Systemic Involvement in Sarcoidosis
System | Estimated Frequency | Comments |
|---|---|---|
Pulmonary | > 90% | Granulomas form in alveolar septa and bronchiolar and bronchial walls, causing diffuse pulmonary disease; pulmonary arteries and veins also involved Often asymptomatic Spontaneously resolves in many patients but can cause progressive pulmonary dysfunction, leading to limitations in physical function, respiratory failure, and death in a few |
Pulmonary lymphatic | 90% | Hilar or mediastinal involvement incidentally detected by chest x-ray in most patients; nontender to tender peripheral or cervical lymphadenopathy in others |
Muscle | 50–80% | Asymptomatic disease with or without enzyme elevations in most patients Sometimes insidious or acute myopathy with muscle weakness |
Hepatic | 40–75% | Usually asymptomatic Manifests with mild elevations in liver function test results, hypolucent lesions on CT scans with contrast Rarely, clinically significant cholestasis or cirrhosis Unclear distinction between sarcoidosis and granulomatous hepatitis when sarcoidosis affects the liver only |
Joint | 25–50% | Ankle, knee, wrist, and elbow arthritis (most common) May cause chronic arthritis with Jaccoud deformities or dactylitis Löfgren syndrome (triad of acute migratory polyarthritis, erythema nodosum, and hilar adenopathy) |
Hematologic | < 5–30% | Anemia due to granulomatous infiltration of bone marrow, sometimes causing pancytopenia Splenic sequestration causing thrombocytopenia |
Dermatologic | 25% |
Biopsy of erythema nodosum lesions is unnecessary because granulomas, characteristic of sarcoidosis, are absent Common skin lesions:
Lupus pernio:
|
Ocular | 25% | Uveitis (most common), causing blurred vision, photophobia, and tearing Can cause blindness Spontaneously resolves in most patients May manifest with conjunctivitis, iridocyclitis, chorioretinitis, dacryocystitis, lacrimal gland infiltration causing dry eyes, optic neuritis, glaucoma, or cataracts Ocular involvement more common among black American people and people of Japanese descent Annual screening indicated for early disease detection |
Psychiatric | 10% | Depression (common), but uncertain whether it is a primary manifestation of sarcoidosis or a response to the prolonged course of disease and frequent recurrences |
Renal | 10% | Asymptomatic hypercalciuria (most common) Chronic kidney disease caused by nephrolithiasis and nephrocalcinosis and requiring renal replacement (dialysis or transplantation) in some patients |
Splenic | 10% | Usually asymptomatic Manifests with left upper quadrant pain and thrombocytopenia or as an incidental finding on ultrasound or CT |
Neurologic | < 10% | Cranial neuropathy, especially the 7th nerve (causing facial nerve palsy) or 8th nerve (causing hearing loss) Optic and peripheral neuropathy (common) May affect any cranial nerve Central nervous system involvement, with nodular lesions or diffuse meningeal inflammation typically in the cerebellum and brain stem (central diabetes insipidus), polyphagia and obesity, and thermoregulatory and libidinal changes |
Nasal sinus | < 10% | Acute and chronic granulomatous inflammation of sinus mucosa with symptoms indistinguishable from common allergic and infectious sinusitis Diagnosis confirmed by biopsy More common in patients with lupus pernio |
Cardiac | 5% | Conduction blocks and arrhythmias (most common), sometimes causing sudden death (cardiac sarcoidosis) Heart failure due to restrictive cardiomyopathy (primary) or Class V pulmonary hypertension (multifactorial) Transient papillary muscle dysfunction and pericarditis (rare) More common among patients of Japanese ancestry, in whom cardiomyopathy is the most frequent cause of sarcoidosis-related death |
Bone | 5% | Osteolytic or cystic lesions Osteopenia |
Oral | < 5% | Asymptomatic parotid swelling (most common) Parotitis with xerostomia Heerfordt syndrome (uveoparotid fever), characterized by uveitis, bilateral parotid swelling, facial palsy, and chronic fever Oral lupus pernio, which may disfigure the hard palate and may involve the cheek, tongue, and gums |
Gastric or intestinal | Rare | Rarely gastric granulomas Rarely intestinal involvement Mesenteric lymphadenopathy that may cause abdominal pain |
Endocrine | Rare | Hypothalamic and pituitary stalk infiltration, possibly causing panhypopituitarism May cause thyroid infiltration without dysfunction Secondary hypoparathyroidism due to hypercalcemia |
Pleural | Rare | Causes lymphocytic exudative effusions, usually bilateral |
Reproductive | Rare | Case reports of endometrial, ovarian, epididymal, and testicular involvement No effect on fertility May subside during pregnancy and relapse postpartum |