What are the most common organs affected by sarcoidosis, other than the lungs, and what are the associated symptoms?

Common Organs Affected by Sarcoidosis Beyond the Lungs and Their Symptoms

The most common organs affected by sarcoidosis beyond the lungs are the skin, eyes, liver, peripheral lymph nodes, heart, and nervous system, each with distinct symptom presentations that significantly impact morbidity, mortality, and quality of life. 1, 2

Skin Involvement (10-30% of cases)

• Common manifestations:

  • Lupus pernio: Violaceous lesions on nose, cheeks, and ears 1
  • Erythema nodosum: Painful red nodules typically on shins 1, 3
  • Maculopapular or violaceous skin lesions 1
  • Subcutaneous nodules 1

• Clinical significance: Lupus pernio and plaque-type lesions are associated with more severe systemic disease and chronic course, while erythema nodosum often indicates acute and more benign disease 3

Ocular Involvement (10-30% of cases)

• Common manifestations:

  • Uveitis (anterior, posterior, or panuveitis) 1
  • Conjunctival nodules or granulomas 4
  • Scleritis and episcleritis 4
  • Lacrimal gland swelling 1
  • Optic neuritis 1
  • Retinitis 1

• Important note: All sarcoidosis patients should be screened for eye involvement as asymptomatic disease can cause permanent vision impairment if untreated 2

Liver and Spleen Involvement (10-30%)

• Common manifestations:
  • Often asymptomatic 2
  • Hepatomegaly or splenomegaly 1
  • Elevated alkaline phosphatase (>3× upper limit of normal) 1
  • Nodules in liver or spleen visible on imaging 1

Cardiac Involvement (5% symptomatic, 25% histological)

• Common manifestations:

  • Atrioventricular block and arrhythmias 1
  • Cardiomyopathy 1
  • Heart failure 1
  • Sudden cardiac death (particularly common in Japanese patients) 1, 5

• Critical note: Cardiac sarcoidosis may be suddenly fatal, making screening essential for all sarcoidosis patients despite often being asymptomatic 2

Neurological Involvement

• Common manifestations:
  • Seventh cranial nerve paralysis (facial nerve) 1
  • Small fiber neuropathy (burning pain, paresthesias) 2
  • Gadolinium enhancement on CNS MRI 1
  • Headache, seizures, or cognitive dysfunction with CNS involvement

Musculoskeletal Involvement

• Common manifestations:
  • Bone lesions: Osteolytic lesions, cysts, or punched-out lesions 1
  • Joint pain and swelling 1
  • Myopathy with muscle weakness

Renal Involvement

• Common manifestations:
  • Treatment-responsive renal failure 1
  • Nephrolithiasis with calcium stones 1
  • Hypercalcemia (10-13% of patients) 4
  • Hypercalciuria (approximately 30% of patients) 4

Systemic Symptoms

• Common manifestations:
  • Fatigue (affects large percentage of patients) 2
  • Weight loss 6
  • Night sweats 6
  • Fever

Diagnostic Considerations

• Diagnosis requires compatible clinical and radiologic presentation, pathologic evidence of noncaseating granulomas, and exclusion of other diseases with similar findings 1
• Elevated angiotensin-converting enzyme (ACE) levels are found in 60-83% of patients 4, 1
• Hypercalcemia (10-13%) and hypercalciuria (~30%) are common findings 4

Treatment Approach

• Treatment decisions should be based on organ involvement, risk for significant morbidity, and impact on quality of life 4
• Glucocorticoids remain first-line therapy for symptomatic disease, though prolonged use causes significant toxicity 4
• Immunosuppressive agents (methotrexate, azathioprine) and anti-TNF medications are used as second and third-line treatments 5, 7

Prognosis

• Mortality rate is approximately 7% over 5 years 5
• Higher mortality is observed in African American women (2.4 times higher than matched cohorts) 1
• Over 60% of deaths from sarcoidosis are due to advanced cardiopulmonary disease 5

Understanding the multi-organ manifestations of sarcoidosis is essential for early diagnosis and appropriate management to prevent long-term complications and improve quality of life.