Granulomatosis with Polyangiitis

History of Present Illness

A 54-year-old woman with history of asthma and appendiceal adenocarcinoma status post-appendectomy presented to the emergency department with 3 weeks of shortness of breath, sinus congestion, cough productive of green sputum, lower extremity edema, and general malaise. She had been treated with 2 weeks of oral amoxicillin–clavulanate without improvement. She was found to be hypoxemic with oxygen saturation of 89% on room air and multiple opacities on chest x-ray. She had an elevated white blood cell count of 33,680/μL (95% neutrophils). Computed tomography (CT) scan of the chest revealed diffuse alveolar hemorrhages, which were confirmed on bronchoscopy. She was admitted to the hospital and subsequently developed oliguric acute renal failure. A renal biopsy was performed, revealing crescentic glomerulonephritis. She was given 4 days of intravenous (IV) methylprednisolone 1 g/day and one dose of 880 mg IV cyclophosphamide. Routine blood culture grew out yeast, and ophthalmology was consulted to evaluate for possible fungal endophthalmitis.

Fluorescein angiogram was performed to further assess the cause of blot hemorrhages and cotton wool spots ( Fig. 61.2 ).

Questions to Ask

• Do you notice any changes in your vision? Any complaints about photopsias or floaters?

• Have you ever had any blood clots or trouble with blood pressure? Does anyone in your family have any bleeding/clotting disorders or eye problems?

• Do you have diabetes?

She notes no personal or family history of eye disease, bleeding, or clotting issues. She has been noticing a “cloud” over her left eye (OS) vision inferiorly, which has been improving.