CBI 3: 52 year old man with hemoptysis: WEGENER’S
GRANULOMATOSIS
Initial Case Presentation
Chief 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/Cocci
 Trauma
 Lung Cancer
 Goodpasture’s
 Mesothelioma
 Laryngeal Cancer
 PE
 CHF
 Vasculitis (Wegener’s)
 Lupus
 Pneumonia
HPI (Pertinent):
 Bright red blood in small amounts continuously last night and this morning. ½ cup total.
 Began 10-14 days ago with dyspnea, NPC, and 7 days of blood streaked sputum

+ night sweats
 35 pack/year smoking history
 X wt loss, chest pain, hematemesis, chest pain, orthopnea, wheezing, easy bruising/bleeding, hematuria.
ROS (Pertinent):
 Rash on fingers noted 1 month ago
 Mild myalgias x1 month
Physical (Pertinent):
 Pulse Ox: 90%
 RR: 24
 Crackles over both lung fields, prominent at bases
 Guiac +
Lab (Pertinent):
 CXR: Bilateral opacities with alveolar filling patterns
 Elevated WBC (PMNs) & Platelets
 Low CO2, PaCO2, PaO2 (mild respiratory alkalosis)
 Elevated BUN/Creatitine (10:1 intrarenal), ESR (inflammation), CRP
 Hematuria w/casts (glomerulonephritis), proteinuria
Learning Issues
 Red rash- Steve
 Goodpasture’s- Jen
 Lupus-Karen
 Wegener’s- Nick
 Obstructive VS Restrictive Lung Dz (lung function tests and causes) Summarize quantifying renal function (calculate GFR, renal plasma clearance, afferent and efferent
arterioles)-Raji
 Antibiotics review-Mike
Friday Session-Presentations
Palmar Rash

Purpura: RBC’s extravasate into superficial space. Palpable VS non-palpable.

Wegner’s skin manifestations: Ulcers, purpura, vesicles, hemorrhage, and papules.

Goodpasture’s skin manifestations: None

SLE skin manifestations:
o Malar facial rash
o Discoid pattern rash
o Subacute cutaneous: Recurrent (papulosquamous or annular) rashes on extremities on face, extremities, and
trunk.
SLE

Systemic 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:
o Malar/discoid rash
o Serositis
o Photosensitivity
o Fever
o Arthritis
o Libman-Sacks endocarditis (mitral
o Hematologic disorders
vegetations)
o Renal disorder (subendothelial wireo Cause of Death: Infection and renal
loop lesions, mesangial hyperplasia)
failure.

Diagnosis: ANA’s-Generic, Anti-DS DNA, Anti-Smith, anti-histone, anti-phospholipid (causes false-positive syphilis
test).

Tx: Glucocorticoids
Goodpasture’s

Autoimmune disease

Etiology: Antibodies to 3 chain of type 4 collagen present in the basement membrane

Pathogenesis: 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:
o Lung: Hemosiderin laden macrophages and type 2 pneumocyte hypertrophy, fibrous septate thickening.
o Kidney: Focal proliferative glomerulonephritis with crescentic morphology.
o Both: Linear deposition of IG along the basement membrane.

Diagnosis: Percutaneous renal biopsy

Tx: Plasmapheresis or immunosuppression.
Wegner’s Granulomatosis

Systemic necrotizing granulomatous vasculitis of small and medium arteries and veins

Etiology: 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:
o Acute/Chronic
o Upper/lower respiratory

Ulceration, septal perforation, saddle nose deformity, sinusitis, pulmonary infiltrates, nodules with
cavitation, hemorrhage, obstructive or restrictive lung disease)
o Renal involvement

Crescentic glomerulonephritis with renal insufficiency.
o Fever
o Weight loss
o Myalgias/arthralgias
o Cutaneous: purpura, ulcers, gangrene
o Neurologic: peripheral neuropathy, cranial neuropathy, infarction, hemorrhage, seizures
o GI: Abdominal pain, diarrhea, bleeding, ulcerations, infarction
o Cardiac: Pericarditis, arteritis, conduction defects, cardiomyopathy
o GU

Diagnosis: Renal biopsy with crescentic GN, granuloma formation, and fibrinoid necrosis. Presence of C-ANCA. +
Rheumatoid factor.

Tx: Cyclophosphamide, corticosteroids.
Spirometry Interpretations

FVC: Forced vital capacity

FEV-1: Forced expiratory volume in one second

FEF 25-75%: Forced expiratory flows between 25-75% of volume

Obstructive
o Increased resistance within the lung causing decreased flows
o FVC: Normal
o FEV-1: Decreases
o FEV1/FVC: Decreases

Restrictive
o Loss of effective lung volume
o FVC: Decreases
o FEV-1: Decreases
o FEV1/FVC: Normal to increased

Quantifying Renal Function

GFR x P inulin = V x Uinulin  GFR = (V x Uinulin) / Pinulin

Can 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 time
o If clearance is > Cin the substance is filtered + secreted
o
If clearance is < Cin the substance is filtered + reabsorbed

Effective renal plasma flow: (Upah x V)/Ppah

RPF: ERPF/.9

Renal 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- FEx
Antibiotic Review- See Mike’s Presentation 
1. Identify categories of diseases that result in hemoptysis and specific disease processes in each
category.
 Airway Disease
o Upper-obstruction, trauma, mucosal ulceration, bronchitis, infection, neoplasm
o Lower-obstruction, neoplasm, trauma, infection
 Pulmonary parenchymal disease: PF, ARDS, infection (tuberculosis, coccidiodes), autoimmune
disease (lupus, Goodpasture’s, Wegener’s), iatrogenic, cocaine use
 Pulmonary vascular disease: PE, AVM (osler-weber-rendupresents w/telangiectasia),
pulmonary hypertension
 Cryptogenic
2. Explain the utility of urinalysis in the diagnosis of renal disease and the particular significance of red
blood cell casts.
 Components of Urinalysis:
o Color/consistency
o Glucose +/o Protein +/o Bacteria +/o pH
o Cells
o Osmolality/specific gravity
o Casts
 The 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).
3. 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 crossreactive 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.