Sarcoidosis Flashcards
What is sarcoidosis?
Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent. Sarcoidosis remits without treatment in approximately two-thirds of people
Factors associated with poor prognosis
- insidious onset, symptoms > 6 months
- absence of erythema nodosum
- extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
- CXR: stage III-IV features
- black people
Possible Xray features?
chest x-ray shows bilateral hilar lymphadenopathy. A combination with typical symptoms (a cough, low-grade fever and erythema nodosum) this makes a diagnosis of sarcoidosis very likely. Sarcoidosis is also more common in people of Afro-Caribbean ethnicity.
Sarcoidosis possible clinical features?
dyspnoea
cough
painful shin lesions
bilateral hilar lymphadenopathy
Management 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.
Imaging investigations 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
Blood test investigations for 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
Differential diagnosis of sarcoidosis?
Tuberculosis
Lymphoma
Hypersensitivity pneumonitis
HIV
Toxoplasmosis
Histoplasmosis
Which syndrome explains a specific presentation of sarcoidosis?
Erythema nodosum
Bilateral hilar lymphadenopathy
Polyarthralgia (joint pain in multiple joints)
TOM TIP: typical presentation case?
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.
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.
