly with bilateral involvement 2:
- ANCA testing: Obtain c-ANCA to evaluate for granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) 1, 2
- Infectious etiologies: Consider serologies for Bartonella henselae (cat-scratch disease) and Francisella tularensis if there is Parinaud's oculoglandular syndrome with preauricular lymphadenopathy 1, 3
- Malignancy: Sebaceous carcinoma can mimic granulomatous conjunctivitis, especially with unilateral presentation and history of recurrent chalazia 2
Treatment Algorithm
Before Initiating Immunosuppression
You must rule out infectious causes before starting any immunosuppressive therapy, as this is critical to prevent catastrophic outcomes 2:
- Perform conjunctival biopsy if the diagnosis remains uncertain or if infectious granulomatous disease (tuberculosis, fungal) needs exclusion 4
- Review tuberculosis screening (interferon-gamma release assay or PPD) given the calcified granulomas on chest imaging 1
Topical Management for Conjunctivitis
- Initial therapy: Start topical corticosteroids for symptomatic relief of conjunctival inflammation 5
- Monitoring: Follow intraocular pressure closely when using topical steroids, as prolonged use increases glaucoma risk 5
- Adjunctive therapy: Consider topical antibiotics like gatifloxacin if secondary bacterial infection is suspected, though this is not the primary pathology 6
Systemic Treatment Considerations
Coordinate with rheumatology or pulmonology for systemic management 2:
- Systemic corticosteroids are first-line for symptomatic sarcoidosis with ocular involvement
- Steroid-sparing agents (methotrexate, azathioprine, mycophenolate mofetil) may be needed for chronic disease 2
- Biologics (anti-TNF agents) are reserved for refractory cases 2
Red Flags Requiring Urgent Ophthalmology Referral
Immediate referral is mandatory for 5, 2:
- Visual loss or decreased vision
- Moderate to severe eye pain
- Corneal involvement (ulceration, infiltrate, perforation risk)
- Necrotizing scleritis
- Signs of systemic vasculitis (renal failure, pulmonary hemorrhage, neurologic symptoms)
- Lack of response to initial therapy within 7-10 days 5
Common Pitfalls to Avoid
- Do not assume all calcified granulomas are benign: While calcified lesions suggest lower risk for active tuberculosis progression, sarcoidosis remains active and requires treatment 1
- Do not start immunosuppression empirically: Always exclude infection first, particularly tuberculosis and fungal disease, given the chest findings 2
- Do not ignore bilateral presentation: This significantly increases the likelihood of systemic vasculitis requiring urgent workup 2
- Do not delay systemic workup: Conjunctivitis may be the initial presentation of serious systemic disease, and early detection is critical for preventing morbidity 1