Sarcoidosis Flashcards
Define sarcoidosis
- Abnormal collections of inflammatory cells (granulomas) that can form as nodules in multiple organs
- Intrathoracic lymphadenopathy
Where does sarcoidosis form?
Most often located in the lungs (90%) or its associated lymph nodes, but any organ can be affected (MSK, liver, eye, skin, CNS, kidneys, spleen, heart)
What is sarcoidosis characterized by?
non-caseating granulomas
What demographics are at the highest risk for sarcoidosis?
- Incidence highest in North American blacks and Northern European whites
- Among blacks, women > men
- Onset of disease 30-40 years
- Genetic predisposition
A. Risk 5x > if + FH
What is the pathophys of sarcoidosis?
- Unknown antigen triggers a cell-mediated immune response
A. Accumulation of T cells and macrophages
B. Release of cytokines
C. Inflammatory process leads to formation of noncaseating granulomas
-Hallmark of sarcoidosis
-Most commonly in the lungs and lymph nodes
What are the clinical presentation sxs for sarcoidosis?
- Asymptomatic
- Fatigue
- Fevers
- Arthralgias
- Wrists, ankles, elbows - DOE
- Cough
- Mild CP
What other sxs may be present in sarcoidosis?
- Skin involvement
- Rash
- Erythema nodosum
- Iritis
- Peripheral neuropathy
- Cardiomyopathy
- Seizures
- Headache
What is Löfgren Syndrome? How is it treated? Who gets it?
- Most common in Scandinavian & Irish females
- Often self-limiting
- NSAIDs help
- Triad
- Acute polyarthritis
- Erythema nodosum
- Hilar adenopathy
What is Blau syndrome? How is it treated? Who gets it?
- Inherited autosomal dominant type in children
- Presents before the age of 4 yr
- Self-limited
- NSAIDs help
- Triad
- Arthritis
- Rash
- Uveitis
Is sarcoidosis most similar to a restrictive or obstructive lung disease?
PFTs demonstrate restrictive pattern
↓ TLC
Normal FEV1/FVC
What happens to ACE levels in sarcoidosis?
Angiotensin converting enzyme (ACE) levels increased in 40-80% of time
What test is performed in sarcoidosis?
↑ Erythrocyte Sedimentation Rate (ESR)
What are the cxr results in sarcoidosis?
- Bilateral hilar adenopathy
2. Diffuse reticular infiltrates
Why is a biopsy performed in sarcoidosis? What are the results?
- Biopsy of lymph nodes, skin lesions, lung biopsy
A. Provides histologic diagnosis
B. Transbronchial or fine needle lung Bx confirms Dx of noncaseating granulomas
What is the treatment for sarcoidosis?
Oral corticosteroids
0.5-1.0 mg/kg/d
Taper slowly over 6 mo
