| SARCOIDOSIS  
SIGNS AND SYMPTOMS
Sarcoidosis is a systemic granulomatous disease of unknown etiology.
Clinical findings may include a debilitating, febrile illness with cough and dyspnea,
fatigue, bilateral hilar lymphadenopathy (visible upon plain film radiograph), erythema
nodosum, alveolitis, acute polymyositis, arthritis, musculoskeletal anomalies, lacrimal or
salivary gland infiltration or sarcoid nodules of the skin. It occurs most frequently in
young adults (20 to 40 years), has a predilection for women and for races of color.
Patients diagnosed with systemic sarcoidosis have nearly 20 percent
incidence of ocular involvement. The most prevalent ocular sign is unilateral, anterior,
granulomatous uveitis. Less common presentations include unilateral nongranulomatous
uveitis, bilateral intermediate uveitis, and bilateral chronically smoldering low-grade
granulomatous ocular inflammation (Lofgren's syndrome).
The common clinical ocular findings associated with sarcoid uveitis
include decreased or hazy vision, pain, photophobia, lacrimation, conjunctival injection,
cells and flare in the anterior chamber, granulomatous iritis with large "mutton
fat" keratic precipitates scattered over the back surface of the corneal endothelium,
iritis spill over leading to anterior vitritis, true vitritis with white exudative debris
in the region of the ora serrata (snowball or snowbank retinopathy) with retinal
vasculitis (candle wax drippings) and phlebitis (venous sheathing).
Nodules of the iris stroma (Busacca nodules), nodules of the pupillary
border (Koeppe nodules), conjunctival granulomas, band keratopathy, posterior synechiae,
cataract formation, secondary glaucoma, retinal hemorrhage, retinal neovascularization,
cystoid macular edema, venous occlusion, optic disc swelling, optic nerve infiltration,
compressive optic neuropathy, proptosis and extra ocular muscle palsy are all documented
findings.
PATHOPHYSIOLOGY
Studies underscore that lymphocytes interact with macrophages. Some
postulate that CD4+ T-helper 1 cells, in concert with macrophages, produce a cascade of
cytokines and chemotactic factors which result in tissue changes and granulomatous lesions
that affect many tissues and allow for the multi-system, multi-symptom nature of this
disease. The clinical features of sarcoidosis mimic those of rheumatologic diseases, with
increasing reports of coexistent autoimmune disease; however, no one has decisively
determined an association.
MANAGEMENT
Manage the ocular signs and symptoms of sarcoidosis by the findings.
Manage uveitis aggressively with topical cycloplegics (e.g. homatropine 5%, scopolamine
0.25%, or atropine 1%, BID), topical steroids such as Vexol (rimexolone 1%) and Pred Forte
(prednisolone acetate 1%), Q1H to QID if necessary, oral steroidal or nonsteroidal
anti-inflammatories. In recalcitrant cases, you may attempt periocular subtenon steroid
injections of Kenalog 40 (triamcinolone) every three to four weeks. Antimetabolites such
as methotrexate and cyclosporin-A have been used effectively in patients intolerant to
steroids. Add topical aqueous suppressants if intraocular pressure control is required.
Topical nonsteroidal anti-inflammatory agents such as Acular (Ketorolac tromethamine) and
Voltaren (diclofenac sodium), QID, may be attempted if you detect cystoid macular edema.
Allow the retinologist to manage peripheral retinal neovascularization and pars planitis
with panretinal photocoagulation and oral or injected steroids respectively.
The primary care physician can lead systemic management. Referral for
laboratory testing across the autoimmunologic, rheumatologic, infectious and inflammatory
spectrum is essential, especially for atypical uveitis or optic neuropathy. Obtain or
suggest to the PCP testing for anemia, leukemia, syphilis, HIV, lupus, Lyme disease,
tuberculosis, arthritis, ankylosing spondylitis and hypertension. The initial battery
could include complete blood count (CBC with differential), fluorescent treponemal
antibody absorption test (FTA-Abs), reactive plasma reagin (RPR), purified protein
derivative with anergy panel (PPD with anergy panel), anti-nuclear antibody (ANA),
angiotensin converting enzyme (ACE), Lyme titre, rheumatoid factor (RF), sickle prep,
chest x-ray (CXR) and sacroiliac joint films. Tests that indicate sarcoidosis most
specifically are the chest x-ray, ACE and gallium scan.
CLINICAL PEARLS
Diagnose sarcoidosis through clinical (laboratory tests
and biopsy) and radiologic evidence. Up to 90 percent of patients with ocular sarcoid have
abnormal chest radiographs. Lung biopsy by tracheobronchial fiber optic techniques is 90
percent accurate. Biopsy of an enlarged, potentially infiltrated lacrimal gland or
conjunctival granuloma is an acceptable alternative and can be handled by most general
ophthalmologists.
A simple but effective practice management tool: a
dictated letter to the primary care physician explaining the ocular problem, the
management, the request for lab studies and the progress reports.
Vitritis without retinitis, vasculitis or phlebitis
should be considered large reticulum cell sarcoma until proven otherwise. The minimum
initial work up for this form of intermediate uveitis includes, but is not limited to, CBC
with differential, FTA-Abs, RPR, PPD, ANA, ACE, Lyme titre, RF and CXR.
Other reports in this section
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