Wegener's granulomatosis presenting as fever of unknown origin in an African-American male.

Wegener’s Granulomatosis (WG) is a rare, multisystem disease of the medium and small sized arteries and veins. It most commonly involves the upper respiratory tract, lungs, and kidneys and often presents as chronic fatigue, upper respiratory infection, sinusitis, and otitis media. Symptoms can include fever, weight loss and fatigue, though these are not usually the primary presenting complaints. Development of the disease is highly skewed across ethnicities, with up to 98% of cases being reported in Caucasians. We present the case of a 56-year-old AfricanAmerican male who presented primarily with complaints of uncontrollable fever of unknown origin (FUO) for past two weeks with accompanying sore throat, nasal congestion, night sweats, malaise, and unexplained weight loss of 10 pounds over the past month. Treatment with antibiotics for one week prior to admission showed no relief of symptoms. Chest xray showed focal course markings in the right upper lobe. Urinalysis revealed microscopic hematuria and leukocyturia. Chest and abdominal CT scans revealed a right lower lobe pulmonary nodule and heterogeneous areas of enhancement in the spleen. Head CT revealed right mastoid opification. Labs revealed proteinase-3 antibody titer > 100, which is characteristic of WG. Steroids and cyclophosphamide were started with relief of presenting symptoms. Renal biopsy showed pauci-immune P-ANCA associated crescentic and focally necrotizing glomerulonephritis and vascilitis. This case is unique in that the patient presented with primary complaint of FUO. WG should be considered as a rule-out in cases of uncontrollable FUO, even if none of the classic triad of symptoms is present. Though rare, WG should be considered in cases involving non-Caucasian patients. INTRODUCTION Wegener’s Granulomatosis (WG) is a very rare, multisystemic disease of the medium and small sized arteries and venules (affecting approximately 3 in 100,000 people) [1]. Cases of WG began to be reported in the 1930’s and 1940’s [2-4]. The disease resembles that of infection, but to this day the cause of WG is unknown. Although it most commonly involves the upper respiratory tract, lung and kidneys [1,5], WG can present in a variety of ways. Approximately 85% of patients with the illness present with runny nose and cold-like symptoms [1]. Cases of rarer manifestation have included: uncontrollable, progressively worsening headaches [6]; retinal occlusion, optic neuritis or vision loss [7]; pericarditis or cerebral vasculitis [7]; and mononeuritis multiplex, cranial or sensory neuropathies, mass lesions or pachymeningitis [7]. Accompanying symptoms often include fever, fatigue, and weight loss, though they are rarely the primary presenting complaints of patients with undiagnosed WG. Most patients will also go on to develop acute renal failure, though only about 20% of cases have this symptom at presentation [5]. Even when asymptomatic, microscopic hematuria with dysmorphic red blood cells, red blood cell casts and non-nephrotic proteinuria can be predictors of later development of renal involvement [1,8,9]. We present a unique case of WG with primary complaint of fever of unknown origin.