Sarcoidosis Flashcards
What is a granuloma?
A small, noncancerous cluster of immune cells (macrophages) that forms in response to chronic inflammation or infection.
What is sarcoidosis?
A systemic granulomatous disease characterised by the formation of non-caseating granulomas, which can affect any organ.
What are the 2 most commonly involved sites in saroidosis?
1) lungs
2) intrathoracic lymph nodes
Pathophysiology of sarcoidosis?
Characterised by an exaggerated immune response, leading to the formation of non-caseating granuloma
What 2 clinical syndromes are associated with sarcoidosis?
1) Lofgren’s syndrome
2) Heerfordt’s syndrome
What is Lofgren’s syndrome?
An acute form of the disease (sarcoidosis).
It usually carries an excellent prognosis
What 4 features are seen in Lofgren’s syndrome?
1) bilateral hilar lymphadenopathy (BHL)
2) erythema nodosum
3) fever
4) polyarthralgia
What features are seen in Heerfordt’s syndrome (uveoparotid fever)? (3)
1) parotid enlargement
2) fever
3) uveitis
all 2ary to sarcoidosis
Clinical features of sarcoidosis?
1) Common:
- cough
- SOB
- chest pain
- fever, fatigue & weight loss
2) Löfgren’s syndrome
3) Uveitis
4) Peripheral neuropathy
5) Cardiac arrhythmias
6) Skin manifestations e.g. erythema nodosum
What bloods are indicated in sarcoidosis?
1) FBC
2) Serum & urine calcium
3) LFTs
4) Serum ACE
5) Serum and urine electrophoresis
What may FBC show in sarcoidosis?
May demonstrate anaemia, leucopenia, or thrombocytopenia, although non-specific.
Role of serum & urine calcium test in sarcoidosis?
Hypercalcaemia & hypercalciuria can occur due to increased vitamin D activation by macrophages in sarcoidosis.
What can cause hypercalcaemia & hypercalciuria in sarcoidosis?
increased vitamin D activation by macrophages in sarcoidosis.
What may LFTs show in sarcoidosis?
Elevated ALP might suggest liver involvement
Serum ACE levels in sarcoidosis?
Raised
Role of serum and urine electrophoresis in sarcoidosis?
To exclude other granulomatous disorders e.g. multiple myeloma.
What pulmonary investigations may be indicated in sarcdoidosis?
1) CXR (1st line)
2) HRCT
3) Pulmonary function tests
4) Bronchoscopy with transbronchial lung biopsy
What can CXR shoe in sarcoidosis?
Bilateral hilar lymphadenopathy
What may pulmonary function tests show in sarcoidosis?
Restrictive pattern with reduced lung volumes and impaired gas transfer.
Bronchoscopy with transbronchial lung biopsy is a key investigation for obtaining tissue diagnosis.
What is a typical finding?
Non-caseating granulomas
Biopsy of the affected organ or tissue (e.g., skin, lymph node) is pivotal for a definitive diagnosis of sarcoidosis (gold standard).
Histologically, what is the hallmark of sarcoidosis?
Non-caseating granulomas
What is the gold standard investigation for diagnosis of sarcoidosis?
Tissue biopsy showing non-caseating granulomas
What does mx of sarcoidosis depend on?
Depends on the severity and extent of organ involvement.
Asymptomatic patients or those with mild disease may not require treatment, as spontaneous remission is common.
1st line xx of sarcoidosis in patients w/ symptomatic or progressive disease?
1st line –> systemic corticosteroids e.g. prednisolone 20-40mg daily
What are some factors associated with a poor prognosis in sarcoidosis?
1) insidious onset, symptoms > 6 months
2) an absence of erythema nodosum
3) extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
4) CXR: stage III-IV features
5) people of Black African ethnicity
Cause of elevated calcium and low PTH in sarcoid?
The elevated calcium and low PTH hormone can occur due to increased production of 1,25-dihydroxyvitamin D by activated macrophages in
the granulomas.
