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CBI 3: 52 year old man with hemoptysis: WEGENER’S GRANULOMATOSISInitial Case PresentationChief Complaint:Martin Smith is a 52 year old male mechanic who comes to urgent care complaining of coughing up blood since the previous evening.Diff Dx: TB/CocciLung CancerMesotheliomaPEVasculitis (Wegener’s) PneumoniaTraumaGoodpasture’sLaryngeal CancerCHFLupusHPI (Pertinent):Bright red blood in small amounts continuously last night and this morning. ? cup total.Began 10-14 days ago with dyspnea, NPC, and 7 days of blood streaked sputum + night sweats35 pack/year smoking historyX wt loss, chest pain, hematemesis, chest pain, orthopnea, wheezing, easy bruising/bleeding, hematuria.ROS (Pertinent):Rash on fingers noted 1 month agoMild myalgias x1 monthPhysical (Pertinent):Pulse Ox: 90%RR: 24Crackles over both lung fields, prominent at basesGuiac +Lab (Pertinent):CXR: Bilateral opacities with alveolar filling patternsElevated WBC (PMNs) & PlateletsLow CO2, PaCO2, PaO2 (mild respiratory alkalosis)Elevated BUN/Creatitine (10:1 intrarenal), ESR (inflammation), CRPHematuria w/casts (glomerulonephritis), proteinuriaLearning IssuesRed rash- SteveGoodpasture’s- JenLupus-KarenWegener’s- NickObstructive VS Restrictive Lung Dz (lung function tests and causes)-Summarize quantifying renal function (calculate GFR, renal plasma clearance, afferent and efferent arterioles)-RajiAntibiotics review-MikeFriday Session-PresentationsPalmar RashPurpura: RBC’s extravasate into superficial space. Palpable VS non-palpable.Wegner’s skin manifestations: Ulcers, purpura, vesicles, hemorrhage, and papules.Goodpasture’s skin manifestations: NoneSLE skin manifestations:Malar facial rashDiscoid pattern rashSubacute cutaneous: Recurrent (papulosquamous or annular) rashes on extremities on face, extremities, and trunk. SLESystemic autoimmune disease seen mostly in young women with higher incidence in African and Hispanic Americans.Etiology: Antibodies to many different self tissues form. HLA DR3 and DR4 predispose people to developing SLE.Pathophysiology: Type III hypersensitivity due to immune complex deposition in the tissues with activation of complement. Clinical Criteria: Malar/discoid rashPhotosensitivityArthritisHematologic disordersRenal disorder (subendothelial wire-loop lesions, mesangial hyperplasia)SerositisFeverLibman-Sacks endocarditis (mitral vegetations)Cause of Death: Infection and renal failure.Diagnosis: ANA’s-Generic, Anti-DS DNA, Anti-Smith, anti-histone, anti-phospholipid (causes false-positive syphilis test).Tx: GlucocorticoidsGoodpasture’sAutoimmune diseaseEtiology: Antibodies to 3 chain of type 4 collagen present in the basement membranePathogenesis: Type II hypersensitivity due to antibodies that cause destruction of the basement membrane in areas with small capillary beds such as the lung and the glomerulus.Clinical: Episode of progressive glomerulonephritis, pneumonitis (hemoptysis, focal consolidations, restrictive lung disease) with unknown trigger. Cause of death: Uremia.Path: Lung: Hemosiderin laden macrophages and type 2 pneumocyte hypertrophy, fibrous septate thickening.Kidney: Focal proliferative glomerulonephritis with crescentic morphology. Both: Linear deposition of IG along the basement membrane.Diagnosis: Percutaneous renal biopsyTx: Plasmapheresis or immunosuppression.Wegner’s GranulomatosisSystemic necrotizing granulomatous vasculitis of small and medium arteries and veinsEtiology: Genetic Pathogenesis: Anti-neutrophil cytoplasmic antibodies (ANCAs) are responsible for inflammation, granuloma formation, and necrosis of vasculature causing end organ damage associated with the wide variety of symptoms seen.Clinical:Acute/ChronicUpper/lower respiratory Ulceration, septal perforation, saddle nose deformity, sinusitis, pulmonary infiltrates, nodules with cavitation, hemorrhage, obstructive or restrictive lung disease)Renal involvementCrescentic glomerulonephritis with renal insufficiency.FeverWeight lossMyalgias/arthralgiasCutaneous: purpura, ulcers, gangreneNeurologic: peripheral neuropathy, cranial neuropathy, infarction, hemorrhage, seizuresGI: Abdominal pain, diarrhea, bleeding, ulcerations, infarctionCardiac: Pericarditis, arteritis, conduction defects, cardiomyopathyGUDiagnosis: Renal biopsy with crescentic GN, granuloma formation, and fibrinoid necrosis. Presence of C-ANCA. + Rheumatoid factor. Tx: Cyclophosphamide, corticosteroids.Spirometry InterpretationsFVC: Forced vital capacityFEV-1: Forced expiratory volume in one secondFEF 25-75%: Forced expiratory flows between 25-75% of volumeObstructiveIncreased resistance within the lung causing decreased flowsFVC: NormalFEV-1: DecreasesFEV1/FVC: DecreasesRestrictiveLoss of effective lung volumeFVC: DecreasesFEV-1: DecreasesFEV1/FVC: Normal to increased Quantifying Renal FunctionGFR x Pinulin = V x Uinulin GFR = (V x Uinulin) / PinulinCan also be done using creatinine, but this overestimates GFR because creatinine is secreted from the tubules as well as being filtered.Clearance: Volume of plasma cleared of a substance per unit timeIf clearance is > Cin the substance is filtered + secreted If clearance is < Cin the substance is filtered + reabsorbedEffective renal plasma flow: (Upah x V)/PpahRPF: ERPF/.9Renal Blood Flow: ERPF / (1-HCT)FF: GFR/RPF (amount of plasma that flows through the kidney that is filtered by the glom)Fractional Excretion: FEx = (Ux x V) / (Px x GFR)Fractional Reabsorption: FRx = 1- FExAntibiotic Review- See Mike’s Presentation Identify categories of diseases that result in hemoptysis and specific disease processes in each category. Airway DiseaseUpper-obstruction, trauma, mucosal ulceration, bronchitis, infection, neoplasmLower-obstruction, neoplasm, trauma, infectionPulmonary parenchymal disease: PF, ARDS, infection (tuberculosis, coccidiodes), autoimmune disease (lupus, Goodpasture’s, Wegener’s), iatrogenic, cocaine usePulmonary vascular disease: PE, AVM (osler-weber-rendupresents w/telangiectasia), pulmonary hypertensionCryptogenicExplain the utility of urinalysis in the diagnosis of renal disease and the particular significance of red blood cell casts. Components of Urinalysis:Color/consistencyProtein +/-pHOsmolality/specific gravityGlucose +/-Bacteria +/-CellsCastsThe components of a urinalysis, as shown above, are many and varied. There are also a number of specific urine tests that can be ordered that examine for the presence/absence of specific molecules to help evaluate specific disease process (ie porphobilinogen in porphyria). Urinalysis is a helpful tool in evaluating the cause of renal/urinary disease because there are characteristic findings which are strongly suggestive of specific diagnoses. RBC casts indicate glomerulonephritis (glomerular inflammation), and can help differentiate renal disease that presents with hematuria from cystitis that presents with hematuria (which would show RBC’s without casts). Explain potential pathogenic mechanisms underlying disease processes that produce the syndrome of pulmonary alveolar hemorrhage and acute glomerulonephritis (red cell casts, and renal insufficiency). Post-streptococcal glomerulonephritis: Antibodies to the group b strep M antigen can be cross-reactive with the glomerular basement membrane and cause glomerulonephritis secondary to a streptococcal infection.Goodpasture’s Syndrome: Short-lived circulating of antibodies directed against the -chain of type 4 collagen (found in the basement membrane) attach to the GBM and the alveolar BM inciting inflammation and the classical presentation of glomerulonephritis and hemoptysis. Inciting stimulus is unknown.Lupus: Lupus is an autoimmune disorder in which circulating anti-nuclear antibody complexes deposit in tissues and incite an inflammatory response that causes tissue damage.Wegener’s: Wegener’s is an autoimmune disorder that is the result of antibodies to neutrophil granule proteins that cause release of inflammatory mediators that cause a systemic vascular inflammation. The production of anti-neutrophil cytoplasmic antibodies (ANCAs) is one of the hallmarks of WG. ................
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