Lacrimal gland sarcoidosis

Key points

Definition: Bilateral lacrimal gland enlargement secondary to sarcoidosis, a systemic idiopathic inflammatory granulomatous disease.
Classic clue: Bilateral lacrimal gland enlargement in a patient with “dry-eye” symptoms and known (or findings consistent with) sarcoidosis.
Despite decades of delving into this dire disease, its etiology remains unknown.
Sarcoid’s ocular and orbital emergence may precede by decades the development of systemic symptoms. ^,
Important for radiologists to recognize the abnormality and/or consider it as a differential diagnosis.
With no absolute diagnostic criterion, sarcoidosis remains a diagnosis of exclusion. ^,
Biopsy remains the gold standard. ^,

Bilateral lacrimal gland enlargement with avid postcontrast enhancement.
Proptosis with medial displacement of globes.
Absence of adjacent bony orbital invasion and destruction are pertinent negative findings.

Decreased T1.
Increased signal on T2.
Avid gadolinium enhancement.
Cranial magnetic resonance imaging should be obtained to exclude involvement of intracranial optic pathways and coexisting neurosarcoidosis.
Parotid gland involvement in 6% of patients with sarcoidosis (see Figure 39-1 , C ).

Discomfort, diplopia, proptosis, ptosis, and eyelid swelling.
Fever, fatigue, weakness, weight loss, malaise.
Pulmonary symptoms include cough, chest pain, and dyspnea.
Enlargement and/or irritation of affected organs.
Sarcoidosis is often referred to as an impersonator or “snowflake” disease because patients predictably present with several symptoms.

Usually presents in 20- to 40-year-olds with a second peak between 50 and 60 years of age.
Incidence is greater in females than in males.
Overall incidence in whites is approximately 20 per 100,000.
May affect any organ system.
Half of patients are asymptomatic.
African Americans, Irish, and Scandinavians most commonly affected. ^,
Lacrimal gland involvement in more than 50% of patients with sarcoidosis causes dry-eye symptoms.
Other ocular involvement includes anterior uveitis, vitritis, vasculitis, choroiditis.
Neurologic involvement of cranial nerves (including optic nerve), pituitary, spinal cord, and leptomeninges.
90% of patients have pulmonary involvement including interstitial infiltrates with pulmonary fibrosis, hypertension, and restrictive pulmonary disease, as well as familiar hilar adenopathy (see Figure 39-1 , B ).
30% of patients have dermatologic involvement including cutaneous sarcoid and erythema nodosum.
25% of patients have cardiac involvement including dysrhythmias and cardiomyopathy.
Workup may reveal renal failure and splenomegaly.
Lofgren syndrome: hilar adenopathy, polyarthralgias, and erythema nodosum.

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