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Wegener Granulomatosis (Wegener's Granulomatosis)

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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 neutrophil proteinase-3 (WEGENER AUTOANTIGEN).

Also Known As:

Wegener's Granulomatosis; Granulomatosis, Wegener; Granulomatosis, Wegener's

Networked: 1990 relevant articles (134 outcomes, 128 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Vasculitis (Vasculitides)
2.	Microscopic Polyangiitis
3.	Sarcoidosis (Schaumann Disease)
4.	Glomerulonephritis
5.	Systemic Vasculitis

Experts

1.	Gross, W L: 38 articles (11/2012 - 03/2000)
2.	Specks, Ulrich: 36 articles (08/2015 - 05/2002)
3.	Gross, Wolfgang L: 30 articles (01/2014 - 05/2002)
4.	Hoffman, Gary S: 29 articles (08/2015 - 05/2003)
5.	Stone, John H: 23 articles (05/2015 - 08/2003)
6.	Csernok, Elena: 21 articles (08/2014 - 05/2002)
7.	Kallenberg, Cees G M: 20 articles (04/2015 - 02/2002)
8.	Lamprecht, P: 20 articles (10/2010 - 05/2000)
9.	Merkel, Peter A: 19 articles (08/2015 - 04/2005)
10.	Guillevin, Loïc: 19 articles (01/2011 - 04/2003)

Drugs and Biologics

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

1.	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. " 07/03/1989 - "Here we report the first results of a prospective study using cyclophosphamide pulse therapy (monthly application) (a) as an alternative treatment in seven cases of active generalized Wegener's granulomatosis, which either showed complications under continuous cyclophosphamide treatment or in which the partial remission was not steady, and (b) as the initial treatment in five newly diagnosed patients with active generalized disease. " 05/21/1988 - "Early diagnosis and treatment of Wegener's granulomatosis with cyclophosphamide has considerably improved the prognosis in this previously fatal disease. "
2.	Antineutrophil Cytoplasmic Antibodies (ANCA)IBA 05/01/1993 - "The identification of a new serologic marker, cytoplasmic pattern antineutrophil cytoplasmic autoantibody (C-ANCA), allows for the early diagnosis of Wegener's granulomatosis and gives the patient the best chance for remission with cytotoxic therapy. " 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. "
3.	rituximab (Mabthera)FDA Link 10/01/2007 - "Rituximab is a well-tolerated, very effective medication for patients with Wegener's granulomatosis. " 07/01/2009 - "Recurrence of Wegener's granulomatosis with de novo intraoral presentation treated successfully with rituximab." 10/01/2008 - "This demonstrates how rituximab may be beneficial for childhood-onset Wegener granulomatosis unresponsive to conventional therapy. " 03/01/2006 - "Sustained 3-year remission after rituximab treatment in a patient with refractory Wegener's granulomatosis." 05/01/2009 - "Rituximab is effective in the treatment of refractory ophthalmic Wegener's granulomatosis."
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.	Prednisolone (Predate)FDA LinkGeneric 02/01/2010 - "He had been treated with long-term predonine therapy after the initial onset of Wegener's granulomatosis. " 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. " 01/01/1991 - "Immunosuppressive treatment with cyclophosphamide and prednisolone has markedly improved the prognosis of Wegener's granulomatosis (WG). " 07/01/2009 - "Deoxyspergualin achieved a high rate of disease remission and permitted prednisolone reduction in refractory or relapsing Wegener's granulomatosis. " 09/01/2008 - "After diagnosis of Wegener's granulomatosis the patient received prednisolone and cyclophosphamide pulse therapy and remission developed rapidly. "
6.	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. " 04/01/2011 - "A case of Wegener's granulomatosis on corticosteroids and immunosuppressive therapy returned with SP on the same side four months later. " 07/01/2003 - "A case report of wegener granulomatosis treated only with corticosteroids for 30 years." 07/01/2001 - "For almost two years the patient was treated with systemic corticoids for suspected limited Wegener's granulomatosis. " 09/01/1994 - "The prognosis for Wegener's granulomatosis (W-G) is good if it is diagnosed early and a combination of corticosteroids and cytotoxic drugs is given. "
7.	Prednisone (Sone)FDA LinkGeneric 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). " 01/01/1992 - "In addition, the analysis reveals, that immunosuppressive therapy with cyclophosphamide and prednisone facilitates complete remission and a long survival rate of patients with Wegener's granulomatosis."
8.	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. " 08/01/1993 - "In recent years, the prognosis of Wegener's granulomatosis (WG) has been dramatically improved by treatment with cyclophosphamide and glucocorticoids, if the diagnosis is made correctly in the early stage of the disease. " 03/15/1992 - "The course of Wegener granulomatosis has been dramatically improved by daily treatment with cyclophosphamide and glucocorticoids. "
9.	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. " 06/01/1968 - "Combined corticosteroid and azathioprine therapy in 2 patients with Wegener's granulomatosis."
10.	TNFR-Fc fusion protein (etanercept)FDA Link 01/27/2005 - "We conducted a randomized, placebo-controlled trial at eight centers to evaluate etanercept for the maintenance of remission in 180 patients with Wegener's granulomatosis. " 01/27/2005 - "Etanercept is not effective for the maintenance of remission in patients with Wegener's granulomatosis. " 02/01/2014 - "Data from the Wegener's Granulomatosis Etanercept Trial (WGET) were used. " 12/01/2012 - "Etanercept, a well-known TNF-α blocker evaluated for GPA in the Wegener's Granulomatosis Etanercept Trial (WGET), was associated with an increase in the development of solid malignancies in comparison to placebo during that trial period. " 12/01/2011 - "Patients in the Wegener's Granulomatosis Etanercept Trial received either daily CYC or weekly MTX and were randomized to etanercept or placebo. "

Therapies and Procedures

1.	Remission Induction 04/01/2004 - "The management of Wegener's granulomatosis can be divided in two stages: induction of remission and maintenance of remission. " 01/01/2009 - "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." 01/01/2007 - "Methotrexate has been shown to be effective for induction of remission in locoregional Wegener's granulomatosis. " 05/01/2006 - "During the Wegener's Granulomatosis Etanercept Trial (WGET), a placebo-controlled trial of etanercept given in addition to standard therapy for remission induction and maintenance, more solid malignancies were observed in the etanercept group than in the group treated with standard therapy alone. "
2.	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." 04/01/2013 - "Reversal of aortic root inflammation after treatment of Wegener's granulomatosis." 01/01/2003 - "Wegener's Granulomatosis after Treatment with Montelukast in a Patient not Receiving Systemic Corticosteroid Treatment."
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." 09/01/1995 - "Plasmapheresis can be effective in combination with the standard therapy in dialysis patients with necrotizing glomerulonephritis (in Wegener's granulomatosis or microscopic polyarteritis). " 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. "
4.	Plasma Exchange 10/01/2010 - "Utilisation of plasma exchange in the treatment of digital infarcts in Wegener's granulomatosis." 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. " 01/01/2002 - "We report the clinical course of 29 patients with Wegener's granulomatosis (WG) treated with plasma exchange (PE) in Norway in the period from 1988 to 1999. " 01/01/2002 - "Clinical outcome of patients with Wegener's granulomatosis treated with plasma exchange."
5.	Transplants (Transplant) 12/01/2011 - "Detailed analysis of graft techniques for nasal reconstruction following Wegener granulomatosis." 09/01/2008 - "Numerous techniques have been discussed for dorsal augmentation with varied success; however, the long-term maintenance of this graft in patients with severely compromised vascularity owing to underlying inflammatory conditions such as Wegener granulomatosis highlights its presumed advantages. " 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. " 03/06/1995 - "Solid organ transplant recipients and individuals receiving treatment for Wegener's granulomatosis have a significant risk of developing PCP. " 01/01/1994 - "Recurrence of Wegener's granulomatosis in renal grafts is rare. "