Two Women with Hemoptysis Ellen Barbouche, MD Primary Care Conference 8 June 2005 NO FINANCIAL DISCLOSURE

Objectives Review differential diagnosis of hemoptysis Update role of imaging in evaluation of hemoptysis Explore specific diagnoses of presented cases –Clinical features –Diagnostics –Treatment and outcomes

Patient #1, History 46 year old female with hypertension 25 pack/year history tobacco, 1 ppd Investment broker and horsewoman Productive cough for 5 days Blood streaked sputum for 24 hours No shortness of breath, rhinitis, or fever Slight pleuritic, anterior chest tightness

Patient #1, Exam T 97.8, P 100, BP 124/92, pulse ox 97% Purulent right posterior nare No cervical, supraclavicular, or axillary nodes Bilateral rhonchi, clear after cough Heart: RRR, no S3, no murmur

Patient #2, History 42 year old, previously healthy 25 pack/year history tobacco, 1ppd Machinist for 6 months and horsewoman One week cough, occasional bloody sputum Slight dyspnea No rhinitis, chest pain, or fevers Swollen, painful right ankle

Patient #2, Exam T 97.0, P 88, RR 12, BP 128/78, pulse ox 98% Nares without mucus or lesion Lungs clear bilaterally Heart: RRR, no murmur, gallop, or rub No cervical, supraclavicular, or axillary nodes Hot, red, swollen left ankle

Hemoptysis Distinguish from upper respiratory tract or GI Airways disease *MOST COMMON –Tertiary hospital study: bronchiectasis, bronchogenic carcinoma, bronchitis Hirshberg, B et al. Chest 1997;112:440. Pulmonary parenchymal disease Pulmonary vascular disease Other

Evaluation of Hemoptysis History and physical Chest radiograph Lab guided by history Possible CT versus bronchoscopy –High resolution chest CT demonstrated more tumors than bronchoscopy, but not bronchitis McGuinness, G et al. Chest 1994;105:1155.

Patient #1, Chest Radiograph

Patient #1, Initial Course Treatment with azithromycin for community acquired, atypical pneumonia 2 week follow up –Decreased cough intensity and sputum production –Decreased, but persistent, dime-sized hemoptysis

Patient #1, Chest Radiograph

Patient #1, Chest CT

Patient #1, Pulmonary Consult Bronchoscopy –Organizing pneumonitis with 49% eosinophilia –Cultures negative

Acute Eosinophilic Pneumonia Idiopathic, possibly hypersensitivity reaction to inhaled antigen –1:10K US military in Iraq March Schorr, AF et al. JAMA 2004;292:2997. –Resolution of AEP despite smoking Kitihara, Y et al. Int Med 2003;42:1016. Classic presentation: one week febrile illness with cough and dyspnea 2/3 patients progress to respiratory failure requiring mechanical ventilation Philit, F et al. Am J Resp Crit Care Med 2002;166:1235.

Acute Eosinophilic Pneumonia Peripheral eosinophilia develops later in course, not at presentation CXR: subtle reticular opacities progressing to bilateral, diffuse mixed alveolar and reticular opacities CT: bilateral, patchy ground-glass or reticular opacities BAL > 25% eosinophilia

AEP, Treatment Uniformly responsive to steroids Typically continue steroids 2-4 weeks after symptom and CXR abnormality resolution Allen, JN et al. AM J Resp Crit Care Med 1994;150:1423.

Patient #1, Post Rx CT

Patient #2, Chest Radiograph

Patient #2, Laboratory WBC 18.9K, 15K neutrophils Hemoglobin 10.3, Platelets 275K Sedimentation rate 47, CRP 5 Creatinine 1.1 UA Protein 1+, WBC 6-10, RBC >50 C-ANCA >1:1280 ANA negative

Wegener’s Granulomatosis, Respiratory Involvement Multisystem vasculitis “Classic” – respiratory tract and kidneys “Limited” – respiratory tract largely Symptoms –Rhinorrhea, often bloody, with oral or nasal ulcers –Cough, hemoptysis, and pleuritic pain –Fever, malaise, eye symptoms, arthritis, rash

Wegener’s, Laboratory Findings Leukocytosis, normocytic anemia, elevated sedimentation rate C-ANCA –Positive >90% limited OR classic –Majority: autoantibodies to proteinase 3 –Minority: p-ANCA with myeloperoxidase antibodies UA –Limited Wegener’s normal UA –Renal involvement may elevate creatinine, and show proteinuria or active sediment

Wegener’s Chest Radiography Nodules, possibly cavitary –Single or multiple –Approximately 50% cavitary Alveolar opacities –< 5% alveolar hemorrhage Pleural opacities Cordier, JF et al. Chest 990;97:906

Wegener’s Diagnosis Histopathologic evidence of vasculitis and granuloma –Most likely from lung biopsy –Renal biopsy more likely to show focal segmental necrotizing glomerulonephritis Jennette, JC et al. Am J Kidney Dis 1994;24:130. Compatible clinical presentation

Wegener’s Treatment Cyclophosphamide, with or without steroids Serious morbidity and mortality due to cyclophosphamide 60-80% recurrence WGETRG NEJM 2005;352:351.

Patient #2, Course Difficult due to chest pain, likely related to Wegener’s Severe anemia due to renal insufficiency, pulmonary hemorrhage, and cyclophosphamide Very gradual improvement, though not yet able to work

Hemoptysis, Conclusions Multiple possible sources of hemoptysis –Bronchiectasis, bronchoalveolar carcinoma, bronchitis most common from lower respiratory tract Most effective work up: history and physical plus chest radiography –Above guide lab, HRCT, and bronchoscopy

Specific Diagnoses Acute Eosinophilic Pneumonia –Idiopathic –Frequently very severe –Excellent response to steroids Wegener’s Granulomatosis –Vasculitis affecting respiratory tract + kidneys –Usually c-ANCA + –Dangerous treatment with frequent recurrences