Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Questions & Answers

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

Overview

What are the hallmark features of granulomatosis with polyangiitis (GPA)?

What are the general signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the ophthalmic signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the ENT signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the pulmonary signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the musculoskeletal signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are renal signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the PNS signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the CNS signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the cutaneous signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the cardiac signs and symptoms of granulomatosis with polyangiitis (GPA)?

What are the GI signs and symptoms of granulomatosis with polyangiitis (GPA)?

Which routine lab test results suggest granulomatosis with polyangiitis (GPA)?

What is the sensitivity and specificity of ANCA testing in the diagnosis of granulomatosis with polyangiitis (GPA)?

Which radiologic findings suggest granulomatosis with polyangiitis (GPA)?

How is sinus disease assessed in the workup of granulomatosis with polyangiitis (GPA)?

Which pulmonary tests are performed in the workup of granulomatosis with polyangiitis (GPA)?

How is the diagnosis of granulomatosis with polyangiitis (GPA) confirmed?

How is remission of granulomatosis with polyangiitis (GPA) induced?

How is remission of granulomatosis with polyangiitis (GPA) maintained?

What is the historical background of granulomatosis with polyangiitis (GPA)?

What are the classification criteria for granulomatosis with polyangiitis (GPA)?

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

What is limited granulomatosis with polyangiitis (GPA)?

What is severe granulomatosis with polyangiitis (GPA)?

What should patients with granulomatosis with polyangiitis (GPA) be educated about?

What causes granulomatosis with polyangiitis (GPA)?

What is the role of ANCAs in the etiology of granulomatosis with polyangiitis (GPA)?

What is the role of genetics in the etiology of granulomatosis with polyangiitis (GPA)?

What is the role of microbes in the etiology of granulomatosis with polyangiitis (GPA)?

Which environmental factors may have a role in the etiology of granulomatosis with polyangiitis (GPA)?

What is the prevalence of granulomatosis with polyangiitis (GPA)?

Which patient groups have the highest prevalence of granulomatosis with polyangiitis (GPA)?

What is the prognosis of granulomatosis with polyangiitis (GPA)?

What are the possible complications of granulomatosis with polyangiitis (GPA)?

What are the mortality rates of granulomatosis with polyangiitis (GPA)?

What are the most common causes of death in patients with granulomatosis with polyangiitis (GPA)?

Presentation

Which clinical history findings are characteristic of granulomatosis with polyangiitis (GPA)?

Which clinical history findings are characteristic of pediatric granulomatosis with polyangiitis (GPA)?

Which clinical history findings are characteristic of pulmonary involvement in granulomatosis with polyangiitis (GPA)?

How are the musculoskeletal symptoms of granulomatosis with polyangiitis (GPA) characterized?

What is the prevalence of renal disease in granulomatosis with polyangiitis (GPA)?

Which clinical history findings are characteristic of granulomatosis with polyangiitis (GPA)?

What is the prevalence of cutaneous involvement in granulomatosis with polyangiitis (GPA)?

What is the prevalence of cardiac involvement in granulomatosis with polyangiitis (GPA)?

Which physical findings are characteristic of granulomatosis?

DDX

Which conditions are included in the differential diagnoses of granulomatosis with polyangiitis (GPA)?

What are the differential diagnoses for Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)?

Workup

What is the role of lab tests in the workup of granulomatosis with polyangiitis (GPA)?

What is the role of ANCA detection in the workup of granulomatosis with polyangiitis (GPA)?

What is the role of imaging studies in the workup of granulomatosis with polyangiitis (GPA)?

What is the role of sinus radiography in the workup of granulomatosis with polyangiitis (GPA)?

What is the role of sinus CT in the workup of granulomatosis with polyangiitis (GPA)?

What is the role of pulmonary testing in the workup of granulomatosis with polyangiitis (GPA)?

What is the role of bronchoscopy in the workup of granulomatosis with polyangiitis (GPA)?

What is the role of biopsy in the workup of granulomatosis with polyangiitis (GPA)?

Which histologic findings are characteristic of granulomatosis with polyangiitis (GPA) in the lungs?

Which histologic findings are characteristic of granulomatosis with polyangiitis (GPA) in the kidney?

Which histologic findings are characteristic of granulomatosis with polyangiitis (GPA) in the skin?

Treatment

How is granulomatosis with polyangiitis (GPA) treated?

How is granulomatosis with polyangiitis (GPA) graded?

Which activity modification are used in the treatment of granulomatosis with polyangiitis (GPA)?

Which specialist consultations are beneficial to granulomatosis with polyangiitis (GPA)?

How is granulomatosis with polyangiitis (GPA) prevented?

What is the role of cyclophosphamide in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of 2-mercaptoethanesulfonate sodium (Mesna) in the treatment of granulomatosis with polyangiitis (GPA)?

What is included in the monitoring of patients receiving cyclophosphamide for the treatment of granulomatosis with polyangiitis (GPA)?

What are the effects on fertility of cyclophosphamide treatment for granulomatosis with polyangiitis (GPA)?

What is the role of rituximab in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of corticosteroids in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of trimethoprim-sulfamethoxazole in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of plasma exchange in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of methotrexate in the treatment of granulomatosis with polyangiitis (GPA)?

Which medications are used of remission maintenance of granulomatosis with polyangiitis (GPA)?

What is the role of azathioprine in the remission maintenance of granulomatosis with polyangiitis (GPA)?

What is the role of methotrexate in the remission maintenance of granulomatosis with polyangiitis (GPA)?

What is the role of leflunomide in the remission maintenance of granulomatosis with polyangiitis (GPA)?

What is the role of rituximab in the remission maintenance of granulomatosis with polyangiitis (GPA)?

What should be considered in the remission maintenance of granulomatosis with polyangiitis (GPA)?

What is the role of IV immunoglobulin (IVIG) in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of mycophenolate mofetil (MMF) in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of etanercept in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of infliximab in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of 15-deoxyspergualin in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of antithymocyte globulins in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of alemtuzumab in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of abatacept in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of HSCT in the treatment of granulomatosis with polyangiitis (GPA)?

What is included in the cyclophosphamide regimen to induce remission in severe or generalized granulomatosis with polyangiitis (GPA)?

What is included the rituximab regimen to induce remission in severe or generalized granulomatosis with polyangiitis (GPA)?

What is included the glucocorticoid regimen to induce remission in severe or generalized granulomatosis with polyangiitis (GPA)?

What is included in the methotrexate regimen to induce remission in localized or milder granulomatosis with polyangiitis (GPA)?

What is the methotrexate regimen for the remission maintenance of granulomatosis with polyangiitis (GPA)?

What is included the glucocorticoid regimen to induce remission in localized or milder granulomatosis with polyangiitis (GPA)?

How long are remission maintenance medications given for granulomatosis with polyangiitis (GPA)?

What is azathioprine regimen for the remission maintenance of granulomatosis with polyangiitis (GPA)?

What is the leflunomide regimen for the remission maintenance of granulomatosis with polyangiitis (GPA)?

What is the glucocorticoids regimen for the remission maintenance of granulomatosis with polyangiitis (GPA)?

Which adjunctive therapies are used in the treatment of granulomatosis with polyangiitis (GPA)?

What is the role of surgery in the treatment of granulomatosis with polyangiitis (GPA)?

What is included in the long-term monitoring of granulomatosis with polyangiitis (GPA)?

Medications

What is the role of medications in the treatment of granulomatosis with polyangiitis (GPA)?

Which medications in the drug class Antibiotics are used in the treatment of Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)?

Which medications in the drug class Antineoplastics, Monoclonal Antibody are used in the treatment of Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)?

Which medications in the drug class Immunosuppressants are used in the treatment of Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)?

Which medications in the drug class Antineoplastics, Alkylating are used in the treatment of Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)?

Which medications in the drug class Corticosteroids are used in the treatment of Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)?