Sarcoidosis Flashcards
What is sarcoidosis?
A type of interstitial lung disease. It is a multisystem granulomatous disorder of unknown cause which affects any organ system, but commonly involves the mediastinal lymph nodes and lung.
How is sarcoidosis often detected?
On routine xray
What are the brief pathophysiology of sarcoidosis?
- Typical sarcoid granulomas consist of focal accumulations of epithelioid cells, macrophages and lymphocytes (mainly T cells)
What are the risk factors for sarcoidosis?
- Family history
- First degree relatives have an increased risk (particularly with caucasians)
Race - Genetics: associated with HLA-DRB1 and DQB1 alleles
How is sarcoidosis usually found?
On a routine CXR in 20-40% of cases as it is often asymptomatic to begin with
What organs does sarcoidosis usually affect?
It can affect any organ but predominantly affects the lungs
How does acute sarcoidosis often present?
- Erythema nodosum (red lumps form on the shins and less commonly thighs and forearms)
- May get polyarthralgia (aches in joints and joint pain) - usually resolves spontaneously
What systemic symptoms would you get in sarcoidosis?
- Fever
- Weight loss
- Fatigue
What respiratory symptoms would you get in sarcoidosis?
- 90% have abnormal CXR with bilateral hilar lymphadenopathy with / without pulmonary infiltrates / fibrosis
- Dry cough
- Progressive dyspnoea
- Reduced exercise tolerance
- Chest pain
What would you see on the chest xray in someone with sarcoidosis?
- Typically presents with bilateral hilar lymphadenopathy
- Pulmonary infiltration
- Used for staging
0 = normal
1 = bilateral hilar lymphadenopathy
2 = pulmonary infiltrates with BHL
3 = pulmonary infiltrates WITHOUT BHL
4 = progressive pulmonary fibrosis, bulla, formation (honeycombing - confluence of two or more elements of the bronchial tree) and bronchiectasis
What would blood tests show in someone with sarcoidosis?
- Raised ESR
- Lymphopenia
- Raised LFT
- Raised Ca
- Raised immunoglobulins
- Serum ACE
- 24 hour urinary calcium if blood serum is normal
Apart from blood tests and CXR what other tests might you do in someone with sarcoidosis?
- Bronchoscopy
- ECG
- Lung function tests
- ## Tissue biopsy
What is the overall management for patients with sarcoidosis?
- Patients with bilateral hilar lymphadenopathy do not need treatment as most will recover spontaneously
- Symptomatic patients at stage 1, or asymptomatic patients at stage ⅔ do not need to be treated
- In general treat extra organ complications
What is the treatment for acute sarcoidosis?
- Bed rest
- NSAID use
What corticosteroids can be used in the treatment of sarcoidosis?
- Prednisolone orally then gradually reduce dose
- In severe illness, give IV methylprednisolone if steroid resistant then
- Methotrexate but close monitoring required
