eMedicine Specialties > Ophthalmology > Unclassified Disorders

Sarcoidosis: Treatment & Medication

Author: Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Coauthor(s): L Raymond DeBarge, MD, Assistant Professor, Department of Ophthalmology, University of Tennessee College of Medicine at Chattanooga
Contributor Information and Disclosures

Updated: Dec 5, 2008

Treatment

Medical Care

The elimination of symptoms is the chief goal in treating sarcoidosis. Asymptomatic cases need no treatment. Expectant observation is prudent before initiating therapy. Reserve treatment for documented disease progression.

Approximately 75% of patients with sarcoidosis will have mild, stable disease or even remission, requiring no therapy. Approximately 25% of patients with sarcoidosis require treatment, including 10% of patients who have extrathoracic indications in critical organs (eg, eye, brain, heart) and 15% of patients who have the indication of progressive pulmonary disease.

Few firm guidelines exist for the treatment of systemic disease. Oral corticosteroids are indicated as the first-line therapy for severe pulmonary disease (eg, symptomatic stage II, progressive stage II, stage III) and systemic disease (eg, cardiac manifestations, neurosarcoid, malignant hypercalcemia).

  • Corticosteroids are the mainstay of therapy for extraorbital sarcoid. The current corticosteroid protocol for systemic disease is 30-40 mg prednisone daily for 8-12 weeks. Gradually taper the dose to 10-20 mg every other day over a period of 6-12 months to establish the minimal effective dose. An alternate protocol is prednisone 1 mg/kg of body weight for 4-6 weeks, followed by slow taper over 2-3 months. Repeat if disease becomes clinically active. Most patients who require longer term steroids can be managed using 10-15 mg of prednisone every other day.
  • Noncorticosteroid drugs are being used more frequently, including anti-inflammatory drugs (NSAIDs); antimalarial agents, such as hydroxychloroquine; and immunosuppressant agents, such as methotrexate, cyclosporine, and azathioprine. Infliximab and thalidomide have also been successfully used in refractory sarcoidosis. Cutaneous sarcoidosis has been successfully treated with tetracyclines.
  • Treatment of neurosarcoid includes medical therapy and radiation.
  • Medical treatment of ocular involvement
    • Corticosteroids are the mainstay of treatment. Topical corticosteroids, depot periocular injections, and oral corticosteroids may be used. Anterior uveitis responds well to topical corticosteroids and cycloplegia. Intermediate and posterior uveitis due to inadequate penetration of topical corticosteroids into the posterior segment responds well to depot (sub-Tenon) injections and/or oral corticosteroids. Factors favoring the oral route of administration for uveitis include optic nerve involvement; intermediate, posterior, or panuveitis; bilateral disease; and coexisting threshold systemic disease.
    • Topical corticosteroids are the treatment of choice for anterior uveitis. Pred Forte 1% (prednisolone acetate) is the criterion standard; generic prednisolone acetate is less effective. Prednisolone phosphate is effective but less potent than prednisolone acetate. Newer agents, such as loteprednol etabonate (Lotemax), may be effective second choices, with less chance of steroid-induced glaucoma. They are particularly useful for maintenance. Weaker steroids, such as rimexolone (Vexol) and fluorometholone (FML), may play a role for patients in remission.
    • No specific treatment guidelines are available, but the following treatment guideline for prednisolone acetate 1% is provided as a practical guideline for acute anterior uveitis. Similar topical corticosteroid regimens have been based in uveitis treatment protocols.
      • 1 drop every 1-2 hours while awake for 1 week
      • 1 drop 4 times daily for 1 week
      • 1 drop 3 times daily for 1 week
      • 1 drop 2 times daily for 1 week
      • 1 drop daily for 1 week
      • Discontinue if anterior chamber cells and flare are eliminated.
    • Reevaluate the inflammation and intraocular pressures at 1- to 2-week intervals for the first month after onset of treatment. If inflammation is not improving at any given taper interval, maintain that dosing and taper only after the cells and flare decrease.
    • Cycloplegia is indicated with acute intense inflammation to relieve ciliary spasm and to prevent formation of posterior synechiae.
    • Topical nonsteroidal anti-inflammatory agents may be used when there is evidence for or concern about steroid glaucoma or the possibility of cataract acceleration as a result of long-term topical steroid use.
    • Intermediate and posterior uveitis usually responds well to depot corticosteroid injections. Topical corticosteroids and cycloplegics may supplement oral and/or sub-Tenon injections. Injections may be repeated at weekly, biweekly, or monthly intervals, up to 3-4 times, before maximal benefit is reached. Oral prednisone treatment guidelines similar to the above systemic guidelines may be used.
    • Orbital sarcoidosis usually requires oral corticosteroids, but retrobulbar injections of corticosteroids may be helpful.
  • Corticosteroid-sparing agents
    • Cyclosporine A: In cases of steroid failures or intolerance, cyclosporine A has been used with mixed results.
    • Methotrexate has been shown to be effective in recalcitrant uveitis.
  • Oral monoclonal antibody therapy is being investigated in the treatment of otherwise nonresponsive uveitis. Interferon therapy is likewise being studied. 
  • Anti-tumor necrosis factor-alpha (TNF-alpha) therapy (ie, infliximab, etanercept, adalimumab) has been used in recalcitrant sarcoid uveitis when no response had been obtained with classical immunosuppressive therapies. If uveitis-associated choroidal/retinal neovascularization is present, intravitreal vascular endothelial growth factor (VEGF) agents, such as bevacizumab, may be helpful.

Surgical Care

  • Surgical treatment of neurosarcoid: Surgery is indicated for hydrocephalus, expanding mass lesions, or lesions that cause increased intracranial pressure.
  • Surgical treatment of ocular involvement: Cataract surgery may be performed when the intraocular inflammation is absolutely controlled. Vitrectomy may be required for severe vitreous opacification. If secondary glaucoma is unresponsive to maximal medical therapy, patients may require either trabeculectomy or glaucoma drainage device implant procedures.

Consultations

  • Consultation with a pulmonologist may be helpful in cases of ophthalmic or neurosarcoid to aid in the diagnosis (eg, bronchoscopy with biopsy) and to coordinate therapy.
  • Neurosurgical consultation may be warranted in cases of hydrocephalus that are resistant to medical therapy.

Medication

Corticosteroids remain the drug of choice for severe systemic disease, neurologic manifestations, and intraocular inflammatory disease.

In recalcitrant cases or in those intolerant of corticosteroids, steroid-sparing drugs, such as MTX, and antimalarials have been used with varying success.

Uveitis may be treated with topical ophthalmic corticosteroid preparations and/or periocular corticosteroid injections. Cycloplegia is optimized with atropine or homatropine.

Corticosteroids

Used for systemic, neurologic, and severe ocular inflammation. Shown to be teratogenic in many species but no adequate and well-controlled studies in pregnant women (steroid-dependent asthmatic women have delivered normal, average weight, full-term infants).

Fluid and electrolyte disturbances include sodium retention, fluid retention, congestive heart failure in susceptible patients, potassium loss, hypokalemic alkalosis, and hypertension. Musculoskeletal associations include muscle weakness, steroid myopathy, loss of muscle mass, osteoporosis, compression fractures, osteonecrosis, and pathologic fractures. GI associations include peptic ulceration and bleeding, pancreatitis, and abdominal distention. Dermatologic associations include impaired wound healing, fragile skin, petechiae, ecchymoses, facial erythema, and increased sweating.

Metabolic associations include negative nitrogen balance due to protein catabolism. Neurologic associations include convulsions, pseudotumor cerebri, vertigo, and headache. Endocrine associations include menstrual irregularities, cushingoid state, secondary adrenocortical and pituitary unresponsiveness, growth retardation in children, decreased carbohydrate tolerance, and latent diabetes mellitus. Ophthalmic associations include cataracts, glaucoma, and exophthalmos.

Frequency of adverse effects is as follows: Weight gain of >20 lb 24%, diabetes mellitus (most common in patients with significant liver disease) 8%, aseptic necrosis 2%, aseptic ulcer 2%, and cataract 1%.

Osteoporosis is a well-recognized problem with chronic corticosteroid usage; calcium supplements and alendronate may be required to prevent fractures.


Prednisone (Deltasone, Orasone, Meticorten)

Synthetic adrenocortical steroid with predominantly glucocorticoid properties. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Adult

30-40 mg PO qd for 8-12 wk; gradually taper dose to 10-20 mg qod over a period of 6-12 mo to establish minimal effective dose
Alternatively, 1 mg/kg PO for 4-6 wk, follow by slow taper over 2-3 mo; repeat if disease becomes clinically active

Pediatric

1-2 mg/kg/d PO for severe vision-threatening uveitis or neurosarcoidosis

Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use


Prednisolone acetate (Pred Forte)

Decreases inflammation and corneal neovascularization. Suppresses migration of polymorphonuclear leukocytes and reverses increased capillary permeability.
In cases of bacterial infections, concomitant use of anti-infective agents is mandatory; if signs and symptoms do not improve after 2 days, reevaluate patient. Dosing may be reduced, but advise patients not to discontinue therapy prematurely.

Adult

1 gtt q1-2h while awake for 1 wk
1 gtt qid for 1 wk
1 gtt tid for 1 wk
1 gtt bid for 1 wk
1 gtt qd for 1 wk
Discontinue if anterior chamber cells and flare are eliminated

Pediatric

Not established; in standard practice, uveitis regimens similar to adult dosages have been used safely

Documented hypersensitivity; acute untreated purulent ocular conditions; acute superficial herpes simplex dendritic keratitis; vaccinia, varicella, and most other viral corneal infections; ocular tuberculosis; fungal diseases of the eye

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Ophthalmic adverse reactions include cataracts, increased intraocular pressure and glaucoma, secondary ocular infections from fungi or viruses, perforation of globe with scleral or corneal thinning; systemic adverse effects rarely may occur with extensive use of topical steroids


Rimexolone (Vexol)

Decreases inflammation and corneal neovascularization. Suppresses migration of polymorphonuclear leukocytes and reverses increased capillary permeability.

Adult

Anterior uveitis: 1-2 gtt into conjunctival sac of affected eye
Week 1: 1 gtt q1h during waking hours
Week 2: 1 gtt q2h during waking hours
Week 3 and on: Taper until uveitis is resolved
See acute uveitis taper regimen in Medical Care

Pediatric

Not established; in standard practice, uveitis regimens similar to adult dosages have been used safely

Documented hypersensitivity; acute untreated purulent ocular conditions; acute superficial herpes simplex dendritic keratitis; vaccinia, varicella, and most other viral corneal infections; ocular tuberculosis; fungal diseases of the eye

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Corneal fungal infections are particularly prone to develop coincidentally with long-term local steroid application; ophthalmic adverse reactions include cataracts, increased intraocular pressure and glaucoma, secondary ocular infections from fungi or viruses, perforation of the globe with scleral or corneal thinning; systemic adverse effects rarely may occur with extensive use of topical corticosteroids


Loteprednol etabonate 0.5% suspension (Lotemax)

A sterile topical anti-inflammatory corticosteroid for ophthalmic use. Indicated for the treatment of steroid-responsive inflammatory conditions of the palpebral and bulbar conjunctiva, cornea, and anterior segment of the globe.

Adult

Up to 1 gtt q1h during first wk of initial treatment

Pediatric

Not established; in standard practice, uveitis regimens similar to adult dosages have been used safely

Documented hypersensitivity; acute untreated purulent ocular conditions; acute superficial herpes simplex (dendritic keratitis); vaccinia, varicella, and most other viral corneal infections; ocular tuberculosis; fungal diseases of the eye

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Corneal fungal infections are particularly prone to develop coincidentally with long-term local corticosteroid use; ophthalmic adverse reactions include cataracts, increased intraocular pressure and glaucoma, secondary ocular infections from fungi or viruses, perforation of the globe with scleral or corneal thinning; systemic adverse effects rarely may occur with extensive use of topical corticosteroids

Mydriatic and cycloplegic agents

Used to induce cycloplegia for pain control and prevention of posterior synechiae in uveitis.


Atropine sulfate (Isopto, Atropair, Atropisol)

Acts at parasympathetic sites in smooth muscle to block response of sphincter muscle of iris and muscle of ciliary body to acetylcholine, causing mydriasis and cycloplegia.

Adult

1-2 gtt in the eye(s) tid

Pediatric

Not established; use with extreme caution

Documented hypersensitivity; excessive use in children or in certain susceptible patients, including with spastic paralysis, glaucoma or a predisposition to narrow-angle glaucoma, brain damage, Down syndrome, or in individuals with a prior history of susceptibility to belladonna alkaloids, may produce systemic symptoms of atropine poisoning (if this occurs, discontinue medication and use appropriate therapy); should not be used in children who previously have had a severe systemic reaction to atropine

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Lacrimal sac compression by digital pressure for 2-3 min after instillation may avoid systemic absorption; to avoid inducing angle-closure glaucoma, an estimation of anterior chamber depth should be made; administration of atropine in infants requires great caution
General signs and symptoms of atropine toxicity include flushing and dryness of the mouth and skin, blurring of vision, fever, tachycardia, mental aberration including irritability or delirium, and loss of neuromuscular coordination; children and infants may develop a rash or abdominal distention; atropine poisoning rarely is fatal and generally is self-limited if medication is discontinued; if accidentally swallowed, physostigmine salicylate may be administered parenterally to provide prompt relief of the intoxication
Ophthalmic adverse reactions include allergic lid reactions, local irritation, hyperemia, edema, follicular conjunctivitis, or dermatitis


Homatropine 2% or 5% solution (AK-Homatropine, Isopto Homatropine)

Sterile topical anticholinergic agent for ophthalmic use. Parasympatholytic drug quite similar to atropine but weaker and with a shorter duration of action.

Adult

1-2 gtt in the eyes tid

Pediatric

Not established; use with extreme caution

Documented hypersensitivity; glaucoma or a predisposition to narrow-angle glaucoma

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Lacrimal sac compression by digital pressure for 2-3 min after instillation may avoid systemic absorption; to avoid inducing angle-closure glaucoma, an estimation of anterior chamber depth should be made; ophthalmic adverse reactions include allergic lid reactions, local irritation, hyperemia, edema, follicular conjunctivitis, or dermatitis

Antineoplastics/antimetabolites

Several agents have been used for severe systemic, ocular, and neurosarcoid unresponsive to corticosteroids. Based on safety and efficacy, MTX and azathioprine are the preferred agents for most patients.


Methotrexate (Folex PFS)

An antimetabolite used to treat certain neoplastic, rheumatologic, and ocular disease. Inhibits enzyme dihydrofolate reductase, ultimately interfering with DNA metabolism, repair, and cellular replication. Appears to have minimal to no carcinogenicity.
Therapeutic response usually begins in 3-6 wk, with potential for continued improvement for another 12 wk or more. Once therapeutic response achieved, dosage should be reduced gradually to lowest possible effective dose. Optimal duration of therapy is not known. Once discontinued, rheumatoid arthritis usually worsens within 3-6 wk.

Adult

7.5 mg PO single dose per wk (adjust gradually to optimal response); not to exceed 20 mg/wk

Pediatric

Not established; use with caution

Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX
Probenecid, NSAIDs, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, can increase MTX plasma levels; may decrease phenytoin plasma levels; may increase plasma levels of thiopurines

Documented hypersensitivity; diarrhea, vomiting, or stomatitis

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Potential for serious toxicity, with toxicity related to dose and frequency of administration; toxic effects can occur at any time, so patients need to be monitored closely; most adverse reactions, if detected early, are reversible; when such reactions occur, dose should be reduced or eliminated; postponing pregnancy for at least 6 mo after cessation of MTX usually is sufficient to avoid teratogenetic effects
May cause anemia, thrombocytopenia, and/or leukopenia; can cause acute and/or chronic hepatotoxicity; caution in hepatic impairment or preexisting liver damage (perform liver function tests at baseline and at 4- to 8-wk intervals)
Usually contraindicated in immunodeficiency states and active infections; leukoencephalopathy has been reported in association with craniospinal irradiation; may induce pulmonary disease with symptoms of fever, cough, and dyspnea; may cause acute renal failure in severe volume depletion and renal impairment

Cytotoxic agents

Inhibit cell growth and proliferation.


Azathioprine (Imuran)

Antimetabolite that interferes with DNA synthesis and inhibits lymphocyte proliferation.

Adult

50-200 mg/d (1-2 mg/kg/d) PO

Pediatric

Not established

Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of MTX metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine

Documented hypersensitivity; low levels of serum thiopurine methyl transferase (TPMT); hepatitis; cytopenias; infections

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Monitoring should include blood count, liver function tests; avoid during infections; all of the cytotoxic agents are teratogenic, and all patients of reproductive age (both men and women) should take birth control while on therapy


Cyclosporine (Sandimmune, Neoral)

Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease for a variety of organs.
For children and adults, base dosing on ideal body weight.

Adult

2-5 mg/kg/d PO bid in divided doses

Pediatric

Not established

Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease cyclosporine concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase cyclosporine toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin

Documented hypersensitivity; uncontrolled hypertension or malignancies; do not administer concomitantly with PUVA or UVB radiation in psoriasis since it may increase risk of cancer; renal impairment; severe hypertension; infections

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Monitoring should include complete blood count, serum creatinine, urinalysis, blood pressure; advise patients about adequate fluid intake and diuresis; avoid infections
Newer formulations (Neoral) are absorbed much better than traditional preparation (Sandimmune) necessitating dosage adjustment and repeat serum levels


Cyclophosphamide (Neosar, Cytoxan)

Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Adult

1-3 mg/kg/d IV qmo

Pediatric

Not established

Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity

Documented hypersensitivity; severely depressed bone marrow function

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Monitoring should include blood count, liver function tests, and urinalysis; advise patients about adequate fluid intake and diuresis and sperm bank storage; avoid infections
Cyclophosphamide has been associated with early menopause and aspermia; all of the cytotoxic agents are teratogenic, and all patients of reproductive age (both men and women) should take birth control while on therapy

Antimalarials

Used for their anti-inflammatory properties.


Hydroxychloroquine sulfate (Plaquenil)

Antimalaria drug highly active against erythrocytic forms of Plasmodium vivax and Plasmodium malariae and most strains of Plasmodium falciparum. Anti-inflammatory effects with rheumatologic disorders but the precise mechanism of action is unknown.

Adult

200-400 mg PO qd

Pediatric

Not recommended

Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption

Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Dermatologic reactions may occur; may cause chloroquine retinopathy; if long-term therapy is anticipated, initial and periodic ophthalmologic examinations are needed; recommended testing includes visual acuity, slit lamp evaluation, visual field testing, and macular investigation for bull's eye macular changes; stop treatment immediately if toxicity is suspected

Nonsteroidal anti-inflammatory drugs

NSAIDs may be used for erythema nodosum and arthritis but have had little success in treating serious ocular inflammatory disease or neurosarcoidosis.


Indomethacin (Indocin)

Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation; inhibits prostaglandin synthesis.

Adult

Moderate-to-severe rheumatoid arthritis dosing: 25 mg PO bid/tid; if tolerated, may increase 25-50 mg if required for continuing symptoms at weekly intervals until symptoms relieved or until total daily dose of 150-200 mg reached; higher dosages do not generally increase effectiveness

Pediatric

<14 years: Not recommended
>14 years: Administer as in adults

Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of MTX toxicity; phenytoin levels may be increased when administered concurrently

Documented hypersensitivity; GI bleeding or renal insufficiency

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May mask signs of infection, cause fluid retention, exacerbate congestive heart failure, inhibit platelet aggregation, and elevate liver enzymes; should be discontinued at least 2 wk prior to elective surgery to avoid hemorrhagic complications

More on Sarcoidosis

Overview: Sarcoidosis
Differential Diagnoses & Workup: Sarcoidosis
Treatment & Medication: Sarcoidosis
Follow-up: Sarcoidosis
References
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References

  1. Crystal RG. Sarcoidosis. In: Fauci AS, Isselbacher KJ, Braunwald E, et al, eds. Harrison's Principles of Internal Medicine. 14th ed. McGraw-Hill; 1998:1922-8.

  2. Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med. Apr 24 1997;336(17):1224-34. [Medline].

  3. Rybicki BA, Major M, Popovich J Jr, et al. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol. Feb 1 1997;145(3):234-41. [Medline].

  4. Bresnitz EA, Strom BL. Epidemiology of sarcoidosis. Epidemiol Rev. 1983;5:124-56. [Medline].

  5. Chapelon C, Ziza JM, Piette JC, et al. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine (Baltimore). Sep 1990;69(5):261-76. [Medline].

  6. Altan-Yaycioglu R, Akova YA, Akca S, et al. Inflammation of the posterior uvea: findings on fundus fluorescein and indocyanine green angiography. Ocul Immunol Inflamm. Jun 2006;14(3):171-9. [Medline].

  7. Baarsma GS, La Hey E, Glasius E, et al. The predictive value of serum angiotensin converting enzyme and lysozyme levels in the diagnosis of ocular sarcoidosis. Am J Ophthalmol. Sep 15 1987;104(3):211-7. [Medline].

  8. Baughman RP, Judson MA, Costabel U. Randomized, double-blind, placebo-controlled trial of infliximab in patients with chronic pulmonary sarcoidosis. Chest. 2005;128:202S.

  9. Baughman RP, Judson MA, Teirstein A, et al. Presenting characteristics as predictors of duration of treatment in sarcoidosis. QJM. May 2006;99(5):307-15. [Medline].

  10. Burton BJ, Breen RA, Janossy G, et al. Use of pulmonary interferon {gamma} responses to mycobacterial antigen to distinguish sarcoid associated optic neuropathy from tuberculosis. Br J Ophthalmol. Jun 2006;90(6):802-3. [Medline].

  11. Caça I, Unlu K, Buyukbayram H, et al. Conjunctival deposits as the first sign of systemic sarcoidosis in a pediatric patient. Eur J Ophthalmol. Jan-Feb 2006;16(1):168-70. [Medline].

  12. Colover J. Sarcoidosis with involvement of the nervous system. Brain. 1948;71:451-75.

  13. DeBroff BM, Donahue SP. Bilateral optic neuropathy as the initial manifestation of systemic sarcoidosis. Am J Ophthalmol. Jul 15 1993;116(1):108-11. [Medline].

  14. Delaney P. Neurologic manifestations in sarcoidosis: review of the literature, with a report of 23 cases. Ann Intern Med. Sep 1977;87(3):336-45. [Medline].

  15. DeRosa AJ, Margo CE, Orlick ME. Hemorrhagic retinopathy as the presenting manifestation of sarcoidosis. Retina. 1995;15(5):422-7. [Medline].

  16. Dev S, McCallum RM, Jaffe GJ. Methotrexate treatment for sarcoid-associated panuveitis. Ophthalmology. Jan 1999;106(1):111-8. [Medline].

  17. Doty JD, Mazur JE, Judson MA. Treatment of sarcoidosis with infliximab. Chest. Mar 2005;127(3):1064-71. [Medline].

  18. Frohman L, Grigorian R, Slamovits T. Evolution of sarcoid granulomas of the retina. Am J Ophthalmol. May 2001;131(5):661-2. [Medline].

  19. GoulD H, Kaufman HE. Sarcoid of the fundus. Arch Ophthalmol. Mar 1961;65:453-6. [Medline].

  20. Henke CE, Henke G, Elveback LR, et al. The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival. Am J Epidemiol. May 1986;123(5):840-5. [Medline].

  21. Honeybourne D. Ethnic differences in the clinical features of sarcoidosis in South-East London. Br J Dis Chest. Jan 1980;74(1):63-9. [Medline].

  22. Hunninghake GW, Costabel U, Ando M, et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis Vasc Diffuse Lung Dis. Sep 1999;16(2):149-73. [Medline].

  23. Ingestad R, Stigmar G. Sarcoidosis with ocular and hypothalamic-pituitary manifestations. Acta Ophthalmol (Copenh). 1971;49(1):1-10. [Medline].

  24. James DG, Neville E, Langley DA. Ocular sarcoidosis. Trans Ophthalmol Soc U K. Apr 1976;96(1):133-9. [Medline].

  25. Johns CJ, Michele TM. The clinical management of sarcoidosis. A 50-year experience at the Johns Hopkins Hospital. Medicine (Baltimore). Mar 1999;78(2):65-111. [Medline].

  26. Jones NP. Sarcoidosis. Curr Opin Ophthalmol. Dec 2002;13(6):393-6. [Medline].

  27. Kiryu J, Kita M, Tanabe T, et al. Pars plana vitrectomy for cystoid macular edema secondary to sarcoid uveitis. Ophthalmology. Jun 2001;108(6):1140-4. [Medline].

  28. Kurup S, Lew J, Byrnes G, et al. Therapeutic efficacy of intravitreal bevacizumab on posterior uveitis complicated by neovascularization. Acta Ophthalmol. May 28 2008;[Medline].

  29. Lama P, Frohman L. Annual review of systemic disease--1997--II. J Neuroophthalmol. Jun 1998;18(2):127-42. [Medline].

  30. Laval J. Ocular sarcoidosis. Am J Ophthalmol. Apr 1952;35(4):551-4. [Medline].

  31. Levinsky L, Cummiskey J, Romer FK, et al. Sarcoidosis in Europe: a cooperative study. Ann N Y Acad Sci. 1976;278:335-46. [Medline].

  32. Lewis SJ, Ainslie GM, Bateman ED. Efficacy of azathioprine as second-line treatment in pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. Mar 1999;16(1):87-92. [Medline].

  33. Lucas DR. Tumours of the orbit. In: Greer's Ocular Pathology. 4th ed. Blackwell Publishers: 1989:243.

  34. Mayers M. Ocular sarcoidosis. Int Ophthalmol Clin. Fall 1990;30(4):257-63. [Medline].

  35. Nosal A, Schleissner LA, Mishkin FS, et al. Angiotensin-I-converting enzyme and gallium scan in noninvasive evaluation of sarcoidosis. Ann Intern Med. Mar 1979;90(3):328-31. [Medline].

  36. Nussenblatt RB, Whitcup SM, Palestine AG. Uveitis: Fundamentals and Clinical Practice. 2nd ed. Mosby-Year Book; 1996.

  37. Obenauf CD, Shaw HE, Sydnor CF, et al. Sarcoidosis and its ophthalmic manifestations. Am J Ophthalmol. Nov 1978;86(5):648-55. [Medline].

  38. Oksanen V. Neurosarcoidosis: clinical presentations and course in 50 patients. Acta Neurol Scand. Mar 1986;73(3):283-90. [Medline].

  39. Petropoulos IK, Vaudaux JD, Guex-Crosier Y. Anti-TNF-alpha therapy in patients with chronic non-infectious uveitis: the experience of Jules Gonin Eye Hospital. Klin Monatsbl Augenheilkd. May 2008;225(5):457-61. [Medline].

  40. Power WJ, Neves RA, Rodriguez A, et al. The value of combined serum angiotensin-converting enzyme and gallium scan in diagnosing ocular sarcoidosis. Ophthalmology. Dec 1995;102(12):2007-11. [Medline].

  41. Prabhakaran VC, Saeed P, Esmaeli B, et al. Orbital and adnexal sarcoidosis. Arch Ophthalmol. Dec 2007;125(12):1657-62. [Medline].

  42. Reich JM. How common is sarcoidosis?. Sarcoidosis Vasc Diffuse Lung Dis. Mar 1999;16(1):108. [Medline].

  43. Roy FH. Ocular Differential Diagnosis. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2002.

  44. Sheppard JD, Nozik RA. Practical diagnostic approach to uveitis. In: Duane TA, Jaeger EW, eds. Clinical Ophthalmology. Vol 4. Philadelphia: JB Lippincott; 1989.

  45. Sheppard JD. Posterior uveitis. In: Nozik RA, Michaelson JB, eds. Ophthalmology Clinics of North America. Vol 6. WB Saunders Co; 1993.

  46. Shetty AK, Zganjar BE, Ellis GS Jr, et al. Low-dose methotrexate in the treatment of severe juvenile rheumatoid arthritis and sarcoid iritis. J Pediatr Ophthalmol Strabismus. May-Jun 1999;36(3):125-8. [Medline].

  47. Siltzbach LE, James DG, Neville E, et al. Course and prognosis of sarcoidosis around the world. Am J Med. Dec 1974;57(6):847-52. [Medline].

  48. Smith JA, Foster CS. Sarcoidosis and its ocular manifestations. Int Ophthalmol Clin. Winter 1996;36(1):109-25. [Medline].

  49. Spalton DJ, Sanders MD. Fundus changes in histologically confirmed sarcoidosis. Br J Ophthalmol. May 1981;65(5):348-58. [Medline].

  50. Stern BJ, Krumholz A, Johns C, et al. Sarcoidosis and its neurological manifestations. Arch Neurol. Sep 1985;42(9):909-17. [Medline].

  51. Vinas FC, Rengachary S. Diagnosis and management of neurosarcoidosis. J Clin Neurosci. Nov 2001;8(6):505-13. [Medline].

  52. Wertheim MS, Mathers WD, Suhler EB, et al. Histopathological features of conjunctival sarcoid nodules using noninvasive in vivo confocal microscopy. Arch Ophthalmol. Feb 2005;123(2):274-6. [Medline].

  53. Yamamoto M, Sharma OP, Hosoda Y. Special Report. The 1991 Descriptive Definition of Sarcoidosis. Sarcoidosis. 1992;9(Suppl1):33-4.

  54. Yee AM, Pochapin MB. Treatment of complicated sarcoidosis with infliximab anti-tumor necrosis factor-alpha therapy. Ann Intern Med. Jul 3 2001;135(1):27-31. [Medline].

  55. Zajicek JP, Scolding NJ, Foster O, et al. Central nervous system sarcoidosis--diagnosis and management. QJM. Feb 1999;92(2):103-17. [Medline].

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Further Reading

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Keywords

sarcoidosis, ocular sarcoidosis, sarcoid, ocular sarcoid, neurosarcoid, neurosarcoidosis, granulomatous disease, noncaseating epithelioid granuloma, granulomata, Boeck disease, Boeck’s disease, Boeck's sarcoid, Hutchinson disease, Hutchinson’s disease

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Contributor Information and Disclosures

Author

Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Coauthor(s)

L Raymond DeBarge, MD, Assistant Professor, Department of Ophthalmology, University of Tennessee College of Medicine at Chattanooga
L Raymond DeBarge, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Physicians, American Medical Association, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Medical Editor

John D Sheppard Jr, MD, MMSc, Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Program Director, Ophthalmology Residency Training, Eastern Virginia Medical School; President, Virginia Eye Consultants
John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

R Christopher Walton, MD, Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, Assistant Dean for Graduate Medical Education, University of Tennessee College of Medicine; Consulting Staff, Regional Medical Center, Memphis Veterans Affairs Medical Center, St Jude Children's Research Hospital
R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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