Sarcoidosis Flashcards
What is sarcoidosis and how does it usually present clinically? (signs rather than symptoms)
Multisystem granulomatous disorder, commonly affecting young adults.
Usually presents with bilateral hilar lymphadenopathy, pulmonary infiltration, hepato-/spleno-megaly, and skin or eye lesions.
Which form of poisoning can mimic sarcoidosis?
Beryllium poisoning
At what age is the peak incidence of sarcoidosis?
30s and 40s
Does sarcoidosis have a genetic association?
No known relation to HLA BUT 1st degree relatives (particularly Caucasians) have increased risk of developing sarcoidosis.
What is the structure of the typical sarcoid granuloma?
Typical sarcoid granuloma: non-caseating, focal accumulation of epithelioid cells, macrophages and lymphocytes (mainly T cells)
What are the typical findings of bronchoalveolar lavage?
-Big increase in number of cells - Increase in lymphocytes (esp CD4 T cells) -Increase in macrophages (but reduced total %)
What are the common presentations of sarcoidosis?
1) Resp symptoms or abnormalities on CXR (50%)
2) Fatigue/weight loss (5%)
3) Peripheral lymphadenopathy (5%)
4) Fever (4%)
What are the stages of sarcoidosis pulmonary involvement?
Stage 1: bilateral hilar lymphadenopathy (BHL) alone
Stage 2: BHL with pulmonary infiltrates
Stage 3: pulmonary infiltrates w/o BHL
Stage 4: fibrosis
What are the DDx of bilateral hilar lymphadenopathy?
- Lymphoma: though rarely affects only hilar LNs
- Pulmonary TB: though rare for hilar LNs to enlarge symmetrically
- Carcinoma of bronchus + LN spread: though rarely symmetrical
What is the common effect of pulmonary infiltrates?
Mostly asymptomatic. Pulmonary infiltrate + NORMAL RFTs highly suggestive of sarcoidosis.
How does the CXR appear throughout the course of sarcoidosis?
Initially = mottling in mid zones. Evolves to generalised fine nodular shadows. Eventually widespread linear shadows (fibrosis).
What are the DDx of pulmonary infiltration?
- TB
- Pneumoconiosis
- Idiopathic pulmonary fibrosis
- Alveolar cell carcinoma
What are the most common extrapulmonary manifestations of sarcoidosis?
Skin
Ocular
What are the skin lesions caused by sarcoidosis?
-Erythema nodosum: sarcoidosis most common cause -Lupus pernio: bluish red infiltrate or swelling esp of nose or ears -Skin nodules
What are the manifestations of ocular sarcoidosis?
-Anterior uveitis: misting of vision, painful red eye -Posterior uveitis: progressive loss of vision
What is uveoparotid fever?
Syndrome of bilateral uveitis + parotid gland enlargement together with occasional development of facial nerve palsy; sometimes seen with sarcoidosis.
What are the metabolic complications of sarcoidosis? Why?
-Hypercalcaemia 10% cases Due to increase in 1,25-dihydroxyvitamin D (due to 1a-hydroxylation occurring in sarcoid macrophages in lung as well as in the kidney)
What are the bone and joint involvements associated with sarcoidosis?
- Arthralgia without erythema nodosum in 5% cases
- Bone cysts (esp digits) + swelling
Can sarcoidosis cause hepatosplenomegaly?
Yes. Rarely clinically significant. Biopsy = granulomas
Can sarcoidosis have cardiac involvement?
Rare but serious. Ventricular dysrhythmias, conduction defects and cardiomyopathy with CCF.
What Ix should be conducted in sarcoidosis? What will they show?
- Imaging: CXR, HRCT (assess diffuse parenchymal involvement)
- FBE: mild normochromic, normocytic anaemia with raised ESR.
- Biochem: hypercalcemia, hypergammaglobulinemia
- Transbronchial biopsy
- Serum ACE level: raised in 75%
- Lung functions tests: restrictive (in progressed disease) but may be normal
Rx for sarcoidosis?
Corticosteroids if disease not improving spontaneously 6/12 after Dx.
What requires mandatory treatment in sarcoidosis?
Eye involvement or persistent hypercalcaemia
What do patients with sarcoidosis generally report?
Respiratory symptoms (dyspnoea, cough, chest pain, haemoptysis)
or
Constitutional symptoms (fever, fatigue, weight loss, anorexia, night sweats).
