Sarcoidosis

Question 1

What is sarcoidosis and how does it usually present clinically? (signs rather than symptoms)

Answer 1

Multisystem granulomatous disorder, commonly affecting young adults.

Usually presents with bilateral hilar lymphadenopathy, pulmonary infiltration, hepato-/spleno-megaly, and skin or eye lesions.

Question 2

Which form of poisoning can mimic sarcoidosis?

Answer 2

Beryllium poisoning

Question 3

At what age is the peak incidence of sarcoidosis?

Answer 3

30s and 40s

Question 4

Does sarcoidosis have a genetic association?

Answer 4

No known relation to HLA BUT 1st degree relatives (particularly Caucasians) have increased risk of developing sarcoidosis.

Question 5

What is the structure of the typical sarcoid granuloma?

Answer 5

Typical sarcoid granuloma: non-caseating, focal accumulation of epithelioid cells, macrophages and lymphocytes (mainly T cells)

Question 6

What are the typical findings of bronchoalveolar lavage?

Answer 6

-Big increase in number of cells - Increase in lymphocytes (esp CD4 T cells) -Increase in macrophages (but reduced total %)

Question 7

What are the common presentations of sarcoidosis?

Answer 7

1) Resp symptoms or abnormalities on CXR (50%)
2) Fatigue/weight loss (5%)
3) Peripheral lymphadenopathy (5%)
4) Fever (4%)

Question 8

What are the stages of sarcoidosis pulmonary involvement?

Answer 8

Stage 1: bilateral hilar lymphadenopathy (BHL) alone

Stage 2: BHL with pulmonary infiltrates

Stage 3: pulmonary infiltrates w/o BHL

Stage 4: fibrosis

Question 9

What are the DDx of bilateral hilar lymphadenopathy?

Answer 9

• 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

Question 10

What is the common effect of pulmonary infiltrates?

Answer 10

Mostly asymptomatic. Pulmonary infiltrate + NORMAL RFTs highly suggestive of sarcoidosis.

Question 11

How does the CXR appear throughout the course of sarcoidosis?

Answer 11

Initially = mottling in mid zones. Evolves to generalised fine nodular shadows. Eventually widespread linear shadows (fibrosis).

Question 12

What are the DDx of pulmonary infiltration?

Answer 12

• TB
• Pneumoconiosis
• Idiopathic pulmonary fibrosis
• Alveolar cell carcinoma

Question 13

What are the most common extrapulmonary manifestations of sarcoidosis?

Answer 13

Skin

Ocular

Question 14

What are the skin lesions caused by sarcoidosis?

Answer 14

-Erythema nodosum: sarcoidosis most common cause -Lupus pernio: bluish red infiltrate or swelling esp of nose or ears -Skin nodules

Question 15

What are the manifestations of ocular sarcoidosis?

Answer 15

-Anterior uveitis: misting of vision, painful red eye -Posterior uveitis: progressive loss of vision

Question 16

What is uveoparotid fever?

Answer 16

Syndrome of bilateral uveitis + parotid gland enlargement together with occasional development of facial nerve palsy; sometimes seen with sarcoidosis.

Question 17

What are the metabolic complications of sarcoidosis? Why?

Answer 17

-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)

Question 18

What are the bone and joint involvements associated with sarcoidosis?

Answer 18

• Arthralgia without erythema nodosum in 5% cases
• Bone cysts (esp digits) + swelling

Question 19

Can sarcoidosis cause hepatosplenomegaly?

Answer 19

Yes. Rarely clinically significant. Biopsy = granulomas

Question 20

Can sarcoidosis have cardiac involvement?

Answer 20

Rare but serious. Ventricular dysrhythmias, conduction defects and cardiomyopathy with CCF.

Question 21

What Ix should be conducted in sarcoidosis? What will they show?

Answer 21

• 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

Question 22

Rx for sarcoidosis?

Answer 22

Corticosteroids if disease not improving spontaneously 6/12 after Dx.

Question 23

What requires mandatory treatment in sarcoidosis?

Answer 23

Eye involvement or persistent hypercalcaemia

Question 24

What do patients with sarcoidosis generally report?

Answer 24

Respiratory symptoms (dyspnoea, cough, chest pain, haemoptysis)

or

Constitutional symptoms (fever, fatigue, weight loss, anorexia, night sweats).

Question 25

What are the extrapulmonary signs of sarcoidosis?

Question 26

What is lupus pernio?

Answer 26

Violaceous patches on the face, especially the nose, fingers or toes.