Vasculite c-ANCA relacionada em paciente com retocolite ulcerativa: relato de caso / c-ANCA associated vasculitis in patients with ulcerative colitis: case report

As manifestações pulmonares da retocolite ulcerativa (RCU) são raras e incluem inflamação de pequenas e grandes vias aéreas, doença parenquimatosa e serosite, entre outras. Uma proporção substancial de pacientes com doença inflamatória intestinal, particularmente aqueles com RCU, apresenta ANCA positivo, a maioria padrão p-ANCA. Apresentamos um caso de paciente com RCU, com c-ANCA positivo, que evoluiu com hemoptise, associada a alterações radiológicas compatíveis com vasculite pulmonar.

The pulmonary manifestations of ulcerative colitis (UC) are rare and include inflammation of small and large airways, parenchymal disease and serositis among others. A substantial proportion of patients with inflammatory bowel disease, particularly those with ulcerative colitis presents positive ANCA, most p-ANCA pattern. We present a case of patient with ulcerative colitis, with positive c-ANCA, which progressed to hemoptysis associated with radiological findings consistent with pulmonary vasculitis.

Vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos: avances en patogenia y tratamiento / Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: advances in pathogenesis and treatment

ANCA (anti-neutrophil cytoplasmic antibodies) associated vasculitis or AAVare a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum. Due to these common features, it is considered that AAV share pathogenic mechanisms. Consequently, a similar therapeutic approach has been developed. A new nomenclature has been recently proposed, with AAV including "granulomatosis with polyangiitis" (GPA, formerly Wegener's granulomatosis), "microscopic polyangiitis" (MPA), "eosinophilic granulomatosis with polyangiitis" (EGPA, formerly Churg-Strauss disease) and renal limited vasculitis. Research on AAV has shown significant advances in the last two decades, including advances in the knowledge of pathogenic mechanisms such as the pro-inflammatory role of ANCA, AAV murine models and genetic links. Evidence on previous immunosuppressive therapies has improved significantly and new, promissory drugs have been introduced, Rituximab being the most important. We review the advances of pathogenic mechanisms and treatment for these diseases.