Presentation
BF, a 43-year-old man with a history of attention deficit hyperactivity disorder (ADHD) and bipolar disorder, was in his usual state of excellent health until March 2014, when he experienced sudden onset of severe, sharp, left-sided abdominal pain radiating to the groin. The pain resolved after an hour, but recurred five times over the next week. Each episode was longer in duration, which prompted him to present to the emergency department of another hospital. He did not experience fevers, chills, night sweats, oral or genital ulceration, rash, visual changes, sinus symptoms, dyspnea, hematuria, or extremity weakness or numbness. His medications included lamotrigine without recent changes. His family and social histories were unremarkable.
On exam at the other hospital, he was afebrile and normotensive, with diffuse abdominal tenderness without guarding or rebound. Laboratory workup revealed a normal complete blood count, serum creatinine and urinalysis. Erythrocyte sedimentation rate (ESR) was 54 mm/hr (ref. <13 mm/hr) and
the C-reactive protein (CRP) was 5 mg/dL (ref. <0.5 mg/dL). A CT of the abdomen with oral and intravenous contrast was performed, which revealed a long left renal artery dissection with surrounding fat stranding, multiple left renal infarcts (see Figure 1) and dissections of both common and right external iliac arteries (see Figure 2). The right common iliac artery was noted to have surrounding soft tissue thickening. A CT angiogram of the chest did not demonstrate any abnormalities of the aorta or the great vessels.
In the absence of signs & symptoms suggestive of a specific etiology, the clinician must rely on inflammatory markers & imaging to make the diagnosis.
He was started on prednisone 40 mg twice daily and admitted to the other hospital. Further laboratory testing revealed negative anti-nuclear cytoplasmic antibody (ANCA), anti-nuclear antibody (ANA), cryoglobulins and a normal reaction to purified protein derivative (PPD). Complements, hepatitis B and C serologies, as well as a toxicology panel were also normal. His abdominal pain resolved during admission. A presumptive diagnosis of a large-vessel vasculitis was made.
Over the next month, he did not have recurrent abdominal pain and prednisone was decreased to 20 mg twice daily. A CT repeated six weeks after initial imaging revealed that the left common iliac dissection had extended into the aorta, with surrounding inflammatory changes. Fat stranding and soft tissue thickening surrounding the right common iliac and left renal artery had improved. His prednisone dose was increased to 60 mg daily, and he was referred to the rheumatology clinic at our hospital for evaluation.