Granulomatosis with Polyangiitis (GPA), an Important Differential Diagnosis in Sepsis
Delme Luff
Anaesthetic CT2, Tunbridge Wells Hospital, Tunbridge Wells, Kent, United Kingdom.
Priyakam Chowdhury
Anaesthetic SpR, Queen Elizabeth Hospital, Woolwich, London, United Kingdom.
Sancho Rodríguez-Villar *
Critical Care Consultant, Queen Elizabeth Hospital, Stadium Road, Woolwich, London, United Kingdom.
*Author to whom correspondence should be addressed.
Abstract
Introduction: Sepsis is a well-recognised medical emergency requiring aggressive medical management, often in a critical care setting. Granulomatosis with Polyangiitis (GPA) (previously named Wegener’s Granulomatosis) is a type of primary systemic antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) [2-4]. AAV can present with a variety of symptoms including those which mimic infective disease. Differentiation between these two diseases is extremely important because their respective treatments differ significantly and delays in commencing therapy can be fatal.
Case Presentation: A 70 year old Caucasian gentleman presented with symptoms suggestive of severe sepsis who failed to respond to standard treatment. However an autoimmune screen revealed that he was in fact suffering from severe generalised GPA. Despite aggressive treatment his disease progressed rapidly and he passed away on day 20 of his admission.
Conclusion: This case report describes a rare and potentially fatal disease which can present with a variety of non specific symptoms. It highlights the importance of differentiating between AAV and syndromes that may mimic AAV, particularly infection (including meningitis) in the ICU settings.
Keywords: Sepsis, granulomatosis with polyangiitis, antineutrophil cytoplasmic antibodies, ANCA-associated vasculitis.