“The Great Masquerader”: A Case of an Uncommon and Frequently Delayed Diagnosis

1University of California, San Francisco, San Francisco, CA
2UCLA, Los Angeles, CA

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 224

Case Presentation:

A 56‐year‐old Mexican woman with no prior medical history presented to an outside hospital with otitis media refractory to antibiotics, culture‐negative mas‐toiditis, and a facial nerve palsy that resolved with steroids. After transfer to our hospital for surgical debridement, she developed hemoptysis and fevers to 38.6°C. Chest CT revealed predominantly upper‐lobe centrilobular pulmonary nodules that persisted despite antibiotic treatment for presumed hospital‐acquired pneumonia. Sputum cultures, fungal serologies, and acid‐fast bacilli (AFB) smears were unrevealing, and a bronchoscopy was performed and sent for bacterial, AFB, and fungal cultures, which were also negative. Because of the patient's tuberculosis (TB) exposure history and symptoms, she was started on empiric rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy while awaiting sputum AFB cultures. She subsequently developed gross hematuria, and her creatinine rose to 1.86. C‐ANCA and PR3 antibodies were positive. Renal biopsy revealed pauci‐immune crescentic glomerulonephritis, confirming a diagnosis of Wegener's granulomatosis in this clinical context. Nearly 2 months after the patient's initial presenting symptoms, cyclophosphamide and predni‐sone were initiated, and RIPE therapy was discontinued. Her hospital course was complicated by bilateral lower‐extremity DVTs while off subcutaneous heparin for a renal biopsy; warfarin was started. She has been closely followed as an outpatient and has experienced resolution of symptoms and tolerated treatment well.

Discussion:

Wegener's granulomatosis (WG) is a systemic small‐vessel vasculitis with a prevalence of 1 in 20,000. It is most prevalent in whites, with a mean age of 50 years at diagnosis. Clinical presentation often includes a prodrome with symptoms mimicking sinusitis, otitis, or upper respiratory infections unresponsive to antibiotics. Nodular or cavitary lung lesions are common and may be initially misdiagnosed as mycobacterial or fungal infection or malignancy. Rapidly progressive glomerulonephritis and nonspecific constitutional symptoms (fevers, myalgias, and weight loss) are other common presenting findings. Before the correct diagnosis is reached, patients often receive antibiotics, surgeries, and other misguided interventions because WG can initially masquerade as other more common diseases. In severe WG, c‐ANCA has a sensitivity and specificity more than 90%. However, a biopsy is needed to secure the diagnosis. Lung biopsies have the highest yield but are more invasive than kidney biopsies, which are nonspecific but can confirm the diagnosis in the appropriate clinical context with a positive c‐ANCA. The incidence of venous thrombosis is increased in WG above that of patients with lupus and rheumatoid arthritis.

Conclusions:

We report this case to increase awareness of WG as a disease that can mimic otitis media and TB and to highlight the importance of prophylactic anticoagulation in hospitalized WG patients with active disease.

Disclosures:

I. Ahronowitz ‐ none; K. Martinez ‐ none; S. Vallabhaneni ‐ none; C. Lau ‐ none

To cite this abstract:

Ahronowitz I, Martinez K, Vallabhaneni S, Lau C. “The Great Masquerader”: A Case of an Uncommon and Frequently Delayed Diagnosis. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 224. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/the-great-masquerader-a-case-of-an-uncommon-and-frequently-delayed-diagnosis/. Accessed September 19, 2019.

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