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Pulm III - Restrictive Dzs, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz SARCOIDOSIS-systemic, unknown cause -SE US, WOMEN, AF AMERICANS-not in Asians -noncaseating (hard) grans in many tissues -Dx of exclusion-exclude fungi, mycobac -bilateral hilar lymphadenopathy, lung in 90% -eye, skin involvement in 1/3-1/2 PATHOGENESIS- -inc. CD4 T cells in lung, IL-2 receptors, active macs ->monocyte influx, alveolitis, grans -oligoclonal T cells-unknown etiology MORPH-has been in almost every organ -unusual central necrosis -chronic-enclosed grans in fibrous rims, hyaline scars -SCHAUMANN BODIES-laminated concretions of Ca, proteins -stellate ASTEROID bodies w/in giant cells in 60% of grans both also in berylliosis LUNGS-no gross ABNLity unless coalesced->nodules -often heals, so various stages present -along lymphatics-LNs in most cases-enlarged, calcified SPLEEN-involved in 3/4 but enlarged in 1/5, liver less often BONE MARROW in 1/5 esp in HANDS, FEET SKIN LESIONS-SubQ nodules, plaques Mucous Membrans, EYE(iritis)->corneal opacity, glaucoma,blindness Eye GLANDS-suppressed lacrimation SALIV GLANDS-Mikulitz Syndrome-parotic, submax, submand glands CLINICAL-varies-(-)Sx-part of routine X-ray -or insidious onset-resp Sx or constitutional Sx Unpredictable course-remissions w/ steroids-65-70% recover fully -20% w/ permanent loss of lung fcn, visual impairment -10% die of CNS, cardiac involvement -3 stages, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz IN GENERAL -reduced TLC, FEV1 NL, CLINICAL-dysp, tachyp->cyanosis -no wheezing -reduced O2-diff capacity, lung vol, compliance -CXR-diffuse small nodules, ground-glass, irreg -can-ɮndary pulm HTN, cor pulmonale -all->scarring->end-stage/honeycomb lung -cause:ENVIRONMENTAL, SARCOID, IPF Pathogenesis: Start w/ ALVEOLITIS->dmg, distortion -fibrosis due to lymphokines, monokines Final result-end-stage fibrotic lung, PNEUMOCONIOSES -inhalation of organic/inorg products, fumes, vapors ->inc. morbidity from asthma, etc. PATHOGENESIS -smallest are most dangerous-go farther, dissolve better -mac response can be overwhelmed by amt of dust -Silica (& quartz)-react chemically w/ lung tissue->cell death -TRIGGER MACS TO FORM INFLAM -smoking worsens all, esp asbestos Types Berylliosis-NUCLEAR, AEROSPACE INDUSTRIES -beryllium->acute pneumonitis -can mimic sarcoidosis if chronic exposure -delayed hypersensitivity->noncaseating grans ->DYSPNEA, cough, wt loss, arthralgias -can stabilize, remit/relapse or -can ->pulm failure ->LUNG CANCER, PNEUMOCONIOSES -inhalation of organic/inorg products, fumes, vapors ->inc. morbidity from asthma, etc. PATHOGENESIS -smallest are most dangerous-go farther, dissolve better -mac response can be overwhelmed by amt of dust -Silica (& quartz)-react chemically w/ lung tissue->cell death -TRIGGER MACS TO FORM INFLAM -smoking worsens all, esp asbestos Types Silicosis-sand blasters, mine workers MOST PREVALENT CHRONIC OCCUP Dz In World -decades of exposure->progressive, nodular, fibrosing -acute form less common - w/ heavy acute exposure -looks like alv proteinosis (lipoproteinaceous in alv) PATHOGENESIS-CRYSTALLINE MORE FIBROGENIC-QUARTZ (noncrystalline-talc, vermiculite, mica) -SiOH interact w/ macs, epith proteins->denature-dmg, fibrosis -cleavage of silica(grinding)->surface free radicals -silica inot macs->recruit more, release mediators -upper lung zones-more fibrotic, less cellular than CWP -other minerals->REDUCED FIBROGENIC EFFECT MORPH-discrete nodules->hard collagenous scars -some w/ central necrosis/cavitation -hyalinized coll in concentric layers, surr by more condensed coll. -honeycomb pattern in lung parenchyma -CXR-EGGSHELL CALCIFICATIONS at LNs -can coalesce to PMF -birefringent silica particles under polarization CLINICAL-usually (-)Sx w/ routine CXR -SOB late in course, w/ PMF->progressive, slow to kill -no CA ass'n, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz HYPERSENSITIVITY PNEUMONITIS Caused by intense/long exposure to Ags, dusts Heightened reactivity – involves ALVS, not bronch From: bac spores, bac, fungi, animal proteins Farmer’s Lung: Actinomycetes spores in hay Pigeon breeder(excreta Ags), Ventilator(thermo bac) Byssinosis in textile workers-cotton, linen, hemp (immune etiology not as clear-wheezing) Type III HS immune complexes for acute -4-6hrs post in sensitized pt ->duffuse nodular infiltrates, acute restrictive Chronic-type IV DTH-progressive resp failure, dysp, cyanosis, dec. compliance, capacity MORPH-interstit pneumonitis w/ lymphos, plasma cells, macs (foamy cytoplasm) -interstit fibrosis, obliterative bronchiolitis -granuloma formation -intra-alv infiltrate in ᡪ%, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz PNEUMOCONIOSES -inhalation of organic/inorg products, fumes, vapors ->inc. morbidity from asthma, etc. PATHOGENESIS -smallest are most dangerous-go farther, dissolve better -mac response can be overwhelmed by amt of dust -Silica (& quartz)-react chemically w/ lung tissue->cell death -TRIGGER MACS TO FORM INFLAM -smoking worsens all, esp asbestos, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz PULM EOSINOPHILIA-Usually immunologic: -Simple Pulm Eos – Loffler syn -irreg intra pulm densities, eos in blood -septa thickened w/ eos, giant cells -sometimes w/ diffuse alv dmg -Tropical Eos – microfiliriae infx -2ndary chronic PE-in infx, drug allergies, HP, asthma, PAN, BP aspergillosis -Idiopathic Chronic Eos Pneumonia -focal consolidation in periph lung -lymphos, eos in alvs, septal walls -fever, night sweats, dyspnea -give steroids, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz DESQUAMATIVE INTERSTITIAL PNEUMONITIS IP w/ prominent agg of macs w/in alvs CLINICAL – slow development of cough, dyspnea ->resp embarrassment, cyanosis, clubbing X-ray-bilateral LL ground-glass infiltrates MORPH-macs contain PAS-(+) granules -some macs w/ lamellar bodies (surfactant) -interstit pneumonitis w/ septal hyperplasia ->epith cells desquamate Unknown cause Tx-steroids clear lungs better than IPF pts, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz PULM ALVEOLAR PROTEINOSIS Obscure cause: GM-CSF receptor defect -in immunosupp, blood CA, opp infx, dusts -Diffuse opacification -Intra-alv accum of lipid, PAS(+) material -sim to surfactant, but not properties -necrosis on hyperplastic pneumocytes ->nonspecific resp difficulty-cough, sputum -chunks of gelatinous material - Sx last for yrs: fever, dysp, cyanosis -or benign -Doesn’t usually->fibrosis, Chest Wall Disorders Diffuse Interstitial (Infiltrative,Restrictive) Dz BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA (BOOP) Common response to infx/inflam injury -Pts w/ cough, dyspnea, recent resp infx -Patchy opacities, interstitial infiltrates -Polypoid fibrous plugs in alvs,bronchioles -br involvement diff from pneumonia Causes:infx, toxins, drugs, coll vasc dz, GVHD w/ BM xplant -pt improves gradually or w/ steroids