Sarcoidosis Flashcards
What is sarcoidosis?
Sarcoidosis is a granulomatous inflammatory condition. Granulomas are nodules of inflammation full of macrophages. The cause of these granulomas developing is unknown.
What is sarcoidosis associated with?
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.
What is the incidence of sarcoidosis?
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.
What is the common presentation of sarcoidosis?
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.
What organs are affected in sarcoidosis?
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
Lofgren’s syndrome
This is a specific presentation of sarcoidosis. It is characteristic by a triad of:
- Erythema nodosum
- Bilateral hilar lymphadenopathy
- Polyarthralgia (joint pain in multiple joints)
DDx of sarcoidosis
Tuberculosis Lymphoma Hypersensitivity pneumonitis HIV Toxoplasmosis Histoplasmosis
What blood tests results are seen in sarcoidosis?
- 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
What is seen on imaging of sarcoidosis?
- 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
What is seen on histology of sarcoidosis?
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 of sarcoidosis?
- 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
Prognosis of sarcoidosis
Sarcoidosis spontaneously resolves within 6 months in around 60% of patients. In a small number of patients it progresses with pulmonary fibrosis and pulmonary hypertension, potentially requiring a lung transplant. Death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the central nervous system.
