Fever of Unknown Origin With Angina
An Atypical Presentation of Polyangiitis With Granulomatosis
A 30-year-old patient was admitted to the hospital with fever up to 40°C, arthralgia, a large 7×10×5-cm subcutaneous abscess in the neck, and multiple truncal abscesses. He had experienced several sterile subcutaneous abscesses for years. On clinical examination, there were tender erythematous, hemorrhagic papules, and nodules on the hands (Figure [A]) and purpura on the lower legs (Figure [B]). Laboratory examinations showed a C-reactive protein level of 250 mg/L and normal leukocyte counts. Computed tomography scans revealed splenomegaly with a longitudinal diameter of 17 cm and multiple focal contrast media enhancements suggestive of splenic abscesses (arrows in Figure [C]). Slight sinusitis maxillaris was found (Figure [D]), but no pulmonary changes were detected in the x-ray (Figure [E]) and computed tomography (data not shown). The large nuchal abscess was removed surgically, and the patient was treated with several antibiotic regimens, including piperacillin, fusidic acid, and moxifloxacin. Nevertheless, the undulating fever persisted. Serial blood and smear cultures remained sterile.
In parallel, the patient developed chest pain with highly elevated troponin T (493 µg/L) and N-terminal pro-brain natriuretic peptide (10 041 pg/mL) but normal creatinine kinase and normal consecutive ECGs (Figure [F]). Transesophageal echocardiography was performed and showed a normal structure and function of the valves without vegetations. Transthoracic echocardiography revealed a reduced left ventricular ejection fraction (45%) resulting from diffuse left ventricle hypokinesia and a pericardial effusion up to 1 cm (Movies I and II in the online-only Data Supplement). Cardiac magnetic resonance tomography revealed a granular pattern of late enhancements in the myocardium (Figure [G] and Movies III and IV in the online-only Data Supplement).
Re-evaluation of this case of fever of unknown origin resulted in further examinations, in which highly elevated cytoplasmic anti-neutrophil cytoplasmic antibody titers (1:2500) were found. Urine analysis showed hematuria and proteinuria of 0.5 g/g creatinine without pathological elevation of serum creatinine. Histopathological evaluation of the nuchal abscess revealed necrotizing and granulomatous inflammation with multinucleated giant cells (Figure [H]). Special stains did not detect fungi and acid-resistant bacilli. Adjacent blood vessels showed chronic vasculitis (Figure [I]). Finally, a kidney biopsy revealed Pauci-immune glomerulonephritis with crescent formation (Figure [J]).
In summary, this patient with fever of unknown origin and angina experienced polyangiitis with granulomatosis (Wegener granulomatosis). He was treated with high-dose corticosteroids and cyclophosphamide, resulting in immediate resolution of fever and prompt physical improvement with stabilization of cardiac function. In line with a previous report,1 we conclude that cardiac involvement in polyangiitis with granulomatosis can be screened by performing echocardiography, but the most sensitive method to evaluate cardiac involvement is magnetic resonance tomography showing late enhancements in the myocardium.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.112.136754/-/DC1.
- Received August 16, 2012.
- Accepted December 26, 2012.
- © 2013 American Heart Association, Inc.