429. Severe cytomegalovirus colitis in a patient with systemic vasculitis: a rare complication
Creators: name: Maria Helena Lourenço1, 2
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case</strong>: A 78-year-old woman was admitted to the emergency room due to abdominal pain, nausea and vomiting. She had a previous diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), with asthma, sinusitis, bullous hemorrhagic vasculitis, symmetric and additive polyarthritis of hands, elbows and tibiotarsal joints, sensorimotor polyneuropathy and peripheral eosinophilia. She was medicated with prednisolone (PDN) 20mg/day (weaning scheme – initial dosage 1mg/kg/day) and cyclophosphamide (2 cycles; CYCLOPS protocol) (previous intolerance to methotrexate), with clinical remission. <strong>Diagnostic Testing</strong>: Blood analysis revealed microcytic hypochromic anemia (hemoglobin 10.3 g/dL) and a raised C-reactive protein (8.5 mg/dL). Abdominal computed tomography scan showed colonic thickening, with perforation of the anorectal transition and extraperitoneal air. The patient underwent surgery, during which a bleeding rectal ulcer was detected. Biopsies were performed, followed by a terminal colostomy and a course of broad-spectrum antibiotics. <strong>Differential &amp; Final Diagnosis:</strong> Cancer was one of the main differential diagnoses considered, due to the patient’ age, symptoms and previous diagnosis (vasculitis may have a tumoral origin). Infectious causes were considered, namely tuberculosis, as the interferon-gamma release assay was positive. However, histological examination of the ulcer showed enlarged cells with thickened nuclear membrane and intranuclear and intracytoplasmic inclusions, typical of cytomegalovirus (CMV) infection and immunohistochemistry was positive to CMV infection. CMV viral load (VL) was 254 IU/mL, compatible with disseminated CMV disease. Two months before, CMV serologies had revealed positive M and G immunoglobulins, with CMV VL &lt;178 IU/mL. No signs or symptoms of other CMV organic disease were documented. <strong>Discussion of Management:</strong> Treatment with ganciclovir (5mg/kg 12/12h) was performed for 21 days, after which CMV VL was undetectable. Secondary prophylaxis with valganciclovir 900mg/day was started in conjunction with the vasculitis treatment (PDN 20mg/day), with no CMV disease recurrence. Isoniazid 300mg/day and pyridoxin 40mg/day were also initiated due to latent tuberculosis diagnosis. <strong>Conclusions:</strong> EGPA is a vasculitis of small- and medium-sized vessels characterized by lung, paranasal sinus, skin, kidney, nervous system and joints involvement, associated with peripheral eosinophilia. It is a potential life-threatening disease. Immunosuppressive drugs are the mainstay of treatment. Glucocorticoids, cyclophosphamide and rituximab induce remission of the severe forms of disease, but also predispose patients to opportunistic infections. CMV infection is common and usually benign in immunocompetent hosts. However, in immunocompromised patients, reactivation can occur, carrying poorer outcomes. Colitis is a rare complication mainly reported in patients with human immunodeficiency virus and transplant recipients, with scarce data in ANCA-associated vasculitis (AAV). There is sparse data regarding prophylactic or preemptive treatment of CMV infection in patients with AAV. This case reinforces the need of guidelines concerning surveillance and prophylaxis of CMV infection in these patients. <strong>Disclosures: </strong>None.