Sarcoidosis Flashcards
What
A granulomatous inflammatory condition. Granulomas are nodules of inflammation full of macrophages. The cause of these granulomas developing is unknown.
It is usually associated with chest symptoms but also has multiple extra-pulmonary manifestations such as erythema nodosum and lymphadenopathy. Symptoms can vary dramatically from asymptomatic (in up to 50%) to severe and life-threatening.
There are two spikes in incidence, in young adulthood and again around age 60. Women are affected more often and it occurs more frequently in black people compared with other ethnic groups.
TOM TIP: The typical MCQ exam patient is a 20-40 year old black woman presenting with a dry cough and shortness of breath. They may have nodules on their shins suggesting erythema nodosum.
Present
Sarcoidosis can affect almost any organ in the body. The most commonly affected are the lungs so sarcoidosis is usually managed by respiratory physicians.
Lungs (affecting over 90%)
- Mediastinal lymphadenopathy
- Pulmonary fibrosis
- Pulmonary nodules
Systemic Symptoms
- Fever
- Fatigue
- Weight loss
Liver (affecting around 20%)
- Liver nodules
- Cirrhosis
- Cholestasis
Eyes (affecting around 20%)
- Uveitis
- Conjunctivitis
- Optic neuritis
Skin (affecting around 15%)
- Erythema nodosum (tender, red nodules on the shins caused by inflammation of the subcutaneous fat)
- Lupus pernio (raised, purple skin lesions commonly on cheeks and nose)
- Granulomas develop in scar tissue
Heart (affecting around 5%)
- Bundle branch block
- Heart block
- Myocardial muscle involvement
Kidneys (affecting around 5%)
- Kidney stones (due to hypercalcaemia)
- Nephrocalcinosis
- Interstitial nephritis
Central nervous system (affecting around 5%)
- Nodules
- Pituitary involvement (diabetes insipidus)
- Encephalopathy
Peripheral Nervous System (affecting around 5%)
- Facial nerve palsy
- Mononeuritis multiplex
Bones (affecting around 2%)
- Arthralgia
- Arthritis
- Myopathy
Diagnosis
Raised serum ACE. This is often used as a screening test.
Hypercalcaemia (rasied calcium) is a key finding.
Raised serum soluble interleukin-2 receptor
Raised CRP
Raised immunoglobulins
Imaging
Chest xray shows hilar lymphadenopathy
High-resolution CT thorax shows hilar lymphadenopathy and pulmonary nodules
MRI can show CNS involvement
PET scan can show active inflammation in affected areas
Histology
The gold standard for confirming the diagnosis of sarcoidosis is by histology from a biopsy. This is usually done by doing bronchoscopy with ultrasound guided biopsy of mediastinal lymph nodes.
The histology shows characteristic non-caseating granulomas with epithelioid cells.
Tests for other organ involvement
U&Es for kidney involvement
Urine dipstick or urine albumin-creatinine ratio to look for proteinuria indicating nephritis
LFTs for liver involvement
Ophthalmology review for eye involvement
ECG and echocardiogram for heart involvement
Ultrasound abdomen for liver and kidney involvement
Treatment
Treatment
No treatment is considered as first line in patients with no or mild symptoms as the condition often resolves spontaneously.
Oral steroids are usually first line where treatment is required and are given for between 6 and 24 months. Patients should be given bisphosphonates to protect against osteoporosis whilst on such long term steroids.
Second line options are methotrexate or azathioprine
Lung transplant is rarely required in severe pulmonary disease
