Peripheral Pulmonary Artery Stenosis (PPAS) Mimicking Chronic Thromboembolic Pulmonary Hypertension (CTEPH) in SAPHO Syndrome: A Case Report and Review of the Literature

Background: Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare systemic auto-inflammatory disease characterized by chronic nonbacterial osteomyelitis and neutrophilic cutaneous involvement.

Case Presentation: We report a case of an 83-year-old man with SAPHO syndrome complicated by pulmonary hypertension attributed to multiple perfusion defects on a ventilation-perfusion scan, suggestive of chronic thromboembolic pulmonary hypertension (CTEPH). Computed tomography angiography revealed peripheral pulmonary artery stenosis (PPAS). He also demonstrated pyoderma gangrenosum (PG) as skin manifestation. Our lethal case of a patient with SAPHO syndrome presented with pulmonary hypertension due to PPAS and PG.

Conclusions: SAPHO syndrome can result in heterogeneous phenotypes as systematic auto-inflammatory diseases.

Keywords: Chronic Thromboembolic Pulmonary Hypertension (CTEPH), Peripheral Pulmonary Artery Stenosis (PPAS), SAPHO syndrome.