ABSTRACT
Granulomatosis with polyangiitis is a systemic, small vessel vasculitis associated with the anti-neutrophil cytoplasmic antibody. We herein report a case of granulomatosis with polyangiitis with paravertebral lesions. A 69-year-old man presented to our hospital with fever, back pain, and myalgia. A computed tomography scan showed multiple lung nodules, while magnetic resonance imaging revealed soft tissue shadows around a thoracic vertebral lesion. A laboratory examination revealed positive myeloperoxidase anti-neutrophil cytoplasmic antibody. He was diagnosed with granulomatosis with polyangiitis. He was treated with oral glucocorticoid and intravenous cyclophosphamide, and the shadows resolved. Physicians should consider granulomatosis with polyangiitis in cases with paravertebral lesions.
Subject(s)
Granulomatosis with Polyangiitis , Aged , Granulomatosis with Polyangiitis/diagnosis , Humans , Male , Spinal Diseases , Thoracic Vertebrae/pathologyABSTRACT
A previous case report of colitis and serine proteinase 3-antineutrophil cytoplasmic antibody positivity in pyogenic arthritis, pyoderma gangrenosum (PG), acne and hidradenitis suppurativa (PAPASH) syndrome with colitis has been published. Herein, we report a similar case of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity. A 26-year-old man presented with recurrent aseptic pyogenic arthritis, acne, hidradenitis suppurativa and PG. Lower gastrointestinal endoscopy was performed, and colitis was observed. No PSTPIP1 gene mutation was found in the gene-sequencing test. Based on these findings and prior case reports, we diagnosed the patient with PAPASH syndrome, a PAPA spectrum disorder complicated by colitis. This patient had PAPASH syndrome with colitis and was MPO-ANCA and anticardiolipin antibodies-positive; it is unclear whether these antibodies play a role in this disease, but it may provide clues to further elucidate its pathogenesis.
