What are the clinical differences between limited and severe granulomatosis with polyangiitis (GPA)?

Updated: Oct 09, 2019
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

GPA is one of the ANCA-associated vasculitides (AAVs) and has a predilection for the upper and lower respiratory tracts and the kidneys. It has a spectrum of clinical presentations and may be divided broadly into limited or severe disease.

Individuals with limited GPA present with clinical findings largely isolated to the upper and lower respiratory tracts and are generally not considered to have organ- or life-threatening disease. Persons with severe disease present with significant multisystem manifestations that may involve the lungs, kidneys, and other organs, in addition to the respiratory tract. Severe disease can also be described as generalized disease.

Consensus does not exist as to whether limited GPA represents early severe disease or an altogether separate clinical entity. The terminology, limited versus severe, can sometimes be problematic because pulmonary and/or renal involvement may be absent at the onset of symptoms.

Longitudinal follow-up of the National Institutes of Health GPA cohort (158 patients who were observed for 6 mo to 24 y) demonstrated that 18% of patients initially had renal disease and that 77% had glomerulonephritis upon later analysis. [3] Thus, patients initially diagnosed with limited GPA may subsequently develop generalized disease with renal involvement.

Analysis of another GPA cohort, that of the Wegener Granulomatosis Etanercept Trial (WGET), suggested that limited disease may be a qualitatively different clinical entity. In the WGET cohort, patients classified as having limited disease had more severe upper respiratory tract damage, were more likely to have flares after remission, and tended to have identical manifestations when they relapsed. [9]

Below is an outline of the differences between limited and severe GPA based on the WGET trial manual of operations.


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