Parenchymal Lung Diseases and Sarcoidosis Flashcards

Question

COP Treatment

Answer 1

Responds well to several months of **steroids**.

Answer 2

"Hamman-Rich Syndrome" * **Pathologic features of ARDS appears acutely in pt without known triggers** * **Fulminant presentation** * Rapid progressive clinical course * Average age 50-55 y/o * Not smoking related

Answer 3

Termed **diffuse alveolar damage (DAD)** * ↑ Pulmonary vascular permeability * Interstitial inflammation * Epithelial cell death * **See hyaline membranes lining alveolar spaces**

Answer 4

Diffuse, bilateral alveolar infiltrates.

Answer 5

* History * Age * Fhx * Drugs, CT diseases, dust exposure, Beryllium, World Trade Center, etc. * Physical exam * CXR

Answer 6

_Need all 3 of the following:_ * Compatible clinical symptoms * Evidence of non-caseating granulomas * Rule out other causes of granuloma

Answer 7

* Affects all races and ethnic groups * **♀ \> ♂** * Peak incidence age **20-39 y/o** * Most affected populations: * Northern Europeans * Japan * African Americans

Answer 8

No single predominant cause of sarcoidosis found.

Answer 9

* Beryllium * Inhalation of inorganic particles * World Trade Center * Wood burning stoves * Moldy environments * Occupational exposures * US Navy, firefighters, metal workers, buliding supplies, agricultural employment * Insecticides

Answer 10

* Microbial bioaerosols * Nontuberculous mycobacterial DNA * Ab to Mycobacterium tubeculosis Ag and proteins * Propionibacterium RNA

Answer 11

* Inciting Ag unknown * **Mφ** stimulation w/ **CD₄₊ T-cell** activation * Predominant CD₄₊ response w/ **variable T_h1/T_h2** paths ⇒ stimulates **fibroblast proliferation** ⇒ collagen production ⇒ **progressive fibrosis** * **Pulmonary Sarcoid granulomas** **form** * Epithelioid giant cells * Granulomas ⇒ persist, resolve, fibrosis

Answer 12

**Non-caseating granulomas which eventual hyalinize.** * **Giant cells** * **Schaumann bodies** ⇒ concentric laminations of calcium or proteins * **Asteroid bodies** ⇒ stellate inclusions

Answer 13

* **May be asymptomatic** ⇒ incidental finding on CXR * **Systemic sx** ⇒ fever, fatigue, night sweats * Other specific sx related to organ involvment * General course: * **⅔ resolve spontaneously** * **⅓ develop fibrosis w/ varying organ dysfunction**

Answer 14

Any organ or tissue may be involved. * Common sites * **Lung** * **Skin** * **Eye** * Less common * Brain and nerves * Heart * Liver and spleen * Bone and joints * Kidney

Answer 15

Stages do not correlate w/ sx, chronicity, or pulmonary function.

Answer 16

Asymptomatic Dyspnea Cough

Answer 17

* 50% ⇒ restrictive pattern * 40% ⇒ combined restrictive and obstructive pattern * **DLCO ⇒ most sensitive indicator of disease** * Most have a bronchodilator response

Answer 18

* Erythema nodosum * Macules, plaques, and papules * Lupus pernio * Löfgren's syndrome

Answer 19

* **SubQ erythematous nodules** * **Lymphocytic panniculitis of SubQ fat** * Usually pretibial * Painful * **Usually self-limiting and benign** * Seen in younger women

Answer 20

* **Often contain granulomas** * Locations: * Neck, upper back and trunk, extremities * Areas of previous scars * Tattoos * **Associated with chronic, poorly responsive disease**

Answer 21

* **Violaceous nodules on nose, cheeks, and lips** * Associated w/ chronic disease & extrapulmonary involvement * Contains granulomas

Answer 22

* _Triad of findings_ 1. **Polyarticular large joint arthritis** 2. **Erythema nodosum** 3. **Bilateral hilar adenopathy on CX**R * Acute presentation * **Often resolves spontaneously or w/ short course of steroids**

Answer 23

* Occurs in 10% of sarcoid pts * Associated with systemic sx * **Fever, night weats, anorexia, weight loss** * Labs: * ↑ Bilirubin, Alkaline phosphatase * Normal transaminases * **Severe hepatic manifestations** ⇒ 1% * Portal HTN w/ varices * Hepatopulmonary syndrome * Cirrhosis and liver failure

Answer 24

* **Granulomatous inflammation and scarring in LV free wall & septum** * Septal affects conducting system * Presentation: * **Restrictive cardiomyopathy** * Dyspnea on exertion, orthopnea, PND * **Conduction abnormalities** * Tachyarrhythmias, bradyarrhythmias, LOC * Diagnosis: * Biopsies often unhelpful * Biopsy site RV, involvement is LV * **Cardiac MRI or PET scan** * EP study * Found in ~ 25% of cases at autopsy * Clinically apparent in 5%

Answer 25

* Sarcoid Mφ have high levels of 25-OH-Vit D hydroxylase * Converts 25-OH Vit D → 1,25-OH Vit D * **↑ calcium absorption ⇒ hypercalcemia and hypercalciuria** * **May cause renal calculi and obstruction** * **Renal failure d/t granulomatous nephritis rare** * Renal disease management * Monitor calcium levels yearly w/ active sarcoidosis * Check 24 hr urinary calcium excretion * **↑ serum or urinary calcium excretion may warrant systemic sarcoid treatment**

Answer 26

* 25% @ autopsy, **10% clinical involvement** * Manifestations: * **Cranial-nerve palsies** * **Headache** * **Ataxia** * **Cognitive dysfunction** * **Weakness** * **Seizures** * Diagnosis: * MRI ⇒ periventricular white matter ∆ * Lumbar puncture ⇒ nonspecific * Lymphocytes, Oligoclonal bands, ↑ ACE levels

Answer 27

* Seen in 25% of cases * **75% have anterior uveitis (iritis)** * Acute or chronic * Sx: **blurred vision, tearing, photophobia** * May lead to **glucoma** and **vision loss** * **30% have posterior uveitis** * Ass. w/ CNS involvement * **Conjunctiva may show yellow nodules or cysts** * **Lacrimal glands involvement** ⇒ dry eyes * All sarcoid pts should have yearly eye exams

Answer 28

* History w/ attention to environmental/occupational exposures, fhx * CXR * PFTs * EKG * Slit lamp eye exam * Biopsy of affected organ

Answer 29

* Infectious * Typical and atypical TB * Fungal * Brucellosis * Rhematologic * Juvenile RA * Polyangiitis w/ granulomatosis * Rheumatoid nodules * Lymphoma * Hypersensitivity pneumonitis * Berylliosis * Inflammatory bowel disease * Granulomatous hepatitis * FB aspiration or IV injection * Common variable immunodeficiency * Lung CA

Answer 30

* Dx is not an indication for treatment * Most manifestations are asymptomatic and not disabling * Many manifestations are self-limiting * Consider instituting therapy when organ function is threatened * Brain, heart, hypercalcemia

Answer 31

* Initial therapy * Prednisone PO * Re-evaluate response at 1-3 months * Decrease to lowest effective dose for 9-12 months * Failure of steroids * Consider immunosuppressive or cytotoxic agents * Methotrexate * Azathioprine * Hydroxychloroquine * Infliximab or other anti-TNF agent

Answer 32

_A granulomatous disease:_ **Spectrum of predominantly interstitial lung disorders caused by intense and prolonged exposure to inhaled organic dusts:** ⇒ Thermophilic bacteria ⇒ True fungi ⇒ Animal protein ⇒ Bacterial products

Answer 33

Hypersensitivity pneumonitis caused by: ## Footnote **Harvested, humid hay which grows thermophilic actinomyces**

Answer 34

Hypersensitivity pneumonitis caused by: ## Footnote **Proteins from bird excreta and feathers**

Answer 35

Hypersensitivity pneumonitis caused by: ## Footnote **Thermophilic bacteria in heated water reservoirs**

Answer 36

* _Acute form_ * Sx appear **4-6 hours** after exposure * Recurrent attacks of **fever, SOB, cough, and leukocytosis** * Diffuse and nodular infiltrates on CXR * PFT w/ restrictive pattern * Early in disease course, progression prevented by removal of agent * Continued exposure ⇒ _chronic form_ * **Progressive respiratory failure** * **SOB** * **Cyanosis** * **↓ TLC and compliance**

Answer 37

* Left ⇒ **Interstitial pneumonitis** * Lymphocytes, Mφ, and plasma cells expand width of alveolar septa * Middle ⇒ **Interstitial fibrosis w/ inflammatory cells in thickened speta** * Right ⇒ **Granuloma formation**

Answer 38

_Several diseases w/ eosinophlic infiltration:_ * **Acute eosinophilic PNA** w/ respiratory failure * **Simple pulmonary eosinophilia** (Loffler syndrome) * **Tropical eosinophilia** 2/2 microfilarial infection * **Secondary eosinophlia** * D/t various infections, hypersensitivity pneumonitis, drug allergies, asthma * **Chronic eosinophilic PNA**

Answer 39

**Non-neoplastic lung reaction to inhalation of mineral dusts or other particles or vapors.** * Agents: * **Coal dust** * **Silica** * **Asbestos** * **Beryllium** * Development depends on: * Size, shape, and bouyancy of particles * Concentration in air * Duration of exposure * Particle solubility and physiochemical reactivity * Additional effects of other irritants

Answer 40

* Coal Workers Pneumoconiosis (CWP) * Anthracosis * Simple CWP * Complicated CWP * Silicosis * Abestosis * Berylliosis

Answer 41

* Asymptomatic * Carbon pigment in Mφ, CT, lymphoid tissue

Answer 42

* Accumulation of Mφ * Little or no pulmonary dysfunction * Coal macules & coal nodules * Upper lobes and upper zones of lower lobes

Answer 43

"**Progressive Massive Fibrosis (PMF)** or **Black Lung**" * Extensive fibrosis * Pulmonary function compromised * Takes many years to develop * Multiple large blackened scars: 2-10 cm * Pigment and dense collagen

Answer 44

* **Most prevalent occupational disease in the world** * Presents after decades of exposure to **sandblasting, mining, stone cutting** * Silica occurs in _amorphous and crystalline forms_ * Crystallin form more fibrogenic

Answer 45

* Tiny, barely palpable nodules initially * Pale to black depending on ± coal dust * Coalesce ⇒ **hard collagenous scars containing** **silica particles** * **May undergo central softening** ⇒ superimposed TB or ischemia * **Eggshell calcification** * Intervening parenchyma compressed or over-expanded ⇒ **honeycomb pattern** * Can end as **Progressive Massive Fibrosis**

Answer 46

Two distinct geometric forms: * **Serpentine** ⇒ curly and flexible fibers * Chrysolite ⇒ used in industry * **Amphibole** ⇒ straight, stiff, brittle fibers * Crocidolite, amosite, tremolite, anthophyllite, actinolyte * Less prevalent but more pathogenic * Esp. mesotheliomas

Answer 47

Can show many different clinical pictures: * Pleural plaques * Pleural effusions * **Parenchymal intersitial fibrosis (aka asbestosis)** * **Mesothelioma** * Bronchogenic carcinoma * Laryngeal and other extrapulmonary neoplasms

Answer 48

* **Affects lower lobes and subpleural areas first** * Middle & upper lobes affected as fibrosis progresses * Visceral pleura ⇒ **fibrous thickening** * Parietal pleura ⇒ **pleural plaques** * Made of dense collagen and calcium

Answer 49

"Parenchymal Interstitial Fibrosis" 1. Fibrosis of respiratory bronchioles & alveolar ducts 2. Fibrosis of alveolar sacs and alveoli 3. Diffuse pulmonary interstitial fibrosis 4. Honeycomb lung

Answer 50

**Asbestos bodies** ⇒ fusiform beaded rods w/ translucent center **Ferruginous body** ⇒ asbestos body coated with iron-containing proteinaceous material

Answer 51

* **Aerospace and nuclear industries** * Acute exposure ⇒ almost non-existent, regulated * _Chronic exposure_ ⇒ **non-caseating pulmonary granulomas with hilar lymphadenopathy** * Less common in spleen and liver * Caused by induction of _cell-mediated immunity_ * **Heavy exposure linked to lung cancer**