MS2 Sarcoidosis

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Author:
jknell
ID:
207312
Filename:
MS2 Sarcoidosis
Updated:
2013-03-14 17:29:51
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Multisystem Disease
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Description:
Sarcoidosis
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  1. Sarcoidosis
    Epidemiology
    • -onset <50 years, peak incidence is 20-40
    • -Worldwide: Northern European has highest incidence
    • -African Americans 3x > Caucasian Americans
    •      -African Americans peak later (40-50); worse prognosis
    • -Female predominance
    • -Low income/poor socioeconomic status → more severe sarcoidosis
    • -Treatment required in ~60% of patients

    • Sarcoid in children:
    • -children<adults (0.06 cases per 100,000)
    • -Present: skin lesions, uveitis, arthritis, stage 1 changes in CXR
    • -Ddx: familial juvenile systemic granulomatosis (Blau's syndrome)
  2. Sarcoidosis
    Environmental triggers (associations)
    • Exposure to irritants - wood-burning stoves, tree pollen, mold
    • Exposure to inorganic particles - insecticides
    • Association with US Navy, metalworking, firefighting
    • PCR assessment of granulomas show: mycobacterial and propiobacterial DNA and RNA
  3. Sarcoid
    pathophys principles
    Pathologic immune response triggered by exposure/inhalation of various environmental antigens
  4. Sarcoidosis
    Genetic factors
    • Familial sarcoidosis is rare
    • Greater concordance in monozygotic twins compared to dizygotic twins
    • Patient with sarcoid 5x more likely to have family member with sarcoid
    • *pattern of organ involvement might also be inherited
    • Phenotype: Class I HLA-B8 antigens associated with acute sarcoidosis
    • Susceptibility: Class II HLA antigens...
  5. Granulomas
    Pathogenesis
    • -Granulomas are centrally organized macrophages & epithelioid cells encircled by lymphocytes
    • -Granulomas confine pathogens
    • -(Macrophages + chronic cytokine stimulation → differentiation to epithelioid cells)
    • Multinucleated giant cells

    • -CD4 T cells and APC initiate and maintain granulomas
    • → Activated CD4+ cells differentiate Th1-like
    • -Secrete IL-2, IFN-γ
    • -Augment macrophage TNF-α production
  6. Granuloma formation
    • Macrophages
    • CD4+ T cells
    • Th1 cells
    • IL-2, IFN-γ
    • Granulomas of sarcoidosis
    • -tight granulomas
    • -giant cells
  7. Sarcoid
    natural history
    • Most detected incidentally on CXR
    • Systemic symptoms (fatigue, night sweats, wt loss) are common
    • 2/3 experience remission within 10yrs of Dx (50% in 3 years)
    • 1/3 have unrelenting disease
    • Recurrence is rare (<5%)
    • Mortality: <5%, typically from pulmonary fibrosis
  8. Sarcoid outcome:
    Granulomas may completely resolve BUT...

    • -Pulmonary fibrosis occurs in ~1/4 patients
    • -Shift from Th1 cells (IL-2) to Th2 (IL-4, -10, -13) maybe important to fibrosis

  9. Löfgren's syndrome
    • Acute sarcoid (~20% of cases)
    • -Fever
    • -Arthritis (ankles in men)
    • -Erythema nodosum (>women)
    • -Bilateral hilar adenopathy
    • -90% spontaneous resolution within 2 years
    • -Scandinavian, Irish, African, & Puerto Rican women
    • -Strong HLA-DQB1 association
  10. Sarcoidosis
    Dx
    • Diagnosis of exclusion
    • -clinical and radiograph findings in 90%
    • -must have histological evidence of noncaseating granulomas (absence of organisms or particles)
    • -Bx (not needed in Löfgren's syndrome)
    • -Bronchoscopy often required
    •      -Transbronchial biopsy: 85% diagnostic yield



    Bronch shows cobble stone, bumpy appearance - can be used to make dx without biopsy in 85% of cases
  11. Sarcoid
    other tests
    • ACE levels are elevated in 60% of pts with sarcoidosis
    • Granulomas make angiotensin converting enzyme
  12. Sarcoidosis
    organ involvement
  13. Sarcoid
    pulmonary symptoms, staging
    • Symptoms:
    • -Dry cough
    • -Dyspnea
    • -Chest pain/tightness and wheezing

    • Stage 1-4stage is a description, not a progression
    • 1. Bilateral hilar lymphadenopathy (no infiltrates)
    • 2. bilateral hilar lymphadenopathy (with infiltrates)
    • 3. infiltrates (no nodes)
    • 4. Nonspecfic scarring/injury (fibrotic bands, bullae, hilar retraction, bronchiectasis, diaphragmatic tenting)
  14. sarcoid stage 1

    • Stage I Lymphadenopathy:
    • DDx:
    • -Primary TB
    • -Endemic Fungal (esp.) Histoplasmosis
    • -Lymphoma
    • -Small cell lung cancer with nodal metastases
  15. Sarcoid stage 3
  16. Sarcoid stage 2: lymphadenopathy + infiltrates
  17. Sarcoid stage 4
  18. Sarcoid
    late stage (II-IV) ddx
    • Nodular pattern:
    • -Silicosis/pneumoconiosiscentrilobular and subpleural nodular
    • -miliary TB: random nodules

    • Fibrotic pattern:
    • -Usual interstitial pneumonia (UIP): base and peripheral fibrosis, honeycombing
    • -Chronic Hypersensitivity pneumonitis: mid zone fibrosis with mosaic pattern
    • -Tuberculosis (more unilateral)
  19. Sarcoid
    pulmonary physiology findings
    • 65% have abnormal PFTs
    • -majority restricted, but mixed obstructive/restrictive is common
    • -may respond to bronchodilators
    • -80% will have normalized within 2 years

    • Pulmonary hypertension relatively common:
    • -14% at rest
    • ->40% at exercise
    • -loss of pulmonary vasculature
    • -occasionally mimics small vessel disease (PAH) with granulomatous narrowing of small pulmonary arteries
  20. Sarcoid
    Cutaneous involvement

    • common: ~30%
    • May be cosmetically/emotionally significant
    • Lesions highly variable:
    • Macules, papules, plaques, single lesions or crops
    • Location: nape of neck, upper back, extremities & trunk; may appear in scars and tattoos


    • Lupus pernio:
    • -indurated, lumpy, violaceous lesions
    • -nose, cheeks, lips, ears
    • -often eroding into cartilage and bone
    • -Women>Men
    • -Associated with chronic disease & extrapulmonary disease
    • -Remission is rare
    • Erythema nodosum:
    • -~10% of sarcoidosis
    • -painful raised nodules
    • -occur in allergy and inflammatory disease
    • -lasts for weeks
    • -Bx: nonspecific septal panniculitis (not useful for dx)
  21. Sarcoidosis
    Cardiac manifestations
    • 5% have clinically apparent cardiac involvement:
    • 25% have cardiac granulmoa at autopsy
    • -Cardiomyopathy
    • -Arrhythmia: commonly bradyarrhythmias (block)

    • Screening with 12 lead ECG
    • Endomyocardial biopsy has low diagnostic value
  22. Sarcoid
    Ophthalmologic complications
    • Eye is involved in 80% of sarcoid patients:
    • ->20 ophthalmologic sx on presentation

    • Slit lamp & fundoscopic exam mandatory:
    • -multifocal chorioditis
    • -swelling of the optic nerve
    • -65% anterior uveitis → insidious cataract, glaucoma, vision loss
  23. Sarcoid
    Neurologic involvement
    • 10% of sarcoid patients have neurologic symptoms
    • 25% have evidence on autopsy

    • -CN palsy > headache > ataxia > cognitive dysfunction > weakness > seizures
    • -~13% with neuro sarcoidosis have NO OTHER organ involved
    • -CSF is non-specific
    • -MRI with gadolinium is sensitive for CNS disease
    • -Tx: aggressive corticosteroids (immunosuppressives)
  24. Sarcoidosis
    Liver and spleen
    • 10% (elevated LFTs); however usually asymptomatic
    • more common in African Americans vs. Caucasian Americans
    • Liver and spleen granuloma seen 10% of the time on CT
    • 60% with hypatic sarcoid have systemic symptoms: fever, night sweats, anorexia, wt loss
  25. Sarcoid
    Bone, joint, muscle involvement
    • Asymptomatic bone lesions are common
    • May cause pain
    • Chronic arthralgias are more common than acute arthritis
  26. Sarcoid
    Renal disease, hypercalcemia
    • Hypercalciuria is common, 40%
    • Hypercalcemia in 10%
    • Nephrolthiasis in 10%
    • *Granulomatous macrophages convert Vit D to a more active metabolite
    • Renal failure from granulomatous interstitial nephritis is rare (<1%)
  27. Sarcoid
    Medications
    • -Most pts do not require treatment; reserved for signficant symptoms
    • -Prednisone is mainstay of therapy (20-40mg/day)

    • Lack of response:
    • -irreversible fibrotic disease
    • -medication noncompliance
    • -inadequate prednisone dose

    • -Methotrexate is used as steroid sparring agent
    • -Hydroxychloroquine used for hypercalcemia, CNS and skin disease
    • -Future: TNF-α blockers (?)
  28. Sarcoid
    transplantation
    • 3% of lung transplants are for sarcoidosis
    • <1% of heart and liver transplants for sarcoidosis
    • survival rates for lung and liver are the same as non-sarcoid patients
    • Survival rates for heart transplant are better for short and intermediate-term survival
    • **Sarcoidosis can recur in lung allograft (but does not affect survival)

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