CASE STUDY QUESTION 01: WHAT WOULD ESTABLISH THE CAUSE OF THIS WOMAN’S COUGH, DYSPNEA AND FEVERS?

TITLE: WHAT WOULD ESTABLISH THE CAUSE OF THIS WOMAN’S COUGH, DYSPNEA AND FEVERS?

(SCROLL TO BOTTOM FOR THE ANSWER)

A 57-year- old woman presents to the emergency department with 3 months of a progressive nonproductive cough, dyspnea, and subjective fevers. She denies any hemoptysis or rashes and has not traveled recently.

During physical exam, you find a depression of the nasal dorsum as seen in image 1. A chestradiograph (CXR) reveals numerous bilateral large nodules and masses, with possible cavitation (see image 2). A computed tomography (CT) scan of the chest again demonstrates the cavitary lesions, which are scattered through the lungs within all lobes (see image 3). A bronchoscopy with bronchoalveolar lavage is performed, collecting samples as shown in image 4.

Which one of the following is most likely to help establishing the cause?

1) Workup for cocaine abuse 2) VDRL and FTA-abs titers 3) Antiproteinase 3 antibody levels 4) Test sputum for acid-fast bacilli

Answer:

Systemic vasculitis, particularly GPA, is highest on the differential diagnosis at this point. Antiproteinase-3 antibodies are highly specific for GPA in the appropriate clinical setting. GPA (formerly Wegener’s granulomatosis) is a small vessel vasculitis (part of the ANCA-associated vasculitides) that typically produces granulomatous inflammation of the respiratory tracts as well as a necrotizing, pauci-immune glomerulonephritis in the kidneys. Although this patient does not have renal manifestations of disease, she definitely has the cavitary lung lesions that are typically seen in GPA. If left untreated, persistent upper respiratory tract inflammation may lead to saddle nose deformity (see Fig. 53.4), nasal septal perforation, and even hearing loss. Despite the high likelihood of GPA, it is prudent to consider infectious etiologies as well. Not only can this patient’s clinical manifestations of vasculitis mimic infection (i.e., fevers, cough, elevated inflammatory markers, cavitary lung lesions), but sometimes infections and vasculitis can occur simultaneously. Saddle nose is also associated with congenital syphilis and cocaine abuse, but this patient has other signs and symptoms that place these conditions lower in our differential diagnosis.

Thank you Dr. Leyla Bojanini for preparing this case!