- Chronic granulomatous disorder.
- Granulomas are inflammatory nodules full of macrophages
- Unknown cause
- Mainly respiratory but all organs affected
Hypercalcaemia + bilateral hilar lymphadenopathy ?sarcoidosis
Causes/Factors
- Aged 20-40 or around 60
- Women
- Black ethnic origin
Tip
The typical MCQ exam patient is a 20-40 year old black female presenting with a dry cough and shortness of breath. They may have nodules on their shins, suggesting erythema nodosum.
Issues per organ
Lungs
- Mediastinal lymphadenopathy
- Pulmonary fibrosis
- Pulmonary nodules
Systemic
- Fever
- Fatigue
- Weight loss
Liver
- Liver nodules
- Cirrhosis
- Cholestasis
Eyes
Heart
Kidneys
- Stones (Hypercalcaemia)
- Nephrocalcinosis
- Tubulointerstitial Nephritis
Others but more non specific
Lofgren’s Syndrome
Refers to a specific presentation of sarcoidosis with a classic triad of symptoms:
- erythema nodosum
- Bilateral hilar lymphadenopathy
- Polyarthralgia (joint pain in multiple joints)
Differentials
The top differentials for the varied presenting features of sarcoidosis are:
- Tuberculosis
- Lymphoma
- Hypersensitivity Pneumonitis
- HIV
- Toxoplasmosis
- Histoplasmosis
Investigations
The blood test findings to remember are:
- Raised angiotensin-converting enzyme (ACE) (often used as a screening test)
- Raised calcium (hypercalcaemia)
- U&Es for kidney involvement
- Urine albumin-creatinine ratio to look for proteinuria
- LFTs for liver involvement
Imaging
- Chest x-ray may show hilar lymphadenopathy
- High-resolution CT scanning may show hilar lymphadenopathy and pulmonary nodules
- MRI can show central nervous system involvement
- PET scan can show active inflammation in affected areas
Management
Conservative management is considered in patients with no or mild symptoms.
Oral steroids (for 6-24 months) are usually first-line where treatment is required. Bisphosphonates protect against osteoporosis whilst on long-term steroids.
Methotrexate is a second-line option.
Lung transplant is rarely required in severe pulmonary disease.
Complications/red Flags
Spontaneously resolves in 50% of patients. Overall mortality of less than 10%