Sarcoidosis Flashcards
sarcoidosis
-Multisystem granulomatous disorder
-unknown cause- autonomic
-Noncaseating granulomas in involved organs
-90% have lung granulomas (appear as defense mechanism) -> immune systems way of addressing things that shouldnt be there
-inflammatory cells are trying to wall something off
-can involve any organ systems- but usually dx with lung imaging
-Prevalence: estimated at 10 to 20 per 100,000
-Incidence is highest in North American blacks (female>male) and northern European whites
-blacks- have more extrapulmonary disease and generally more severe -> whites primarily asymptomatic
-Onset: 20-60 years of age (younger)
-? Etiology
-Overall mortality 1-5 %
sarcoidosis: pathophysiology
-Initial lesion within the pulmonary system is CD4+ T cell alveolitis -> then development of noncaseating granulomas
- CD4+ T cell alveolitis: inflmmation of alveoli due to WBC accumulation
-Granulomas have tightly packed central area composed of macrophages, epithelioid cells, multinucleated giant cells surrounded by lymphocytes, monocytes, mast cells, and fibroblasts (inflammatory cells)
-Other tissues commonly involved: 30% of pts:
-Skin
-Eyes
-Lymph nodes- very common and usually how its dx
caseating vs noncaseating
-caseating: Necrosis; “cheesy” appearance
-noncaseating: center of granuloma does not have necrosis -> center is clustered macrophages
are granulomas specific for sarcoidosis
-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
sarcoidosis symptoms
-50% asymptomatic and seek medical attention after abnormal findings on CXR
-Typically, bilateral hilar and right paratracheal lymphadenopathy
-Malaise, fever, and dyspnea of insidious onset
-Cough, chest pain
-Symptoms referable to the skin, eyes, peripheral nerves, liver, kidney, or heart may also cause the patient to seek care.
sarcoidosis: physical findings
-Erythema nodosum- GOOD SIGN! -> remits within 6-8 weeks
-Parotid gland enlargement
-Hepatosplenomegaly
-Lymphadenopathy
-Crackles are (uncommon)- can cause interstitial lung disease
-+/- wheezing- very high association with asthma
other organ physical exam findings
-ocular:
-Found in 22-50%
Granulomatous uveitis (anterior (80%)>posterior)
Uveitis – acute or chronic
Kerato conjunctivitis, retinal hemorrhages, band keratopathy
-peripheral lymphadenopathy*:
-Found in 75%
Cervical, axillary, epitrochlear and inguinal
-rheumatologic:
-25-39% joint involvement; 2 forms – acute and chronic (<6 months).
-May precede other manifestations by years
-Knees > ankles > elbows > wrists > small joints of hands (typically 2-6 joints).
-Periarticular swelling > effusions
-Synovial fluid often non-inflammatory
-Tenosynovitis and heel pain may occur
-Chronic:
-knees, PIPs (associated with chronic cutaneous sarcoid lesions)
sarcoidosis diff dx
-Infections:
-Mycobacteria
-Coccidioidomycosis
-Histoplasmosis
-Brucellosis
-Tularemia
-Syphilis
-Toxoplasmosis
-Malignancy: Lymphoma
-Autoimmune: Wegener’s, Churg-Strauss, Primary biliary cirrhosis
-Other:
-Hypersensitivity pneumonitis
-Exposures: Talc, Beryllium
basic sarcoidosis work up
-comprehensive eval in all pts suspected
-History/PE
-CBC, BUN, Cr, LFTs, electrolytes, Ca.
-Urinalysis
-ACE level*- nonspecific
-CXR
-PFTs (spirometry, volumes, diffusion measurements)- usually normal limits but can show asthma
-Histology
-electrocardiogram
-ophthalmologic exam
-tuberculin skin test
hilar adenopathy
-mc sign
-typically found for other reasons
-suspect lymphoma vs sarcoidosis
-need to bx
sarcoidosis common lab abnormalities
-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%)
sarcoidosis dx
-3 elements
-1) Compatible clinical and radiographic manifestations
-2) Exclusion of other diseases that present similarly
-Other granulomatous diseases and lymphoma
-3) Histopathologic detection of noncaseating granulomas
-Bx of easily accessible sites (eg, palpable lymph nodes, skin lésions, or salivary glands)
-Transbronchial lung biopsy (bronchoscopy) has a high yield (75–90%)***
-If no accessible extrapulmonary sites
where to biopsy?
-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
radiographic staging with chest x-ray
-stage 0- normal- excellent prognosis
-stage 1- bilateral hilar adenopathy +/- paratracheal adenopathy - excellent
-stage 2- bilateral hilar adenopathy + pulmonary infiltrates - good
-stage 3- pulmonary infiltrates + NO hilar adenopathy - fair to good
-stage 4- pulmonary fibrosis- poor to fair
stage 1 sarcoidosis
- bilateral hilar adenopathy +/- paratracheal adenopathy
-50% of pts
-no respiratory symptoms
-60-80% spontaneous remission
