Sarcoidosis COPY

Question 1

sarcoidosis

Answer 1

Definition: Multisystem granulomatous disorder
- 90% have lung granulomas
- ? ethiology
Typically 20-60 yrs old

Question 2

sarcoidosis: pathophysiology

Answer 2

Initial lesion within pulmonary system is CD4+ T Cell alveolitis which is followed by noncaseating granulomas
Granulomas are nonspecific and can be found in many diseases

Question 3

-Other tissues commonly involved: _____% of pts

Answer 3

: 30% of pts:
-Skin
-Eyes
-Lymph nodes- very common and usually how its dx

Question 4

caseating vs noncaseating

Answer 4

Caseating: Necrosis; “cheesy” appearance
- TB

-noncaseating: center of granuloma does not have necrosis -> center is clustered macrophages

Question 5

are granulomas specific for sarcoidosis

Answer 5

-NO
-In liver granulomas are nonspecific
-In skin granulomas can represent a nonspecific reaction to foreign body
-Granulomas are also caused by:
-Histoplasmosis
-Tuberculosis
-Cancer
-Lymphoma- main thing you want to exclude -> bx to exclude this

Question 6

sarcoidosis symptoms

Answer 6

50% = asymptomatic + its an incidental finding

Sx: FEVER*
- Malaise, insidious dyspnea, cough, chest pain
- Fever is uncommon in other ILDs
-other sx referable to the skin, eyes, peripheral nerves, liver, kidney, or heart may also cause the patient to seek care.

Question 7

sarcoidosis: physical findings

Answer 7

-Erythema nodosum- GOOD SIGN! -> remits within 6-8 weeks
-Parotid gland enlargement
-Hepatosplenomegaly
-Lymphadenopathy
-+/- wheezing- very high association with ASTHMA

Question 8

other organ physical exam findings

Answer 8

ocular: uveitis, conjunctivitis
peripheral lymphadenopathy
joint involvement

Question 9

basic sarcoidosis work up

Answer 9

-comprehensive eval in all pts suspected
-History/PE
-CBC, BUN, Cr, LFTs, electrolytes, Ca.
-Urinalysis
-ACE levels high*- nonspecific
PFTs (spirometry, volumes, diffusion measurements)- usually normal limits but can show asthma
-Histology
-electrocardiogram
-ophthalmologic exam
-tuberculin skin test

Question 10

hilar adenopathy

Answer 10

-mc sign
-typically found for other reasons
-suspect lymphoma vs sarcoidosis
-need to bx

Question 11

sarcoidosis common lab abnormalities

Answer 11

-Leukopenia
-Elevated ESR
-High alkaline phosphatase
-Angiotensin-converting enzyme (ACE) levels elevated in 75% with active disease
-Neither sensitive nor specific enough to have diagnostic significance
-Hypercalcemia (5%)- uncommon
-Hypercalciuria (20%)

Question 12

sarcoidosis dx

Answer 12

Diagnosis is made with Three Essential Elements
1) Clinical and radiographic manifestations
2) Exclusion of other granulomatous diseases
3) Histopathologic detection of noncaseating granulomas
- bx with Transbronchial Lung Biopsy (preferred)
-Bx of easily accessible nodes (eg, palpable lymph nodes, skin lésions, or salivary glands)

Question 13

where to biopsy?

Answer 13

-Transbronchial lung biopsy:
-Recommended procedure in most cases
-allows you to do 2 types of bx-> lung tissue and mediastinal and hilar lymph nodes
-Diagnostic yield 60-90%
-4-5 lung biopsies
-Low risk
-EBUS: Hilar nodes bx - ultrasound guidance
-Mediastinoscopy and VATS: bx under mediastinum -> more invasive
-Lymph nodes, when palpable

Question 14

?

Answer 14

Typical presentation - stage 1; no sx
Enlarged hilar adenopathy -> concern for lymphoma and sarcoidosis
- need to bx

Question 15

stage 1 sarcoidosis

Answer 15

• bilateral hilar adenopathy +/- paratracheal adenopathy
  -50% of pts
  -no respiratory symptoms
  -60-80% spontaneous remission

Question 16

stage 2 sarcoidosis

Answer 16

-bilateral adenopathy + pulmonary inflitrates
-25% of pts
-symptoms:
-fever
-weight loss
-dyspnea
-50-60% spontaneous remission

Question 17

stage 3 sarcoidosis

Answer 17

• just pulmonary inflitrates
  -15% of pts
  -significant respiratory impairment
  -<30% spontaneous remission
  -adenopathy may be less at this point

Question 18

stage 4 sarcoidosis

Answer 18

• pulmonary fibrosis - irreversible
  -5% of pts
  -chronic respiratory impairment
  -high mortality rate

Question 19

prognostic factors

Answer 19

-E. nodosum and acute inflammatory manifestation result in high rate of spontaneous remission! (>80%)

Adverse prognostic factors:
-Lupus pernio

Question 20

treatment

Answer 20

-60% experience spontaneous resolution (83% with acute) -> dont treat just follow PFTs, eye exams etc.

-An additional 10-20% have resolution with STEROIDs
-Major goal: prevent FIBROSIS
-first line tx: steroids help prevent fibrosis
-give steroids if they have SX or ocular, serious respiratory, cardiac, neurologic impairment; hypercalcemia

Question 21

systemic treatment? + what is tx regimen

Answer 21

PREDNISONE 20-40 mg daily Max, 60 mg
-For 8-12 weeks then taper down to keep patient on 5-10 mg and continue for 1 year

Question 22

prognosis

Answer 22

-Best: hilar adenopathy alone
-Worse: fibrotic involvement of lung parenchyma
-20% suffer irreversible progressive lung impairment
-Death from pulmonary insufficiency 5%
-Erythema nodosum portends good outcome
-Pretibial red or violet subcutaneous nodules