University of Iowa Health Care

Ophthalmology and Visual Sciences

EyeRounds.org

Sarcoidosis:

53-year-old African American female with gradual, painless loss of vision in both eyes

Andrew Doan, MD, PhD Michael Wall, MD

February 21, 2005

Chief Complaint: Gradual, painless loss of vision in both eyes.

History of Present Illness: 53-year-old African American female with gradual, painless loss of vision OS 2 months prior to exam. "My vision went black, but it came back over one month in the left eye. I also have a history of goiter." One month later, she experienced gradual, painless loss of vision OD to no light perception (NLP).

She denied HA, jaw pain, scalp tenderness, but reported 50 lbs weight loss, fatigue, and severe dry eyes.

PMH: significant only for goiter which has been stable for several years. No other medical problems. No previous ocular history.

EXAM:

  • Vision: NLP OD, 20/40 OS
  • Pupils: 6 mm in dark, 6 mm in light OD; 6 mm in dark, 3 mm in light OS; >3.0 Log Unit (very big) RAPD OD.
  • IOP: 18 mmHg OD, 20 mmHg OS
  • EOM: full motility OU.
  • Anterior segment exam unremarkable.
  • DFE: normal macula, vessels, and periphery OU. Optic nerves were normal with 0.4 C/D ratio OU without evidence of pallor or edema.
  • ECHO of the orbits were normal without evidence of thyroid ophthalmopathy.

Goldmann Visual Field OS with visual loss. GVF

MRI: Coronal image of the head, T1 weighted, post-contrast, with fat suppression - enhancement of right optic nerve
MRI

MRI: Axial image of the orbits/head, T1 weighted, post-contrast, with fat suppression - nodular enhancement of right and left optic nerves.
MRI


Requested Labs & Studies

  • ESR = 75 mm/hr
  • CRP = 3.5 mg/dl (normal < 0.5 mg/dl)
  • ACE = 62
  • CBC wnl
  • ALT = 79 U/L
  • AST = 74 U/L
  • Alkaline Phosphatase = 189 U/L

Discussion

Although more common in African Americans, sarcoidosis can be found in Caucasians. In Iowa, we see numerous new cases yearly, and sarcoid must be on the differential of unilateral or bilateral vision loss associated with systemic manifestations, e.g. weight loss, fatigue, breathing problems, fevers, and dry eyes. Sarcoid can manifest in the lacrimal and parotid glands; thus, patients may complain of severe dry eyes or dry mouth. The above MRI imaging studies denote nodular inflammation of both optic nerves (right greater than left optic nerve).

An elevated ACE and liver enzymes in this patient suggest lung and liver disease. The chest x-ray showed a single, large nodule in the left upper lobe. Without a CT scan and biopsy, the differential diagnosis should include lung tumor at this point. A CT scan was performed which showed perihilar lymphadenopathy as well as the large left upper lobe nodule. Bronchoscopy was performed, and aspirate showed numerous non-necrotizing granulomas, which supports the diagnosis of sarcoidosis. Sarcoidosis can be associated with uveitis or retinal lesions; however, the absence of uveitis or retinal disease does not rule-out sarcoid.

Sarcoid is a multi-systemic disease that may affect the: skin, brain, eyes, lungs, spleen, thyroid, and liver. It is a vision threatening and deadly disease, and sarcoid is responsive to aggressive immunosuppression therapy with high dose steroids.

This patient was admitted to the neurology service for neuro-sarcoidosis and started on IV steroids. Her vision in the right eye did not improve, but the left eye improved from 20/40 to 20/20 in two days. Her visual field also improved:

GVF
GVF before treatment GVF 2 days after Steroids IV
GVF GVF

Diagnosis: Sarcoidosis

EPIDEMIOLOGY

  • Prevalence: 10-40 cases per 100,000.
  • African Americans affected more than Caucasions by ~10:1.
  • Affects all ages with most patients being 20- to 40--year-olds.

SIGNS

  • conjunctival and retinal granulomas & retinal vasculitis
  • vision loss that may recover
  • non-necrotizing granulomatous involvment of mutiple organ systems: skin, brain, eyes, lungs, spleen, thyroid, and liver.
  • pulmonary nodules and lymphadenopathy
  • elevated ACE
  • elevated LFTs if liver invovled

SYMPTOMS

  • uveitis (absence does not rule out disease)
  • weight loss
  • fatigue
  • breathing problems
  • fevers
  • dry eyes (involvement of lacrimal gland)
  • dry mouth (involvement of parotid gland

TREATMENT

  • If vision or life threatening, then admission to hospital for IV steroids.
  • May need long-term steroid and immunosuppression therapy.

Differential Diagnoses

  • Atypical optic neuritis
  • lymphoma
  • paraneoplastic syndrome
  • vasculitis

Suggested citation format: Doan A, Wall M. Sarcoidosis: 53-year-old African American female with gradual, painless loss of vision in both eyes.EyeRounds.org. February 21, 2005; Available from: http://www.EyeRounds.org/cases/case3.htm.

last updated: 02-21-2005
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