PO:37:266 | Long-term manifestation of graft-versus-host disease presenting as a vasculitic-like syndrome: a rare case report

Camilla Mazzanti¹, Gianluca Santoboni¹, Claudio Angrisani¹, Massimiliano De Simone¹, Giuliana Marciani¹, Alessandra Fiorentini¹ | ¹Ospedale Santa Rosa Viterbo, Italy

Introduction. Graft-versus-host disease (GVHD) is a common complication following allogenic hematopoietic stem cell transplantation, tipically manifesting within the first few months post-transplant.However, its presentation as a long-term, delayed manifestation is exceedingly rare. We report a unique case of a patient who developed a vasculitis-like syndrome several years after transplantation, ultimately diagnosed as a manifestation of chronic GVHD. This case underscores the importance of considering GVHD in the differential diagnosis of vasculitic syndromes in transplant recipients, even many years post-procedure.

Clinical Case. The patient is a 71-year-old woman admitted with persistent abdominal pain and constipation lasting several days. Her medical history is complex, including treatment for acute myeloid leukemia with chemotherapy and stem cell transplant about eight years earlier, along with graft-versus-host disease managed with corticosteroids and infliximab. She also has a thoracic aortic aneurysm treated with endovascular stenting, dyslipidemia, and type 2 diabetes. Initial tests showed high white blood cell count (WBC 19,900) with a predominance of neutrophils, and elevated C-reactive protein (CRP 90.9 mg/L), indicating inflammation. Imaging studies, including MRI and contrast-enhanced CT scans, revealed a dilated thoracoabdominal aorta (35 mm diameter) with thickened, irregular walls and signs of ulceration, suggesting aortitis. The patient underwent urgent endovascular repair. Follow-up angiography showed some stenosis in the arteries, which was managed with additional stenting. Eight months later, re-evaluation revealed ongoing inflammation, with lab tests showing elevated CRP (26.2 mg/L), ESR (112 mm/h), and fibrinogen (80). PET-CT scans showed mild radiotracer uptake near the splenic artery and along the endograft, indicating persistent aortitis or periaortitis. Based on these findings, a diagnosis of large vessel vasculitis was considered. Due to the patient’s diabetes, which made high-dose corticosteroids risky, immunomodulatory therapy with tocilizumab (162 mg weekly subcutaneously) was started. This case highlights the importance of a multidisciplinary approach in managing complex vasculitis. Discussion This case is particularly noteworhty because it illustrates a rare, delayed presentation of GVHD as a vasculitis-like syndrome occurring several years after transplantation. While GVHD typically manifests within the first 100 days post-transplant, late-onset GVHD can occur and may involve various organs. Its presentation as a vasculitic processi s uncommon and can pose diagnostic challenges. This case emphasizes the need for clinicians to mantain a high index of suspicion for GVHD in transplant recipients presenting with vasculitic symptoms, regardless of the time elapsed since transplantation. Recognizing this atypical manifestation is crucial for timely diagnosis and appropriate management.

Conclusions. Long-term GVHD can presenta s a vasculitis-like syndrome years after allogenic stem cell transplantation. Awareness of this rare manifestation is essential for accurate diagnosis and optimal patient care.