Pulmonary Board Review

Escher Howard-Williams

6/16/08

Topics To Discuss

• Oxygen Desaturation During Exercise

• Causes of Bronchitis/Exacerbations in COPD

• Diagnosis of Wegener’s

COPD

• Heterogeneous disorder that includes:– Emphysema– Chronic Bronchitis– Obliterative Bronchiolitis– Asthma w/ bronchitis

• GOLD: A disease states characterized by airflow limitation that is not fully reversible. Usually progressive and is associated with abnormal inflammatory response

Diagnosis of COPD

• Stage 0: Normal spirometry, chronic cough and sputum production

• Stage I: Mild FEV1/FVC <70% predicted and FEV ≥ 80% predicted

• Stage II: FEV1/FVC < 70% and FEV1 ≥ 50% and < 80% predicted

• Stage III: FEV1/FVC < 70% and FEV1 ≥ 30% and < 50% predicted

• Stage IV: FEV1/FVC < 70% and FEV1 < 30% predicted or FEV1 < 50% predicted and chronic respiratory symptoms

COPD Exacerbation

• GOLD - a report produced by NHLBI & WHO defines COPD exacerbation as - acute increase in sx beyond normal daily variation

• Includes one or more of the following cardinal symptoms (over 2 days):– Cough increases in frequency and severity– Sputum production increases in volume and/or

changes character– Dyspnea increases

Etiology

• It is estimated that:– 50-60% COPD exacerbations are due to

respiratory infections – 10% are due to environmental pollution– 30% are of unknown etiology

Viral Infections

• Most infection are thought to be viral, and have been confirmed by viral culture or serology in 18 - 64% of exacerbation

• The most common viruses are influenza, parainfluenza, coronavirus, and rhinovirus

Bacterial Infections

• Viral infections cause the majority of COPD exacerbations, bacterial infection also trigger exacerbations

• Most common bacterial causes:– Haemophilus influenzae– Moraxella catarrhalis– Streptococcus pneumoniae

• Less Common include– Kebsiella, Peduomonas– Rarely - Chlamydia and Legionella

Treatment of COPD Exacerbation

• Treatment of exacerbations of COPD includes antibiotics directed against H. influenzae, M. catarrhalis, and S. pneumoniae – The use of antibiotics in exacerbations of COPD is

based on placebo-controlled trials that found that antibiotics improve clinical outcomes in many patients with an exacerbation of COPD

• Brochodilator, sterdoids, O2

Abx Treatment

"Standard"Doxycycline 100 mg BIDTrimethoprim-sulfamethoxazole DS BIDAmoxicillin 500 mg BID

"Modernized list"Amoxicillin-clavulanate (Augmentin) 875 mg BIDAzithromycin (Zithromax) Z pack (6-250 mg)Cefpodoxime (Vantin) 200 mg BIDCefuroxime (Ceftin) 250-500 mg BIDCefprozil (Cefzil) 500 mg BIDLoracarbef (Lorabid) 400 mg BIDLevofloxacin (Levaquin) 500 mg QDCiprofloxacin (Cipro) 500 mg BID

Severe exacerbation

• Treatment should consist of broad spectrum antibiotics that cover gram positives, gram negatives and consider double covering pseudomonas

Other Pharmacotherapy

• Stable COPD:– Bronchodilaters– Anticholinergics– Steroids– (No mucolytics or leukotrienes)

Note on Surgery

• Bullectomy may reduce sx in pt w/ bulla causing compression of adjoining lung tissue -> improve lung function

• Lung Volume Reduction surgery - FEV <20% and diffusion capacity <20% and people with upper lobe disease had better outcomes

• Lung Transplant - FEV1 <35, PaO2 55-60, PaCO2 >50

Arterial Desaturation w/ Exercise

• Exercise testing provides insight into severity of exertional dyspnea and functional stability

• Walk is usually done over 6 minutes• Desaturation is considered < 88% or

PaO2 <55 (or 89% and PaO2 60 in R heart failure, pulm htn, crit >55%)

• Some use - drop in sat by 5% points

Treatment?

• Underlying causes– PE– CHF– ILD/IPF– COPD

• Pulmonary rehabilitation

• Provide Oxygen

Pulmonary Rehabilitation

• Includes exercise, counseling / nutrition

• Most patients walk 500-600 meters

• With rehab can clinically improve walk distance by approximately 50 meters

• Goal is improve QOL

• No effect on survival or hospitalization rate

Controlled O2 Therapy

• Long term administration of O2 to COPD patients >15 hrs/day increases survival, hemodynamics, exercise capacity, lung mechanics and mental status

• Goal is sat >90% or PaO2 >60

Wegener’s

• Systemic vasculitis of small and medium vessel arteries– Rarely in large vessels

• Generally affects upper and lower respiratory tracts and kidneys– A limited form is present in 25% of cases

that manifests in upper respiratory tract only (or kidneys only)

Presenting signs

• The most common URT presenting symptoms include:– persistent rhinorrhea– purulent/bloody nasal discharge – oral/nasal ulcers – polyarthralgias – myalgias – sinus pain

• Less common symptoms are hoarseness, stridor, earache, both conductive and sensorineural hearing loss, or otorrhea

Presenting Signs

• Most common LRT symptoms:– Cough– Dyspnea– Hemoptysis (due to an alveolar capillaritis,

necrotic lesions, or endobronchial disease)– Pleuritic pain

Other Manifestations

• Upper and lower airways, including the subglottic region / trachea

• Joints (myalgias, arthralgias, arthritis)• Eyes (conjunctivitis, corneal ulceration,

episcleritis/scleritis, optic neuropathy, proptosis, diplopia, retinal vasculitis, and uveitis)

• Skin (vesicular, palpable purpuric, ulcerative, and hemorrhagic lesions)

Other Manifestations

• Nervous system (mononeuritis multiplex, cranial nerve abnormalities, central nervous system mass lesions, external ophthalmoplegia, hearing loss)

• Less commonly, the gastrointestinal tract, heart (pericarditis, myocarditis, conduction system abnormalities), lower genitourinary tract (including the prostate), parotid glands, thyroid, liver, or breast

General lab findings

• leukocytosis

• thrombocytosis

• marked elevation of ESR

• normochromic, normocytic anemia

Urinary Findings

• Proteinuria

• Hematuira

• Red Cell Cast

• Leukocyturia

Radiographic Findings

• Nodular Disease

• Infiltrates CXR– Diffuse bilateral patchy low denisty or

localized lesions and cavities

• CT– Bilateral nodueles cavitary and airspace

disease with focal consolidation and ground glass

Lung Biopsy

• Small and medium vessel vasculitis with necrotizing graulomas and inflammatory infiltrates

Diagnosis

• The American College of Rheumatology proposed clinical criteria for patients enrolled in studies of Wegener's and distinguish other forms of vasculitis (pre-ANCA)– Nasal or oral inflammation (painful or painless oral ulcers or

purulent or bloody nasal discharge)– Abnormal chest radiograph showing nodules, fixed

infiltrates, or cavities– Abnormal urinary sediment (microscopic hematuria with or

without red cell casts)– Granulomatous inflammation on biopsy of an artery or

perivascular area

ANCA

• Approximately 90 percent of patients with active, generalized Wegener's granulomatosis are ANCA-positive

• Limited forms of the disease up to 40% of patients may be ANCA-negative

• Among Wegener's pts w/ ANCA, 80-90%, PR3-ANCA+, the rest are MPO-ANCA+

• Dual positivity may rarely occur and generally indicates the presence of another condition ie SLE

• The majority of patients with renal-limited vasculitis are ANCA-positive, 75-80% MPO-ANCA +

PER MKSAP

• C-ANCA in a pt with active disease has a snesitivity of 90-95% and a specificity fo 90% and is considered the most helpful laboratory finding to make the diagnosis of Wegener’s

Tissue Diagnosis

• Dx of Wegener's confirmed by tissue biopsy at a site of active disease.

• Typical finding on upper respiratory tract biopsies is acute and chronic inflammation, often with granulomatous features. – These findings are consistent with but not diagnostic of

Wegener's granulomatosis since the vasculitis is seen in only about one-third of cases.

Other Tissues

• Skin reveals a leukocytoclastic vasculitis with little or no complement and immunoglobulin on immunofluorescence.

• Kidney biopsy typically reveals a segmental necrotizing glomerulonephritis that is usually pauci-immune on immunofluorescence or electron microscopy

Standard Treatment

• Combination therapy of corticosteroids and cyclophosphamide is the standard of care

• 3-6 months the disease is generally controlled and tx can be switched to azathioprine – This does not increase the rate of relapse

or change kidney function