Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features

Granulomatosis with polyangiitis (GPA) presents a wide spectrum of manifestations from the common respiratory symptoms to infrequent neurological and cardiac complications. The challenge in diagnosis and management makes the rapidly progressive disorder one of the most challenging dilemmas in clinical medicine.

An extensive electronic literature search was done using multiple databases (e.g. PubMed) from January 2007 to August 2017 to gather information on the etiopathogenesis, clinical presentation, and current treatment management plan of GPA. The language used was English. Search items included 'Granulomatosis with Polyangiitis symptoms,' 'Wegener's Granulomatosis,' and 'Treatment of Granulomatosis with Polyangiitis' for the databases. Inclusion criteria consisted of published articles in the English language which reported on the clinical manifestations of GPA, as well as on the treatment of the disease. Exclusion criteria included articles that: (1) did not specifically report on GPA vasculitis but also focused on discussion of other vasculitis such as MPA or Churg-Strauss; (2) reported on treatment/symptoms of GPA prior to 2007.

This review provides an overview of work undertaken in key areas of GPA research, including its complex multifactorial etiology, clinical manifestations, treatment strategies, and treatment-related adverse effects.

Major advances in the understanding and treatment of GPA over recent decades have contributed to the notable decline in morbidity and mortality of patients. The ultimate goal is an improved prognosis through outcome measures which assesses the disease control with minimal adverse effects of intensive immunosuppressive regimens, an integral part of the clinical approach to improve the quality of life of GPA patients.