We report a case of classic polyarteritis nodosa complicated with renal infarction. A 14-year-old boy manifested fever, abdominal pain, watery and bloody diarrhea, and weight loss. Laboratory findings indicated anemia, increased levels of C-reactive protein, and erythrocyte sedimentation rate. Lower gastrointestinal endoscopic examination revealed multiple colorectal ulcerations, and histopathological findings were non-specific, suggesting gastrointestinal involvement of Behcet disease. The patient was referred to our hospital, and suspected to have vasculitis syndrome since the abnormal laboratory findings included persistently increased levels of FDP-E/fibrin monomer as well as inflammatory markers, and the extraordinary high excretion of beta 2-microglobulin, which indicated abrupt and massive expression of HLA class I molecule on endothelial cells due to interferon-gammanemia. To examine the site of vasculitis, 3D-CT angiography was applied to demonstrate bilateral renal infarction and renal artery microaneurysms. Together with the clinical, laboratory, and 3D-CT angiographic findings, he was finally diagnosed as having classic polyarteritis nodosa. After 12 month-course of intravenous cyclophosphamide pulses and prednisolone/azatioprine therapy, complete disappearances of inflammatory manifestations, and renal infarction and microaneurysms were documented. The diagnosis of classic polyarteritis nodosa is frequently delayed because both clinical symptoms and signs, and laboratory findings are not disease-specific, but early diagnosis and treatment are necessary to prevent serious organ damage. In addition to the precise estimation of laboratory findings such as inflammatory markers, and FDP-E/D-dimer/fibrin monomer, the newly developed 3D-CT angiography, a less invasive imaging technique, will be helpful to diagnose patients with classic polyarteritis nodosa, and intervene the disease progression with early and active treatment.
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http://dx.doi.org/10.2177/jsci.31.415 | DOI Listing |
Cardiovasc Pathol
November 2024
Department of Precision and Regenerative Medicine and Jonian Area, Pathology Unit, University of Bari, Bari, Italy.
Semin Neurol
February 2025
Department of Neurology, Virginia Commonwealth University, Richmond, Virginia.
Z Rheumatol
September 2024
Institut für Neuropathologie, Charité - Universitätsmedizin, Berlin, Deutschland.
Rheumatology (Oxford)
September 2024
Department of Pediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK.
Cureus
April 2024
Department of Ophthalmology, Hospital Ampang, Selangor, MYS.
Antinuclear cytoplasmic antibody (ANCA)-related scleritis is a potentially sight-threatening inflammatory condition that may occur as a primary vasculitis disorder or as a secondary vasculitis in a variety of inflammatory conditions. While ANCA has been classically associated with primary vasculitis diseases such as granulomatosis with polyangiitis (GPA), microscopic polyarteritis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), it is interesting that in cases of lupus spectrum disease (LSD), both ANCA and atypical p-ANCA have been observed as secondary autoantibodies. Scleritis is a rare ocular manifestation of lupus disease with an incidence of around 1%.
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