Sarcoidosis

Question 1

definition of sarcoidosis

Answer 1

mutlisystem inflammatory disaease with protean manifestations. large spectrom of organ system invovlement with variable course and prognsis and unclear indcations for treatment.

Question 2

etiology of sarcoidosis

Answer 2

genetic influence (80% among MZ twins) combined with environmental factors (smoking actually protective).BTNL2 gene may increase risk. HL class II type may be associated with disease development, phenotype, and environmental interactions

Question 3

stages of immunopathogenesis of sarcoidosis

Answer 3

1. initiation: antigen stimulates T cell activation. TNF probably plays a role
2. granuloma formation: macrophages differentiate into the core, T cells are recruited, fiboblast and collagen also encase cells.
3. disease either goes into remission or becomes chronic.

Question 4

3 features for sarcoidosis diagnosis

Answer 4

1. presence of clinical and radiographic picture consistent with sarcoidosis
2. non-caseating granulomas present
3. exclusion of other causes

Question 5

features of pulmonary sarcoidosis

Answer 5

dyspnea, dry cough, chest pain, fatigue. exam seems normal. on function testing, you will see more restriction than obstruction and a decrease in DLCO. see adenopathy.
may also see pulmonary hypertension and hypoxia

Question 6

sarcoidosis staging based on X-ray

Answer 6

O- IV
0: no lung involvement
1: bilateral lympadenopathy (LAD)
II: LAD + infiltrates
III: parenchymal disease but no LAD
IV: pulmonary fibrosis

Question 7

How are stages useful for sarcoidosis?

Answer 7

Stage 0 and 1 most likely to have sponateous remission; III and IV are least likely. stages don’t predict flares and are not progressive

Question 8

sarcoidosis staging based on CT

Answer 8

I, II: nodes
III: confluent nodules
IV: bronchiectasis, fibrosis, and air trapping
asymmetric LAD, cavitated/spiculated nodules, and pleural effusions all unusual

Question 9

Lofgren’s syndrome

Answer 9

fever, erythema nodosum, polyarthritis, bilateral hilar adenopathy. form of acute sarcoidosis. increases chances of spontaneous remission

Question 10

Warning systems for sarcoidosis

Answer 10

eye, heart, kidney, neuro

Question 11

sarcoidosis of skin

Answer 11

many manifestation. erythema nodosum is most common: painful. no granulomas on biopsy
lupus pernio is another form and requires immediate treatment

Question 12

eye involvement in sarcoidosis

Answer 12

anterior uveitis more than posterior. 10% go completely blind. consider cataracts too

Question 13

Cardiac involvement in sarcoidosis

Answer 13

2nd leading cause of death, including sudden cardiac death. many ppl have baseline cardiac fivindings. but this is hard to diagnose (look at interventricular septum)

Question 14

Kidney involvement in sarcoidosis

Answer 14

hypercalcemia frequent complication

Question 15

neurosarcoid

Answer 15

uncommon. can invovle brain base, cranial nerves (esp VII) hypothalamus, pituitary. may be central or peripheral.

Question 16

What is signficicance to CD4: CD8 ratio?

Answer 16

if BAL CD4> CD8, probably sarcoid. specific but not sensitive.

Question 17

What other diseases present like sarcoidosis?

Answer 17

mycobacteria, fungus, esp. histoplasmosis, hypersensitivity pneumonitis, beryllium and other heavy metal exposure, lymphoma, Wegener’s and Churg-Strauss

Question 18

prognostic predictors

Answer 18

chest x-ray. African americans have worse prognosis. E nodosa and lofgrens and arthritis, isolated uveitis, and isolated CNVII all have good prognosis. worse prognosis: age over 40, cardiac problems, hypercalcemia, chronic uveitis, lupus pernio, nephrocacinosis, neurosarcoidosis, progressive pulmonary fibrosis, pulmonary HTN.

Question 19

when is sarcoidosis treatment warranted?

Answer 19

III and IV disease. sometimes for stage II. indicated with problems iwth lung function, cardiac/neuro involvement, uveitis that doesn’t respond to steroids, hypercalcemia.
relapse is 75% if treatment is stopped after starting it.

Question 20

treatment for acute sarcoidosis

Answer 20

predinso 20-40 mg/day; more for cardiac/neuro attacks. consider alternate agent if not better after 3 months. taper dose after 3 months but expect to keep pt on meds for at LEAST a year. steroids treat symptoms but don’t change long term progression

Question 21

Non-steroidal sacoidosis treatment

Answer 21

methotrexate, esp for lung, CNS, eye, skin
anti TNF agents (infliximab): last line agent
azathioprine for hepatic/ocular
leflunomide for lung