Sarcoidosis Lecture Powerpoint Flashcards
Sarcoidosis
Chronic multisystem clinically heterogenous (not everyone presents the same, variable presenting) inflammatory granulomatous disorder of unknown etiology (possibly autoimmune, genetic, or environmental) frequently affecting the lungs but also skin, liver, heart, eyes, noninfectious but is inflammatory, commonly affecting young aduults and middle age adults 20-40, 2nd peak 65 years old in females
Granuloma
Mass or nodule of chronically inflamed tissue, product of overactive immune response, physiologic product of immune response that acts as a shield to protect against pathogens - typically beginning as microscopic but may coalesce, chronic inflammation or recurring leads to scarring of tissue, in lungs this leads to pulmonary fibrosis and a restrictive lung disease
Sarcoidosis presents with ___ granulomas, while something infectious such as Tb has ___ granulomas
Non-caseating (cells not necrotic)
Caseating (necrotic cheese like center)
Where do sarcoidosis granulomas primarily occur? (7)
- lungs >90%
- lymph nodes
- eye
- liver
- skin
- spleen
- rheumatologic manifestations in joints (15-25%)
Sarcoidosis epidemiology
Global disease with variable diagnostic criteria may appear at any age but peaks at 20-40 and then 65 in females, more common in african americans and greater severity, some genetic links to which organs are affected
Individuals at risk for being more likely to get sarcoidosis (5)
- healthcare workers
- nonsmokers
- exposure to agricultural dust, insectisides, pesticides
- lumbar workers
- firefighters
Sarcoidosis etiology
exact unknown, thought to be due to multiple causative agents, abnormal immune response to an undetermined toxin, drug, or pathogen, as well as genetic factors
Sarcoidosis pathophysiology (4 major events)
-interplay of many different cells, cytokines and other inflammatory mediators in susceptible hosts
1) exposure to one or more unknown antigens
2) activation of APC’s (macrophages or dendritic cells)
3) T cell response in effort to eliminate the antigen (release f inflammatory mediators)
4) granuloma formation (nonspecific exaggerated response to shield tissue from pathogen affecting organ function, focal accumulation of epithelioid cells, macrophages, lymphocytes (CD4 and 8))
-most granulomas resolve spontaneously however repeated cycles may lead to scarring in up to 25%
Serum findings of sarcoidosis pateints
- decreased T lymphocytes because sequestered to granulomas
- relative slight increase in B cells in serum relative to other immune type cells
Sarcoidosis symptoms (is it symptomatic all the time? Pulmonary/lymph node findings, nonspecific findings, eye findings, skin findings, myocardial findings, GI findings, Neurological findings, rheumatologic findings)
- often asymptomatic up to 50% incidental CXR finding but can cause acute organ failure in severe
- hilar lymphadenopathy, dry nonproductive cough, SOB, chest discomfort
- nonspecific fatigue, weight loss, anorexia, malaise, night sweats, fever
- conjunctival nodules, uveitis, blurred vision, ocular discomfort
- erythema nodosoum on knees, nodules
- arrhythmias, cardiomyopathy, pericardial effusion
- abdominal fullness
- cranial nerve palsies, meningeal involvement
- acute can cause symmetric polyarthritis** swollen and painful joints possibly bones often working distal to proximal**, bone swelling and stiffness and dactylitis (swelling of digits)
Physical exam to assess sarcoidosis (5)
- pulmonary function tests to assess restrictive lung dz
- listen to lung for diminished breath sounds indicating fibrosis
- CXR
- skin lesions
- eye lesions
CXR finding seen in sarcoidosis patients
Bilateral symmetric hilar lymphadenopathy with reticulonodular pattern, see nodular pulmonary infiltrates in later stage
CXR classification system for sarcoidosis
Stage 0 -normal
Stage 1 - bilateral hilar lymphadenopathy
Stage 2 - bilateral hilar lymphadenopathy and reticulonodular infiltrates in parenchyma
3 - bilateral pulmonary infiltrates lack of hilar adenopathy
4 - fibrosis scarring, commonly with upward hilar retraction
Restrictive lung disease pattern on pulmonary function test in sarcoidosis
Normal level of flow (vertical) but decreased volume, seen in late stage sarcoidosis
Erythema nodosum
Inflammatory disorder charaacterized by tender, ereythematous, nodular eruptions commonly on the extensor surfaces of lower legs associated with sarcoidosis
Lupus pernio
Chronically raised, indurated lesions commonly on face associated with sarcoidosis
Acute sarcoid arthritis presentation
-typically occurs in 1st 6 months of onset, commonly presents as febrile arhthropathy, begins distal and moves proximal up the body, pain, stiffness, usually symmetric polyarticular pattern
Chronic sarcoid arthritis presentation
-symptoms >6 months following onset of sarcoidosis, less common, less severe, often affects 2-3 joints, , monoarthritis may occur
Lofren’s syndrome
Acute polyarthritis, symmetric bilateral hilar lymphadenopathy, erhythema nodosum, form of sarcoid with good prognosis as often self limited and has a 90% remission rate easily treated with NSAIDS
Sarcoidosis diagnosis (4)
- Diagnosis of exclusion
- clinical presentation consistent with disease
- histopathologic evidence of noncaseating granuloma following biopsy via bronchoscopy if in lungs
- exclude other conditions
Labs and imaging for sarcoidosis (7)
- CXR
- PFT
- CBC
- Serum ACE
- 24 hr urine for hypercalciuria
- bronchial biopsy
- synovial fluid biopsy
Kveim siltzbach skin test
Fairly sensitive and highly specific for sarcoidosis, similar to intradermal PPD test but NOT approved in US and not typically recommended
Differential diagnosis of sarcoidosis (4)
- infectious granulomas such as TB, fungus
- lymphoma
- foreign body reaction
- gout
Only __ type of sarcoidosis can be treated
Pulmonary
Pulmonary sarcoidosis treatment options (2)
- prednisone
- supplemental ACTH
Sarcoidosis prognosis
-majority resolve within 2 years, no evidence of lung parenchyma involvement generally requires no treatment, poor prognosis if >40 at onset, cardiac involvement, etc
Sarcoidosis general treatments (4)
- oral corticosteroids (or topical depending on manifestation)
- NSAIDS
- ACTH analogue
- Methotrexate, chloroquine, cyclosporine, TNF inhibitors infliximab and adalimumab
Sarcoid arthritis treatment options (3)
- low dose systemic steroid
- NSAIDS
- methotrexate
