A 55-year-old man was transferred to our hospital for further evaluation of altered mental status and fusiform cerebral aneurysms.
History of present illnessA 55-year-old African American male was in good health 5 months prior when he underwent a colonoscopy as part of a routine colon cancer screening. After the procedure, he developed colitis and was treated with antibiotics for 5 days with resolution of his symptoms. One month after this episode he was admitted at an outside hospital for constellation of fever, bilateral ankle swelling, and petechial pruritic rash on his lower extremities. The patient did not experience other symptoms such as weight loss, cough, hemoptysis, painful vision loss, oral ulcers, neurologic deficits, or gastrointestinal bleeding. During this hospitalization, the patient was found to have an elevated creatinine level of 2.65 mg/dl. Additional laboratory values were hemoglobin (9.8 gm/dl), white blood cells (5,000/ll), platelets (200,000/ll), normal liver enzymes, and low albumin (2.8 mg/dl). The serologic studies revealed elevated rheumatoid factor (RF; 114 IU/ml, normal value <20 IU/ml) and low complements C3 (49 mg/dl, normal value 80-252 mg/dl) and C4 (9 mg/dl, normal value 12-17 mg/dl), while other serologic markers, antineutrophil cytoplasmic antibody (ANCA), proteinase 3 (PR3) antibody, myeloperoxidase (MPO) antibody, antinuclear antibody (ANA), and cryoglobulins, were reported to be negative. His urinalysis revealed red blood cell (RBC) casts, dysmorphic RBCs, and 2+ protein. Given his presentation with constitutional symptoms, rash, and elevated creatinine with subnephrotic range proteinuria and casts, he underwent a kidney biopsy, the findings of which revealed "necrotizing glomerulonephritis with crescents, pauci-immune staining pattern with glomerular C3 dense deposit, which could be seen in infectious process-like endocarditis/abscesses." The patient was diagnosed with ANCA-associated vasculitis (AAV) based on a pauciimmune staining pattern and cresentric glomerulonephritis on his kidney biopsy results, and he was started on 60 mg of daily oral prednisone and 100 mg of daily oral cyclophosphamide. The patient continued these doses of prednisone and cyclophosphamide for 3 months without significant improvement in his proteinuria (persistently 2+ on urine dipstick, 1.5 gm on quantification) and creatinine levels (remained elevated in range 1.6-2.2 mg/dl).The patient was admitted again at an outside hospital for evaluation of shortness of breath and chest tightness. He was found to have mild elevation in cardiac enzymes and underwent cardiac catheterization as part of cardiac assessment. Soon after cardiac catheterization, he became delirious and was found to have an intracranial hemorrhage. An angiogram at the outside hospital highlighted an intracranial aneurysm; he was transferred to our hospital for embolization of the aneurysm. Upon arrival, he was intubated for airway protection and underwent repeat angiogram with demonstration of 2 fusiform aneurysms.
Medical and famil...