Sarcoidosis Flashcards
What is it?
Sarcoidosis is a granulomatous inflammatory condition. Granulomas are nodules of inflammation full of macrophages.
what organs are affected?
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
What is lofgern’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)
Differential Diagnosis?
Tuberculosis
Lymphoma
Hypersensitivity pneumonitis
HIV
Toxoplasmosis
Histoplasmosis
Blood Tests?
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 from biopsy?
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 organs?
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
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?
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.
