L30 Diffuse Parenchymal lung disease (part II)

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L30 Diffuse Parenchymal lung disease (part II)
2013-02-20 19:51:36
Pulmonary II

Diffuse parenchymal lung disease, part 2
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  1. Autoimmune-associated parenchymal lung disease
    • Rheumatoid Arthritis
    • Systemic Lupus Erythematosis
    • Systemic scleraderma
    • Polymyositis-dermatomyositis (Anti-synthetase syndrome)
    • Sjögren's syndrome
    • Wegner's granulomatosis
    • Goodpasture's sysndrome
  2. Rheumatoid Arthritis
    • Can present with:
    • -Effusions
    • -Nodules
    • -Rounded atelectasis
    • -Bronchiolitis
    • -Bronchiectasis
    • - Interstitial fibrosis (although, usually mild)

    Pts often on Methotrexate for RA, some data shows MTX can cause interstitial fibrosis
  3. SLE
    • -Acute pneumonitis
    • -Interstitial fibrosis
    • -Serositis (fluid in the pleural space or pericardium)
    • --> pleurisy or pericarditis
    • -Chest pain
    • "shrinking lung syndrome" = progressive decrease in lung volume; CT shows lung pulling away from pleura (swirling star appearance)
  4. Systemic sclerosis
    aka Scleraderma
    • Severe fibrosis
    • Pulmonary arterial hypertension
  5. Polymyositis-dermatomyositis
    • interstitial fibrosis
    • Bronhiolitis obliterans with organizing pneumonia (BOOP)
    • Respiratory muscle weakness--> respiratory distress/failure
    • Repeated aspiration events from dysphagia
  6. Sjögren's syndrome
    • Interstitial fibrosis
    • Lymphocytic infiltration of alveoli
    • -lymphocytic interstitial pneumonia (LIP)
    • -Lymphoma
  7. Wegener's Granulomatosis
    • *We"C"ner's granulomatosis
    • -c-ANCA
    • -C pattern: upper respiratory, lungs, kidneys
    • --Also can cause ocular sclerosis, cutaneous vasculitis, neuropathy, arthritis...
    • -Anti-"C"rine protease (PR3) - anti-serine protease

    • -Small vessel vasculitis, necrotizing granulomatous disease...
    • Renal: focal glomerulonephritis
    • Pulmonary disease:
    • -Necrotizing nasal granlumas (saddle nose deformity, perforated septum)
    • -Subglottic stenosis
    • -Nodules, infiltrates, cavities:

    • c-ANCA
    • -Antibody levels correlate with disease activity
    • -c: cytoplasmic

    • Course:
    • -high morbidity/mortality: 90% mortality at 2 years if not treated
    • -respiratory failure, pumonary hemorrhage, infections, renal failure, DVT, PE, hearing loss

    • Tx: Immunosuppression is mainstay of therapy
    • -Steroids, cyclophosphamide, methotrexate, azathioprine, etanercept
    • -TMP/SMZ (anti-Staph med)
  8. Goodpasture's syndrome

    • -Pulmonary hemorrhage (leading to respiratory failure and post-inflammatory fibrosis)
    • -Glomerulonephritis:
    • -Anti-type IV collagen antibodies

    • Presentation:
    • -hematuria
    • -hemoptysis
    • -classically in young, adult males
    • Tx: immunosuppression, plasmapheresis
  9. Idiopathic Interstitial Lung Diseases (ILD)
    • Sarcoidosis
    • Idiopathic pulmonary fibrosis (IPF)
    • Nonspecific interstitial pneumonitis (NSIP)
    • Cryptogenic organizing pneumonia (COP)
    • Desquamative interstitial pneumonitis (DIP)
    • Acute interstitial pneumonia (AIP)
    • Respiratory bronchiolitis interstitial lung disease (RBILD)
  10. Sarcoidosis
    Demographics, etiology, histopathology
    • -Idiopathic granulomatous inflammation
    • -Organs: lungs, lymph nodes, eyes, skin, heart, joints, bone, kidneys, brain, liver...

    • Demographics:
    • -onset: 20-40 year olds
    • -♀>♂
    • -African Americans, Northern European whites
    • -Sarcoid mimic in women who are post breast CA chemotherapy

    Etiology: unknown; conflicting data

    • Histopathology:
    • -Noncaseating granulmoas
    • -Absence of organisms, foreign bodies
    • -Dx of exclusion
  11. Sarcoidosis
    Staging, clinical/lab features, treatment
    • Staging: based on CXR (or CT sometimes)
    • -Stage 0: normal CxR
    • -Stage I: lymphadenopathy ONLY
    • -Stage II: lymphadenopathy AND parenchymal disease
    • -Stage III: Parenchymal disease ONLY (...idea being that LN has burned out)
    • -Stage IV: extensive destruction and architectural distortion

    • Features:
    • -Accelerated 1,25 vit D formation leads to increased absorption (often see calciuria, rarely see hypercalcemia)
    • -Angiotensin Converting Enzyme (ACE): can be elevated (useless test)
    • -CD4+ predominance in airways and granulmoas (measure CD4+:CD8+ ratio from BAL) --nonspecific
    • ----High CD8:CD4 is suggestive of hypersensitivity pneumonitis
    • -Löfgren's syndrome: hylar lymphadenopathy, erythema nodosum, fever, arthralgias

    • Clinical:
    • -Variable presentation
    • -PFTs may show restriction and/or obstruction
    • -often spontaneous remission (lower stages don't often require treatment; best prognosis for remission)
    • -Can progress with significant morbidity/mortality (hard to predict)

    • Tx:
    • -Corticosteroids are mainstay
    • -Steroid-sparing agents (to minimize side effects of steroids): Methotrexate, Azathioprine, Mycophenolate mofetil
    • -New therapyies being explored (Inflizimab, etanercept, ustekinumab, golimumab)
  12. Stage II sarcoidosis: lymphadenopathy and parenchymal disease
  13. Stage IV sarcoidosis: extensive destruction and architectural distortion

    -Corina and diaphragm are higher than usual due to fibrosis/volume loss
  14. Idiopathic pulmonary fibrosis
    Dx: clinical +/- pathology

    • Presentation:
    • -Dry cough, insideous exertional dyspnea
    • -Dry "velcro" crackles on exam
    • ->50yrs old
    • -slight male>female
    • -military history (?)
    • -more common in smokers or former smokers

    • Imaging:
    • -reticulonodular pattern
    • -subpleural honeycombing
    • -traction bronchiectasis (due to fibrosis, different from CF bronchiectasis)
    • -basilar predominance
    • -"ground glass" (sometimes)

    • Pathology:
    • -Usual interstitial pneumonia (UIP) pattern: =interstitial fibrosis
    • =fibroblastic foci
    • =paucity of active alveolitis or bronchiolitis
    • =absence of granulomas

    • Clinical course:
    • -Variable
    • -may be complicated by PAH, cor pulmonale, clubbing, exertional hypoxemia, syncope, exacerbations
    • -Progressive tend to be fatal quickly
    • -stable tends to be stable

    • Tx: no proven therapy
    • -often employ steroids (but seem to make pts worse more often then not)
    • -Pirfenidone -fibroblast inhibitor: (not available in US); shows mild survival benefit
  15. Non-specific Interstitial Pneumonia
    • Presentation:
    • -younger age, more acute onset than IPF
    • -Imaging shows more areas of "ground glass"
    • -Areas of active alveolitis on pathology (less fibrosis typically seen)

    • Tx: much better response to immunosuppression
    • -may stabilize or resolve completely
  16. Cryptogenic Organizing Pneumonia (COP)
    • -Clinical syndrome (not to be confused with BOOP, which is a pathologic description)
    • -NOT associated with other diseases

    • Presentation:
    • -Acute to subacute
    • -may mimic CAP
    • -Does not improve with antibiotics
    • -Alveolar infiltrates, airway plugging with fibrosis material, bronchiolar inflammation

    • Histopathology: airway plugging with fibrinous material

    • Tx: steroid therapy (good response):
    • Pre:
    • Post:
  17. "weird stuff"
    • chronic eosinophilic pneumonia
    • pulmonary alveolar proteinosis
    • lymphagioleiomyomatosis
  18. Chronic Eosinophilic Pneumonia
    ≠Acute eosinophilic pneumonia (rapid, febril, sick)

    • Presentation:
    • -subacute to chronic
    • -weight loss, cough, dyspnea, fevers

    • CXR: "photo negative" of pulmonary edema
    • edema:
    • CEP: clear hilum, peripheral opacities

    -May see eosinophils on CBC, always seen on BAL

    • Tx: very responsive to steroids
    • -disease often recurrs after tx tapered off
  19. Pulmonary Alveolar Proteinosis
    • -Primary alveolar disease (not of the interstitium)
    • -Excessive accumulation of surfactant-derived proteinaceous phospholipids

    • Primary: Associated with anti-GM-CSF antibody
    • Secondary: varies, often associated with blood malignancy

    • CxR: "Crazy paving" pattern on CT
    • -not sensitive nor specific
    • (not a CT)
    • (CT:)

    • histology: proteinaceous material in alveoli

    • Tx: whole lung lavage
    • -Liters of fluid, under general anesthesia
    • -performed unilaterally in most cases

    -Exogenous GM-CSF (relatively effective in primary PAP)
  20. Lymphangioleiomyomatosis
    proliferation of smooth muscle cells, typically involving lymphatics, vasculature, and airways

    • Presentation:
    • -spontaneous pneumonthorax (cysts rupture)
    • -chylous pleural effusion
    • -hemoptysis
    • -Obstructive or restrictive pattern on PFTs

    • Imaging: shows cystic, reticular markings, prominent interlobular lymphatics

    • Epidemiology:
    • -found almost exclusively in women of childbearing age
    • -Estrogen drives smooth muscle proliferation

    • Tx: variable effectiveness
    • -Anti-estrogen therapy
    • -Oophorectomy
    • -Tamoxifen

    -Lung transplant often required