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Dry Cough and Clubbing in a 45-Year-Old Woman

Case Editor - Judd Flesch

Reviewed By Clinical Problems Assembly

Submitted by

Jamie L. Garfield, MD

Fellow

Pulmonary and Critical Care Medicine

Temple University School of Medicine

Philadelphia, Pennsylvania

Victor Kim, MD

Assistant Professor of Medicine

Temple University School of Medicine

Philadelphia, Pennsylvania

Submit your comments to the author(s).

History

A 45-year-old white female presents to pulmonary clinic for evaluation of increasing chest tightness, cough and dyspnea.  She has a history of chronic respiratory symptoms since childhood and frequent exacerbations of bronchitis, but no history of respiratory failure. She reports that until 1 year ago she was limited by mild pleuritic chest tightness and shortness of breath with heavy exertion.  She now reports daily symptoms of fatigue, dyspnea, and exercise intolerance with minimal exertion. She has persistent dry cough and occasionally expectorates granular mucus. She has been using 3 liters per minute of supplemental oxygen via nasal cannula for the past year.

Her past medical history is significant for polycythemia, gastroesophageal reflux disease, fibronodular breast disease, chronic tinnitus with hearing loss and genital herpes. Her current medications include famotidine, diltiazem, valacyclovir, inhaled fluticasone, salmeterol and albuterol. She has a 30 pack-year smoking history, having quit smoking 5 years ago. She drinks 1-2 beers per week and she denies illicit drug use. She is a homemaker and reports no occupational exposures. Her parents, siblings and 2 children (ages 16 and 24) are alive and well with no respiratory disease or malignancy.

Physical Exam

On physical examination, her heart rate is elevated at 112 beats per minute. Oxygen saturation on 2 liters of oxygen via nasal cannula is 89%. Her vital signs are otherwise normal. Her head and neck exam is benign. Her pulmonary exam is significant for diffuse inspiratory and expiratory dry crackles, more prominent at the bases bilaterally. Cardiac exam reveals regular rhythm with no murmurs, rubs or gallops. There is clubbing but no cyanosis or edema of her extremities.

Lab

White blood cell count 12,900/mm3, Hemoglobin 15.5 mg/dl, Platelet count 473,000/mm3.

BUN 9 mg/dl, creatinine 1.0 mg/dl, calcium 8.8 mg/dl and phosphate 4.9 mg/dl.

PT, PTT, electrolytes, glucose and liver function tests were within normal limits.

Arterial blood gas on 3L/min supplemental oxygen revealed a pH of 7.39, PCO2 40 mm Hg and PaO2 62 mm Hg.

Studies:

Echocardiogram: left ventricular ejection fraction was 55-65% and grade 1 diastolic dysfunction was noted; right ventricle was mildly dilated and had mildly depressed systolic function; pulmonary artery systolic pressure was estimated to be 48-52 mm Hg; right atrium was mildly dilated and there was mild tricuspid regurgitation.

Cardiac Catheterization: PAP: 30/6/13; PCWP: 2; CO: 3.38; CI: 2.16.

Pulmonary Function Tests: FEV1/ FVC: 74%; FEV1: 0.96 L, 39%; FVC: 1.29L, 42%; TLC: 2.27L, 53%; RV: 1.14L, 79%; DLCO: 3.11L, 57%.

6-Minute Walk Test: Resting SpO2: 86% RA; 92% on 2L/min; 8L/min was required to maintain oxygen saturation above 90% at the end of 6 minutes; the patient was able to walk 211 meters.

Figures


Figure 1: Posterior-anterior (PA) chest radiograph with extensive, poorly defined nodular opacities throughout both lungs and apical bullous disease.

Figure 2: Coronal view of high-resolution chest CT showing extensive bilateral parenchymal calcifications, focal areas of mild emphysematous changes predominantly in the upper lobes, and mild mediastinal and hilar adenopathy.

Figure 3: Bronchoalveolar lavage: Slightly mucoid, tan fluid; cultures revealed no growth; cytology showed no evidence of malignancy; a few white blood cells were noted, predominantly macrophages; laminated calcified concretions and debris were also present. Von Kossa stain positive.

Question 1

What is the most likely diagnosis?

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