Granulomatosis with Polyangiitis (Wegener's Granulomatosis)

A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and KIDNEYS. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against MYELOBLASTIN.

Also Known As:

Networked: 2912 relevant articles (217 outcomes, 207 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Microscopic Polyangiitis
2.	Vasculitis (Vasculitides)
3.	Churg-Strauss Syndrome (Allergic Angiitis)
4.	Granulomatosis with Polyangiitis (Wegener's Granulomatosis)
5.	Sarcoidosis (Schaumann Disease)

Experts

1.	Specks, Ulrich: 64 articles (01/2022 - 05/2002)
2.	Guillevin, Loïc: 51 articles (08/2022 - 04/2003)
3.	Merkel, Peter A: 42 articles (01/2022 - 09/2004)
4.	Hoffman, Gary S: 41 articles (01/2019 - 05/2003)
5.	Gross, W L: 40 articles (01/2013 - 03/2000)
6.	Stone, John H: 38 articles (01/2022 - 08/2003)
7.	Pagnoux, Christian: 37 articles (03/2022 - 04/2003)
8.	Kallenberg, Cees G M: 36 articles (01/2018 - 02/2002)
9.	Gross, Wolfgang L: 36 articles (07/2014 - 05/2002)
10.	Langford, Carol A: 31 articles (01/2022 - 01/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Granulomatosis with Polyangiitis:

1.	Rituximab (Mabthera)FDA Link 03/01/2013 - "Rituximab has proven effective in the treatment of complicated granulomatosis with polyangiitis (Wegener's, GPA). " 10/01/2007 - "Rituximab is a well-tolerated, very effective medication for patients with Wegener's granulomatosis. " 11/01/2013 - "Rituximab (RTX) is an anti-CD20 antibody used successfully in granulomatosis with polyangiitis (GPA) for induction and maintenance of remission. " 07/01/2009 - "Recurrence of Wegener's granulomatosis with de novo intraoral presentation treated successfully with rituximab." 12/01/2019 - "Rituximab is recommended to induce remission of severe granulomatosis with polyangiitis (GPA). "
2.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2010 - "While remission is achieved in the majority of Wegener's granulomatosis (WG)-patients with cyclophosphamide, maintenance of remission remains a challenge due to the high rate of relapses. " 02/01/1978 - "A case of generalized Wegener's granulomatosis in childhood: successful therapy with cyclophosphamide." 06/01/1998 - "Treatment with daily oral cyclophosphamide (CY) has improved survival in Wegener's granulomatosis (WG), but is associated with severe and potentially lethal adverse effects. " 05/21/1988 - "Early diagnosis and treatment of Wegener's granulomatosis with cyclophosphamide has considerably improved the prognosis in this previously fatal disease. " 09/01/1999 - "In particular, in contrast to Wegener's granulomatosis, the need for routine cyclophosphamide treatment is unconfirmed and requires further study."
3.	Antineutrophil Cytoplasmic Antibodies (ANCA)IBA 01/01/2017 - "Skewing toward Treg and Th2 responses is a characteristic feature of sustained remission in ANCA-positive granulomatosis with polyangiitis." 10/01/1999 - "The conclusion is that antineutrophil cytoplasmic antibodies status can be a very useful diagnostic adjunct to the evaluation of patients with suspected Wegener's granulomatosis, but is not a substitute for clinical expertise and histopathologic data during the course of providing patient care. " 03/01/1994 - "In cases of Wegener's granulomatosis with salivary symptoms, measurement of salivary gland functional capacity, in addition to the c-ANCA test, may help to monitor disease activity. " 01/01/1993 - "If ANCA-tests are failing in patients with Wegener's granulomatosis or microscopic polyarteritis, markers of activated coagulation are helpful in evaluation of disease activity. " 01/01/1992 - "Serial ANCA titers reverted to normal in only two of the four patients with Wegener's granulomatosis who attained clinical remission. "
4.	Methotrexate (Mexate)FDA LinkGeneric 06/15/2002 - "High rate of renal relapse in 71 patients with Wegener's granulomatosis under maintenance of remission with low-dose methotrexate." 01/01/2008 - "Does the dose of methotrexate influence the rate of relapse in patients with Wegener's granulomatosis?" 11/01/2006 - "We report a case of generalized Wegener's granulomatosis (WG) successfully treated with high-dose methotrexate (MTX) and predonisolone (PSL). " 11/01/2006 - "[A case of refractory Wegener's granulomatosis successfully treated with high-dose methotrexate]." 08/01/2005 - "Methotrexate for Wegener's granulomatosis: what is the evidence?"
5.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2004 - "Treatment of Wegener's granulomatosis, often a life-threatening disease, has greatly improved, considering that before corticosteroids and immunosuppressives were available, the average survival time of patients amounted to no more than 5 months. " 01/01/2020 - "He was diagnosed with wegener granulomatosis (WG) and was treated with corticosteroids and ciclophosphamide. " 01/01/2017 - "It is important to maintain a high index of suspicion for pseudovasculitis because long-term corticosteroid use to treat granulomatosis with polyangiitis can lead to detrimental effects." 04/01/2011 - "A case of Wegener's granulomatosis on corticosteroids and immunosuppressive therapy returned with SP on the same side four months later. " 05/15/2004 - "The proportion of CD4(EM) cells in corticosteroid-dependent patients with systemic lupus erythematosis or Wegener granulomatosis did not differ from patients without cGVHD that received a transplant. "
6.	Azathioprine (Imuran)FDA LinkGeneric 12/06/1969 - "Sustained azathioprine-induced remission in Wegener's granulomatosis." 10/01/2004 - "High-dose intravenous azathioprine pulse treatment in refractory Wegener's granulomatosis." 07/01/2001 - "Azathioprine toxicity mimicking a relapse of Wegener's granulomatosis." 04/01/1975 - "A case of Wegener's granulomatosis is presented with control of lung lesions by corticosteriods and renal lesions by azathioprine. " 02/01/1968 - "Treatment of advanced Wegener's granulomatosis with azathioprine and duazomycin A."
7.	GlucocorticoidsIBA 10/01/1990 - "This study sought to determine the efficacy, toxicity, and immunologic effects of glucocorticoids (GC) and intermittent high-dose intravenous CP ("pulse" CP) in the treatment of 14 patients with Wegener's granulomatosis (WG). " 05/15/2008 - "Effects of glucocorticoids on weight change during the treatment of Wegener's granulomatosis." 11/01/2010 - "We report the case of a 32-year-old patient with Wegener's granulomatosis (WG) associated with a (biopsy - proven) renal inflammatory pseudotumour (IPT) of the left kidney treated by a partial nephrectomy, glucocorticoids and immunosuppressive drugs, in whom a relapse of renal IPT was found 6 years after the diagnosis of the first IPT. " 05/01/2022 - "Among 131 patients (101 granulomatosis with polyangiitis, 30 microscopic polyangiitis), 27 (21%) received no IVMP, 64 (49%) low-moderate and 29 (22%) high-dose IVMP, while 9 (7%) received low, 75 (57%) moderate and 47 (36%) high initial doses of oral glucocorticoids. " 07/01/1990 - "New cavitary lung lesions typical of Wegener's granulomatosis led to treatment with increased glucocorticoid dosage, resulting in rapid resolution of the pancreatitis and pulmonary lesions. "
8.	Prednisone (Sone)FDA LinkGeneric 01/01/2017 - "PC recruitment and Web-based informed consent was compared with CoE recruitment for a randomized controlled trial (RCT) of continuing versus stopping low-dose prednisone for maintenance of remission of patients with granulomatosis with polyangiitis (GPA). " 07/01/2003 - "To report the case of an individual who we suspect has a 30-year history of Wegener granulomatosis treated only with prednisone. " 04/01/2015 - "Complete remission (CR) was defined as Birmingham Vasculitis Activity Score/Wegener's Granulomatosis (BVAS/WG)=0 off prednisone. " 10/01/2014 - "Complete remission was defined as the absence of disease activity measured by a Birmingham Vasculitis Activity Score for Wegener's granulomatosis of 0 and not qualified by the prednisone dosage at the time. " 09/01/2013 - "To determine the most important reason for failure to achieve the primary outcome, 7 hierarchical categories of reasons were defined retrospectively (uncontrolled disease, adverse event leading to therapy discontinuation, severe flare, limited flare, Birmingham Vasculitis Activity Score for Wegener's Granulomatosis >0, prednisone treatment at any dosage, and other). "
9.	Immunosuppressive Agents (Immunosuppressants)IBA 05/01/1978 - "It is important to recognize these unusual renal manifestations as features of Wegener's granulomatosis because of the therapeutic efficacy of cytotoxic immunosuppressive agents in this disease." 01/01/2002 - "The prognosis of Wegener's granulomatosis (WG) improved following the introduction of immunosuppressant agents. " 07/01/2016 - "Given that these findings met the diagnostic criteria for granulomatosis with polyangiitis (GPA), he was treated with immunosuppressive agents. " 09/01/2003 - "We describe the fourth reported case of PML in a patient receiving immunosuppressants for Wegener's granulomatosis. " 05/01/1994 - "Wegener's granulomatosis (WG) is a potentially fatal disease in which early diagnosis and administration of immunosuppressive agents is essential to successful treatment. "
10.	Prednisolone (Predate)FDA LinkGeneric 11/29/2017 - "Later that year, he was also diagnosed with granulomatosis with polyangiitis and was treated with prednisolone. " 01/01/2009 - "Standard treatment for Wegener's granulomatosis involves a combination of prednisolone and cylophosphamide, however biologic agents are now being used in refractory cases. " 02/01/2010 - "He had been treated with long-term predonine therapy after the initial onset of Wegener's granulomatosis. " 01/01/2022 - "Granulomatosis with polyangiitis diagnosed during the treatment of otitis media with prednisolone in a patient with anti-neutrophil cytoplasmic antibody-associated vasculitis: A case report." 01/01/1991 - "Immunosuppressive treatment with cyclophosphamide and prednisolone has markedly improved the prognosis of Wegener's granulomatosis (WG). "

Therapies and Procedures

1.	Therapeutics 03/01/1988 - "The remarkable efficacy of this double therapy confirms the diagnostic hypothesis of Wegener's granulomatosis." 01/01/1993 - "In conclusion MTP pulse therapy as the initial treatment may be beneficial in treating patients with Wegener's granulomatosis." 12/01/2005 - "Medical therapy is the mainstay of treatment in Wegener's granulomatosis, whereas surgery is reserved for selected head and neck manifestations." 05/01/2003 - "Anti-inflammatory therapy specifically antagonizing the proinflammatory effect of IL-1beta may be a promising treatment for patients with Wegener's granulomatosis with renal manifestations." 11/01/2011 - "The aim of the study was to assess cardiac involvement in patients with Wegener's granulomatosis (WG), who failed to achieve remission following >6 months induction therapy for life or organ threatening disease. "
2.	Remission Induction 04/01/2004 - "The management of Wegener's granulomatosis can be divided in two stages: induction of remission and maintenance of remission. " 06/26/2009 - "He was diagnosed with limited Wegener's granulomatosis and received remission induction therapy. " 11/01/2009 - "Persistent hematuria after induction of remission in Wegener granulomatosis: a therapeutic dilemma." 11/01/2022 - "The authors provide an overview of the current state of AAV remission-induction after relapse and maintenance therapies, and discuss new strategies recommended to prevent and treat relapses, focusing on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). " 01/01/2007 - "Methotrexate has been shown to be effective for induction of remission in locoregional Wegener's granulomatosis. "
3.	Plasmapheresis 03/01/1990 - "Wegener's granulomatosis: efficacy of plasmapheresis." 01/01/1983 - "[Treatment of Wegener's granulomatosis using plasmapheresis]." 04/01/1982 - "Wegener's granulomatosis treated by plasmapheresis and hemodialysis." 01/01/2010 - "The purpose of this study was to report the use of plasmapheresis in the management of choroidal vasculitis associated with Wegener granulomatosis. " 06/01/2013 - "Successful use of plasmapheresis for granulomatosis with polyangiitis presenting as diffuse alveolar hemorrhage."
4.	Aftercare (After-Treatment) 01/01/2011 - "[A case of Wegener granulomatosis after treatment for non-tuberculous mycobacteriosis]." 09/01/2005 - "Chest radiographs are useful for follow-up observation of patients with Wegener's granulomatosis after treatment." 11/29/1990 - "A 65-year-old man with pulmonary infiltrates after treatment for Wegener's granulomatosis." 11/01/2021 - "The Case | Unexplained inflammation after treatment for granulomatosis with polyangiitis." 04/01/2013 - "Reversal of aortic root inflammation after treatment of Wegener's granulomatosis."
5.	Plasma Exchange 07/01/2020 - "We report a case of Wegener's granulomatosis (WG) who very well responded to the combination strategy of therapeutic plasma exchange (TPE) and immunosuppression. " 07/01/2020 - "Therapeutic plasma exchange: A life-saving modality in Wegener's granulomatosis." 01/01/2020 - "Refractory optic perineuritis related to granulomatosis with polyangiitis treated with intensive immunosuppressive therapy combined with plasma exchange." 11/01/2003 - "Finally, plasma exchange for Wegener granulomatosis with severe renal dysfunction appears not to offer any benefit over immunosuppressive therapy." 11/01/2003 - "Finally, a large case series of the use of plasma exchange in Wegener granulomatosis was published. "