What is included in the long-term monitoring of 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

Patients with GPA should have regularly scheduled follow-up visits with the physician primarily responsible for managing his or her disease. Since recurrences occur frequently, patients should be monitored closely clinically, with radiologic studies and laboratory examinations that include renal function, erythrocyte sedimentatoin rate (ESR), ANCA levels, and urinalysis. Visits should also focus on untoward effects of therapeutic agents.

Infection is a major contributor to morbidity and mortality in GPA and often mimics a disease flare or manifests as atypical features caused by treatment-related immunosuppression. Providers should have a low threshold for treating suspicious symptoms with antibiotics.

Prophylaxis against Pneumocystis pneumonia is essential while patients are receiving conventional therapy for GPA. This can be achieved with TMP-SMZ single-strength once daily or double-strength formulation three times per week. Dapsone 100mg daily can be used in sulfa-allergic patients.

All patients who require long-term glucocorticoid treatment are at risk for glucocorticoid-induced osteoporosis. Baseline bone mineral density should be evaluated. If the density is normal, patients should take daily calcium and vitamin D supplementation. If the density is diminished at baseline or if long-term glucocorticoid use is anticipated, bisphosphonate therapy should be added. Risk versus benefit of bisphosphonate therapy should be discussed with women of childbearing age.


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