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Flashcards in Granulomatous lung disease Deck (10)
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Granuloma formation

-Compact aggregates of epithelioid cells to confine pathogens
-Form in response to TNFa causing macrophages to differentiate into epithelioid cells
-Epithelioid cells then fuse to form giant cells which may have foreign body inclusions
-Some granulomas will undergo necrosis (TB-> caseous necrosis) and some do not
-Granulomas form from Th1 responses, whereas Th2 responses cause fibrosis


Causes of lung granuloma diseases

-Inhaled antigens: coccidiomycosis, TB, beryllium (non necrotizing)
-Drugs: methotrexate, dilantin, talc, bleomycin
-Sarcoidosis (non necrotizing, indistinguishable from beryllium), other autoimmune


Hx for granulomatous lung disease

-Travel: bakersfield associated w/ coccidiomycosis
-Occupation: farms usually associated w/ fungi and/or hypersensitivity
-Pigeons: hypersensitivity



-Multisystem granulomatous disease w/ no known etiology
-Lofgren syndrome: arthritis, erythema nodosum, bilateral hilar adenopathy
-Heerfordt's syndrome: uveitis, parotid gland inflammation, +/- facial nerve involvement
-ACE levels may be elevated, there will be hypercalcuria and hypercalcemia


Radiographic stages of sarcoidosis

-Stage 1: bilateral hilar adenopathy, mediastinal adenopathy
-Stage 2: adenopathy and parenchymal lung disease
-Stage 3: parenchymal lung disease only
-Stage 4: fibrosis and cystic changes
-Bilateral hilar adenopathy most common finding
-Upper and mid lung shows coarse reticular and cystic changes and volume loss, upward displacement of the hila and main bronchi resulting from fibrosis


Rx of sarcoidosis

-Steroids and immunosuppression
-Common spontaneous remission
-Overall good prognosis
-Relapses occur


Hypersensitivity pneumonitis (HP)

-Acute: responds to steroids
-Sx include cough, fever, chest tightness, malaise
-Radiography: diffuse nodular changes
-Chronic: progressive dyspnea and weight loss
-Radiography: upper lobe fibrosis, reticular infiltrates, honeycombing


Dx of HP

-Immunoprecipitation of IgG toward the Ag
-Histopathology, radiography
-Exposure, occupation, PIGEONS


Chronic beryllium disease (berylliosis)

-Disease is indistinguishable from sarcoidosis, thus occupational Hx is key
-Dx made w/ positive beryllium lymphocyte proliferation test
-Steroids are the Rx
-Seen in miners, aerospace engineers, manufacturers of light bulbs


ANCA-associated granulomatous vasculitis

-Granulomatosis polyangiitis (GPA AKA wegener's): granlumatous inflammation w/ no etiology
-Involves URT (sinusitis, saddle nose, epistaxis), lungs (alveolar hemorrhage and hemoptysis), and kidneys (GN and hematuria)
-Dx requires biopsy, c-ANCA should be present but negative result doesn't rule out GPA
-Immunosuppression and steroids are Rx