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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
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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
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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)
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Systemic sclerosis
aka Scleraderma
- Severe fibrosis
- Pulmonary arterial hypertension
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Polymyositis-dermatomyositis
- interstitial fibrosis
- Bronhiolitis obliterans with organizing pneumonia (BOOP)
- Respiratory muscle weakness--> respiratory distress/failure
- Repeated aspiration events from dysphagia
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Sjögren's syndrome
- Interstitial fibrosis
- Lymphocytic infiltration of alveoli
- -lymphocytic interstitial pneumonia (LIP)
- -Lymphoma
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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)
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Goodpasture's syndrome
Rare.
- -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
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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)
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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
 
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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
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New therapyies being explored (Inflizimab, etanercept, ustekinumab, golimumab)
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Stage II sarcoidosis: lymphadenopathy and parenchymal disease
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Stage IV sarcoidosis: extensive destruction and architectural distortion
-Corina and diaphragm are higher than usual due to fibrosis/volume loss
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Idiopathic pulmonary fibrosis
(IPF)
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
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Non-specific Interstitial Pneumonia
(NSIP)
- 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
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"weird stuff"
- chronic eosinophilic pneumonia
- pulmonary alveolar proteinosis
- lymphagioleiomyomatosis
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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
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Pulmonary Alveolar Proteinosis
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Lymphangioleiomyomatosis
(LAM)
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
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