eMedicine Specialties > Ophthalmology > Unclassified Disorders

Sarcoidosis

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

Introduction

Background

Sarcoidosis is an inflammatory multisystem granulomatous disease of unknown etiology.

Although sarcoidosis predominantly affects the lungs, it often manifests within the eye. In addition to the pulmonary and ocular signs, other organ systems may be affected, including the skin, lymph nodes, liver, spleen, heart, central and peripheral nervous systems, musculoskeletal system, and salivary glands.

Sarcoidosis was first identified over 125 years ago by 2 dermatologists working independently, Dr. Jonathan Hutchinson in England and Dr. Caesar Boeck in Norway, both of whom described its skin findings. Sarcoidosis was originally called Hutchinson disease or Boeck disease. Dr. Boeck went on to fashion today's name for the condition from the Greek words sark and oid, meaning fleshlike. The term describes the skin eruptions that are frequently caused by sarcoidosis.

Sarcoidosis is diagnosed when the classic clinical and radiologic findings are supported by histologic evidence of widespread noncaseating epithelioid granulomata. Although best known for its thoracic involvement, the ocular, neurologic, and extrapulmonary manifestations of sarcoidosis may cause significant complications, including blindness, meningitis, arthritis, renal disease, systemic morbidity, dermatitis, and death.

Pathophysiology

Sarcoidosis is characterized by the formation of noncaseating granulomas in tissues without another known cause for granulomatous disease. Sarcoidosis is ultimately a diagnosis of exclusion. The true causative organism or antigen has not yet been identified, although some form of atypical mycobacteria continues to be suspected.

Clinical disease occurs when granulomata affect the involved tissues as space-occupying lesions, distorting normal architecture and, hence, function. Most frequently, sarcoidosis presents with bilateral hilar lymphadenopathy, pulmonary infiltration, and ocular and skin lesions. Other organs involved include the liver, spleen, lymph nodes, salivary glands, joints, heart, nervous system, muscle, and bones. Remote effects of sarcoidosis also may be seen with overproduction of 1,25-dihydroxyvitamin D, which may cause hypercalcemia and hypercalciuria. Clinical manifestations depend on ethnicity, chronicity of illness, site and extent of tissue involvement, and activity of the granulomatous process.

Extrathoracic manifestations involving the liver, skin, heart, and/or eye are the presenting findings in 40% of patients with sarcoidosis. Immunological features of sarcoidosis include suppression of delayed-type hypersensitivity, heightened helper T-cell–type 1 immune response at disease sites, B-cell hyperactivity with circulating immune complexes, and increased levels of fibrinogenic cytokines. Ocular involvement occurs in approximately 25% of patients with sarcoidosis.

Frequency

United States

The prevalence of sarcoidosis is estimated to be 10-20 cases per 100,000 people. The incidence varies among geographic regions as well as with ethnicity. In the United States, African Americans have a 3-fold increased risk for sarcoidosis compared to whites, while Asians are rarely affected. African Americans are more likely to present acutely and to have more severe disease than whites who tend to present with asymptomatic and chronic disease. A number of studies have demonstrated a rural predominance in American adults with this disorder.

International

Sarcoidosis is a relatively common disease with worldwide distribution, affecting all genders, races, and ages. In Europe, the prevalence ranges from 3-50 cases per 100,000 people. In Japan, the incidence is less than 2 cases per 100,000 people. Ascertaining the true rate in underdeveloped nations is difficult because of the frequent failure to diagnose the disease and its misdiagnosis as tuberculosis.

Mortality/Morbidity

Pulmonary fibrosis and cardiac involvement are the 2 leading causes of death. Overall mortality ranges from 1-5%. Cardiac disease is the most commonly reported cause of death in Europe, while pulmonary disease accounts for the majority of the mortality in US patients.

Race

Sarcoidosis is much more frequent in certain ethnic and racial groups, ranging from less than 1 case up to 64 cases per 100,000 people worldwide.

  • In studies by Crystal, Newman, and Rybicki, a preponderance of disease occurred in American blacks as compared to whites.1,2,3 The reported ratio of American blacks to whites ranges from 3:1 to 17:1.
  • Sarcoidosis tends to be more severe and chronic in American blacks, with a higher risk of extrathoracic manifestations.
  • American blacks have twice the ocular and ocular adnexal involvement as American whites.

Sex

A slight female preponderance and susceptibility have been noted in several studies, as noted by Bresnitz.4

Age

Most symptomatic patients are aged 20-40 years. Sarcoidosis also can be seen in children and elderly persons. Children do not appear to show the female predominance that is noted in adults. Additionally, in the United States, children do not seem to demonstrate the higher prevalence in African Americans that is seen in adult populations.

Clinical

History

Sarcoidosis may present with symptoms referable to a single organ or to multiple organ systems. Historical presentation depends on the severity of the organ(s) involved. Five percent of cases are asymptomatic; the diagnosis is suspected and pursued after a chest radiograph is performed for an unrelated reason.

  • Constitutional symptoms: Most patients have symptoms of fatigue, malaise, anorexia, weight loss, and fever at onset.
  • Pulmonary symptoms: Dyspnea on exertion, retrosternal chest pain, and cough may occur in one third to one half of all patients. Even in patients with primarily extrathoracic sarcoidosis, subclinical pulmonary sarcoidosis is often present.
  • Systemic syndromes
    • Löfgren syndrome represents a more acute presentation, comprising hilar lymphadenopathy, erythema nodosum, and polyarthralgia.
    • Heerfordt syndrome (uveoparotid fever) includes uveitis, salivary gland involvement, and cranial nerve palsies.
  • Extrapulmonary involvement: This includes (in order of frequency) lymphadenopathy, skin lesions, ocular lesions, and upper respiratory, marrow and splenic, hepatic, renal, neurologic, musculoskeletal, cardiac, and endocrine manifestations.
  • Neurologic sarcoidosis: General symptoms include headache, dizziness, facial weakness, hemiparesis, paresthesias, gait disturbances, impaired memory, decreased hearing, seizures, and rarely psychiatric symptoms.
  • Neuro-ophthalmologic involvement: Symptoms include diplopia from cranial nerve palsies and decreased vision with or without scotoma from optic nerve infiltration or edema. Visual symptoms from glaucomatous optic nerve damage may be superimposed.
  • Ocular sarcoidosis: Symptoms from uveitis include blurred vision, photophobia, floaters, redness, scotomata, and pain. Periocular lesions may produce dry eye symptoms, as well as disfiguring lid, periocular, and adnexal lesions. Orbital involvement by a mass lesion may cause proptosis and diplopia.

Physical

  • Pulmonary system: Physical signs of pulmonary involvement include dry rales, restricted lung volumes, abnormal gas exchange, abnormal arterial blood gas parameters, and occasional hemoptysis. Clinical staging is completed using international radiologic criteria. Radiologic findings reflect interstitial lung disease involving alveoli, vessels, and bronchioles.
  • Lymphatic system: Hilar lymphadenopathy may be seen in up to 90% of patients.
  • Cardiovascular system: Serious cardiac dysfunction is noted in 5-10% of patients with sarcoidosis.
  • Skin: Up to 25% of patients with sarcoidosis will have 1 or more skin lesions. Cutaneous manifestations of sarcoidosis include erythema nodosum and chronic maculopapular lesions.
  • Renal and endocrine systems: Clinically significant renal tubular, glomerular, and arterial involvement is rare.
  • Gastrointestinal system: Liver biopsy results reveal granulomatous involvement in 40-90% of patients, but clinically significant liver abnormalities are rare.
  • Neurologic system
    • Lesions may occur anywhere in the central or peripheral nervous systems. Neurosarcoidosis carries a mortality rate of 10%, twice the overall mortality rate of sarcoidosis.
    • Cranial neuropathy and hypothalamic/pituitary lesions tend to occur early and respond favorably to treatment. Space-occupying lesions, peripheral neuropathy, and neuromuscular involvement tend to occur later and portend a chronic course. Neurosarcoid usually presents with other systemic manifestations.
    • Cranial nerve involvement commonly occurs and may be bilateral. Classically, the most common lesion of neurosarcoid is a unilateral seventh (facial) nerve palsy. Most cases of seventh nerve palsy are lower motor neuron type lesions. Nerve palsies involving the auditory, glossopharyngeal, or vagus nerves have been described.
    • Peripheral mononeuropathy or polyneuropathy may occur.
    • Sarcoidosis may present with seizures or meningitis. Cerebrospinal fluid (CSF) findings in patients with central nervous system (CNS) involvement may include pleocytosis (mainly lymphocytosis), elevated protein levels or cells (up to 81% of patients), and decreased glucose levels (20% of patients). CSF angiotensin-converting enzyme (ACE) levels may be elevated in 50% of patients, and lysozyme levels, beta2-macroglobulin levels, and CD4/CD8 ratio may be increased.
    • Space-occupying lesions may occur in any part of the CNS. Supratentorial granulomatous lesions are more common than infratentorial masses. Symptoms are nonspecific and include headaches, lethargy, seizures, papilledema, and optic atrophy. The most common intracranial sites of involvement are the hypothalamus, the third ventricle region, and the pituitary gland. Intraspinal lesions occasionally are seen and may mimic multiple sclerosis (MS). One third of patients with spinal cord involvement are asymptomatic.
  • Neuro-ophthalmologic manifestations
    • Oculomotor, trochlear, or abducens nerve (cranial nerve III, IV, or VI) involvement is rare but may cause diplopia or ptosis.
    • The optic nerve is commonly involved, occurring in 5-38% of patients with neurosarcoid. Postmortem studies and physiologic studies (visual-evoked potentials) indicate a high degree of subclinical disease. Coexisting uveitis also may obscure optic nerve involvement. Optic nerve or chiasmal involvement is seen in one third to one half of patients with neurosarcoid, with two thirds of patients having unilateral involvement and one third of patients having bilateral disease. Nonspecific optic nerve head swelling (papillitis), direct invasion of the optic disc or nerve, or papilledema due to increased intracranial pressure may be present.
    • Examination of the optic nerve head may reveal edema, vascular engorgement, nerve head elevation, hemorrhage, or optic atrophy. If the lesion is retrobulbar, the optic nerve may appear normal. Isolated infiltrative optic neuropathy as the initial manifestation of sarcoidosis is rare. Retrobulbar optic nerve lesions may masquerade as optic nerve glioma or meningioma. Neuro-ophthalmic findings may present without systemic evidence of disease and elude diagnosis until a biopsy is performed.
  • Ocular manifestations
    • Involvement of the eyes and adnexa ranges from 25-54% of patients. Most patients with ophthalmic sarcoidosis have evidence of systemic involvement at the time of the initial examination.
    • Ocular syndromes: Heerfordt syndrome is characterized by uveitis, which may precede the parotid enlargement and occasionally papilledema. Löfgren syndrome, which is characterized by erythema nodosum, bilateral hilar adenopathy, and arthralgias, is associated with anterior uveitis in 6% of patients.
    • Skin and adnexa: Lacrimal gland involvement occurs in 15-28% of patients. Lacrimal gland involvement is usually a painless, bilateral, palpable swelling of the gland. Moderate-to-severe keratitis sicca may result.
  • Anterior segment disease
    • Conjunctiva: Granulomatous nodules are the most common lesions. Keratoconjunctivitis sicca presumably results from infiltration of the lacrimal and minor lacrimal glands or from chronic inflammation of the lacrimal ductules.
    • Cornea: Interstitial keratitis rarely may occur. Band keratopathy is infrequent. Scleritis is rare. Cataract is a frequent complication of uveitis and/or the corticosteroids used in treatment.
    • Uveitis
      • Anterior uveitis is the most common ocular manifestation of sarcoidosis, although up to one third of patients with posterior uveitis may have no signs of anterior segment inflammation.
      • The signs of anterior uveitis typically include granulomatous or mutton-fat keratic precipitates, iris nodules, posterior synechiae, and peripheral anterior synechiae.
      • Sequelae of chronic uveitis include anterior and posterior synechiae, iris bombé, glaucoma, cystoid macular edema, vitritis, retinal neovascularization, and cataract.
    • Glaucoma: Both open-angle and angle-closure mechanisms may be seen in sarcoidosis. Most patients with intraocular pressure elevation have a chronic open-angle–type glaucoma. This picture is complicated by steroid-responsive glaucoma, in which approximately 6% of the population develops open-angle glaucoma when given topical corticosteroids for 4-6 weeks. Therefore, gonioscopy is essential in the evaluation of patients with elevated intraocular pressure.
  • Posterior segment disease: Posterior findings occur in 25-30% of patients with sarcoidosis.
    • Vitreous: Clinical features seen in the vitreous cavity comprise cellular infiltrates, opacities, haze, syneresis, posterior vitreous detachment, and hemorrhages. Grayish green-white opacities (or snowballs) may be seen in the anterior inferior vitreous. The opacities often occur in chains and are described as a string of pearls. Vitreous hemorrhage is rare and may result from retinal neovascularization.
    • Vessels: Periphlebitis is not uncommon; in sarcoidosis, perivasculitis is limited to the retinal veins, tends to be segmental, and involves the small branch veins. En taches de bougie (candle-wax drippings) is used to describe the perivenous exudates. Periphlebitis tends to resolve dramatically on steroid therapy. Central retinal vein occlusions are rare.
    • Retina: Neovascularization was found in approximately one fourth of Spalton's patients and was the most significant factor contributing to poor prognosis. Sequelae included vitreous hemorrhage, ischemia, and retinal detachment. Neovascularization may respond to corticosteroids and warrant a therapeutic trial. Panretinal photocoagulation and/or vitrectomy may be required for neovascularization. Retinal detachment is rare.
    • Cystoid macular edema (CME) is the accumulation of extracellular fluid in the outer plexiform layer of the retina. Intravenous fluorescein angiography (IVFA) may demonstrate dye leakage in the perifoveal area when frank cystoid spaces are not identified ophthalmoscopically.
    • Retinal pigment epithelium (RPE): Focal and discrete subretinal mottling may be seen in the inferior equatorial RPE but occasionally may be seen in the posterior pole.
    • Pars plana exudates may be indistinguishable from idiopathic pars planitis. Histopathology may reveal fibrinous exudates overlying the pars plana and extending into the vitreous.
    • Choroid: Lesions are typically multiple, yellowish, elevated nodules.
  • Orbital disease: Approximately 20% of patients with ophthalmic findings of sarcoid have soft tissue involvement of the orbit or lacrimal gland.
    • Sarcoidosis involving the orbital space may present as a mass lesion with proptosis, ptosis, or ophthalmoplegia.
    • The lacrimal gland may be enlarged on the basis of sarcoid infiltration.

Causes

The etiology of sarcoidosis remains unknown. Genetic and environmental factors may play a role, but a review of the literature strongly suggests that hereditary and environmental factors alone may not account for all cases of sarcoidosis.

Sarcoidosis appears to be an antigen-mediated granulomatous process characterized by cytokine dysregulation. Sarcoidosis is not a malignancy nor is it an autoimmune disorder. Various infectious, allergic, chemical, and drug causes have been suggested and dismissed for lack of conclusive evidence. Mycobacteria and atypical pathogens have been repeatedly suggested as etiologic factors. One hypothesis is based on mounting evidence of mycobacterial DNA in granulomas. DNA from slow-growing mycobacteria, preferentially persisting in macrophages, has been found to elicit an allergic reaction in the setting of a hyperresponsive host. Whether the exogenous DNA in the granulomas is incidental or causal in the pathogenesis remains to be seen.

Two leading hypotheses have evolved regarding the etiology. One hypothesis is that sarcoidosis is most likely the result of exposure of a susceptible host to a potential etiologic agent. An alternative hypothesis cedes that sarcoidosis may be a clinical syndrome that includes a collection of different diseases, each with a different etiology.

More on Sarcoidosis

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