eMedicine Specialties > Rheumatology > Vasculitis

Wegener Granulomatosis

Author: Patricia J Papadopoulos, MD, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Coauthor(s): Robert John O'Brian, MD, Staff Rheumatologist, National Naval Medical Center
Contributor Information and Disclosures

Updated: Aug 4, 2009

Introduction

Background

Wegener granulomatosis (WG) is a rare multisystem autoimmune disease of unknown etiology. Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels.

In 1897, Peter McBride likely gave the first written description of a patient with the condition. Thirty-four years later, Klinger described a 70-year-old physician with constitutional symptoms, joint symptoms, proptosis, widespread upper respiratory tract inflammation leading to saddle nose deformity, glomerulonephritis, and pulmonary lesions. In 1936, Wegener reported three patients with similar clinical features and published his findings on their distinct clinical and histopathologic findings, leading to the eponymous designation of the disease. In 1954, Goodman and Churg provided the definitive description of WG upon their identification of a triad of pathological features that characterize the disease, including (1) systemic necrotizing angiitis, (2) necrotizing granulomatous inflammation of the respiratory tract, and (3) necrotizing glomerulonephritis.

WG is one of the antineutrophil cytoplasmic antibody (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 WG 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. Those with severe disease present with significant multisystem manifestations, which may involve the lungs, kidneys, and other organs, in addition to the respiratory tract.

Consensus does not exist as to whether limited WG 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 WG 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.1 Thus, although a patient may initially be diagnosed with limited WG, he or she may subsequently develop generalized disease with renal involvement.

Analysis of another WG 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 WG had more severe upper respiratory tract damage, were more likely to have flares after remission, and tended to have identical manifestations when they relapsed.2

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

Limited Wegener granulomatosis

This designation is reserved for cases that fulfill the modified American College of Rheumatology (ACR) criteria for the classification of WG (see below) in the absence of disease features that pose immediate threats to either a critical individual organ or to the patient's life.

Specifically, this means the following:

  1. The patient has no red blood cell (RBC) casts in the urine.
  2. If hematuria is present (but no RBC casts), the serum creatinine level is 1.4 mg/dL or less, and there must be no evidence of a rise in the creatinine level of more than 25% above the patient’s baseline level.
  3. Pulmonary involvement must be circumscribed, such that the room air PO2 level is greater than 70 mm Hg or the room air O2 saturation by pulse oximetry is greater than 92%. Pulmonary hemorrhage may be treated as limited disease provided there is no evidence of progression of process. In the absence of data on progression, pulmonary hemorrhage may be treated as severe disease at the discretion of the physician.
  4. No disease may exist within any other critical organ (eg, the GI tract, eyes, CNS) that, without the immediate institution of maximal therapy (ie, pulse methylprednisolone or daily oral cyclophosphamide), threatens the function of that organ and/or the patient’s life.2

Severe Wegener granulomatosis

Any patient with WG whose disease is not classifiable as limited has severe disease, by definition.2

Pathophysiology

The pathogenesis of WG has not been fully elucidated, but both cellular and humoral immunity are thought to be involved. WG has three characteristic pathologic manifestations: vasculitis of the small- to medium-sized vessels, "geographic" necrosis, and granulomatous inflammation, particularly in the airways. The initial pathologic lesion is that of the granuloma believed to be caused by cellular immune processes. Environmental exposures, including respiratory tract infections, have been implicated as inciting factors. A better understanding of the progression from granuloma to vasculitis may shed light on the possible etiology and pathogenesis of WG. It is probable that there is complex interaction between the environment and host factors, many of which are genetically determined. Cellular immune processes are also involved in tissue injury owing to the inflammatory cascade.

The discovery of antibodies to cytoplasmic antigens within neutrophils (ANCAs) in most patients with WG suggests the role of humoral autoimmunity. WG is usually associated with the presence of diffuse staining cytoplasmic ANCA (C-ANCA) directed against serine proteinase 3 antigen (PR3-ANCA), the so-called Wegener autoantigen. The other AAVs include microscopic polyangiitis, renal–limited vasculitis, and Churg-Strauss syndrome, which are most commonly associated with perinuclear staining ANCA (P-ANCA) directed against myeloperoxidase (MPO-ANCA).

A pathogenic role for PR3-ANCAs in WG has been proposed. In vitro effects of PR3-ANCA described to date include activation of primed neutrophils, leading to production of reactive oxygen species, and release of lytic enzymes such as elastase and PR3, which act to promote tissue injury.3 Indirect in vivo evidence suggests a pathogenic role for ANCA in that rising titers can sometimes herald a disease relapse. In addition, over 90% of patients with severe WG have been reported to have ANCA positivity during active disease.4 However, despite the in vitro evidence for a pathogenic role, currently, no definitive in vivo evidence has been found. Further research is necessary to further elaborate the role of PR3-ANCA in WG.

Typically, most autoimmune diseases are attributed to a genetic predisposition combined with exposure to an inciting factor. Genotypic associations in WG include (1) carrying a defective allele for alpha-1 antitrypsin, (2) possessing certain polymorphisms of the CTLA-4 (cytotoxic T-lymphocyte antigen 4) or PTPN22 genes, and (3) exhibiting certain forms of the Fcγ receptor IIIb on the surface of neutrophils and monocytes/macrophages.3,5,6,7

Living in northern latitudes, farming, drug and environmental allergies, and exposures to solvents or silica have all been linked to the development of WG.7 Reports vary as to whether disease onset is associated with a seasonal peak.

Nasal carriage of Staphylococcus aureus is associated with relapses of WG (relative risk, 9.0). Prophylactic treatment with trimethoprim-sulfamethoxazole (TMP-SMX) can reduce the likelihood of relapse by 60%,3 supporting S aureus carriage as a possible inciting factor for WG.8

Frequency

United States

WG is a rare disease with an as yet undetermined incidence. The prevalence of WG in the United States is estimated to be 3 cases per 100,000 people.

International

The incidence and prevalence of WG in the United Kingdom is estimated at 10.2 cases and 250 cases per million population, respectively.

Mortality/Morbidity

  • Untreated severe WG is associated with a very high (>90%) mortality rate. With aggressive therapy, the mortality rates improve.
    • Historically, patients with WG had a mean survival of 5 months from diagnosis; the mortality rate was 82% at 1 year.
    • The introduction of corticosteroids prolonged the median survival by only 7.5 months.
    • Before the introduction of cyclophosphamide-based regimens, severe WG was nearly always fatal within 1 year of diagnosis.
    • According to a meta-analysis, with current treatments, the 5-year survival rate ranges from 74%-79%.9
    • The most common causes of death in WG include infection, respiratory and renal failure, malignancy, and cardiovascular disease.
  • Most morbidity is currently treatment related.

Race

WG is more common in individuals of northern European descent (approximately 90%); it occurs less commonly in blacks.

Sex

In European populations, WG is slightly more common in men, with a male-to-female ratio of 1.5:1.10 Women are more likely to have limited disease.2

Age

Onset of WG may occur at any age; patients typically present at age 35-55 years. WG is rare in childhood (see the article Wegener Granulomatosis in eMedicine's Pediatrics: General Medicine volume for more information).

Clinical

History

The clinician must remain mindful that Wegener granulomatosis (WG) has a spectrum of clinical presentations.

  • Patients may report the following chronic, nonspecific constitutional complaints:
    • Fevers, night sweats
    • Fatigue, lethargy
    • Loss of appetite
    • Weight loss
  • Ocular involvement occurs in many patients, including conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, nasolacrimal duct occlusion, and proptosis.
  • ENT symptoms include the following:
    • Chronic sinusitis is the most common initial complaint (67%); failure to respond to conventional treatment is suggestive. Rhinitis and epistaxis occur in 22% and 11% of patients with WG, respectively.11
    • Development of a saddle nose deformity caused by collapse of nasal support is common (see Image 1).

      Saddle nose deformity in a 26-year-old man with W...

      Saddle nose deformity in a 26-year-old man with Wegener granulomatosis. Image courtesy of P. Papadopoulos, MD.

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      Saddle nose deformity in a 26-year-old man with W...

      Saddle nose deformity in a 26-year-old man with Wegener granulomatosis. Image courtesy of P. Papadopoulos, MD.

    • Serous otitis media and hearing loss are the presenting manifestations of WG in some patients.
    • See the article Wegener Granulomatosis in eMedicine's Otolaryngology and Facial Plastic Surgery volume for more information.
  • Pulmonary disease may cause pulmonary infiltrates (71%), cough (34%), hemoptysis (18%), chest discomfort (8%), and dyspnea (7%).11 Diffuse alveolar hemorrhage (DAH) due to alveolar capillaritis is reported in 5%-45% of cases.12
  • Musculoskeletal symptoms are common. Arthralgias are usually polyarticular and symmetric, affecting both small and large joints. Arthritis can also occur and typically affects large joints but is rarely deforming.
  • Renal disease is present in 17% at initial diagnosis and is usually asymptomatic.1 Renal failure occurs in 11% at presentation.11 Renal disease manifests as crescentic necrotizing glomerulonephritis characterized by urinary sediment with more than 5 RBCs per high-power field (HPF) or erythrocyte casts.
  • The nervous system is affected by vasculitis in 22% of patients.11 Clinical manifestations include mononeuritis multiplex and cranial nerve abnormalities. (See the article Wegener Granulomatosis in eMedicine's Neurology volume for more information.)
  • The skin is involved in 45% of patients with WG due to vasculitis and can lead to palpable purpura or skin ulcers.11 (See the article Wegener Granulomatosis in eMedicine's Dermatology volume for more information.)

Physical

  • General: Patients may be febrile and appear ill.
  • Head, ears, eyes, nose, and throat
    • Sinusitis and disease in the nasal mucosa are the most common findings.
    • Ocular findings include scleritis, keratitis, uveitis, episcleritis, and conjunctivitis.
    • Proptosis may signal retrobulbar granuloma.
    • Xanthelasma has been reported.
    • Otitis media and/or hearing loss may be present.
    • Purulent or sanguinous nasal discharge may be seen.
    • Saddle nose deformity may be present (see Image 1).

      Saddle nose deformity in a 26-year-old man with W...

      Saddle nose deformity in a 26-year-old man with Wegener granulomatosis. Image courtesy of P. Papadopoulos, MD.

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      Saddle nose deformity in a 26-year-old man with W...

      Saddle nose deformity in a 26-year-old man with Wegener granulomatosis. Image courtesy of P. Papadopoulos, MD.

    • Oral involvement is rare; however, a classic presentation includes "strawberry gingival hyperplasia."
  • Pulmonary/respiratory
    • Atelectasis may be present, with examination findings of dullness on percussion, decreased breath sounds, and crackles on auscultation.
    • Lower respiratory tract involvement may also produce signs of pulmonary consolidation and/or pleural effusion.
    • Hoarseness, cough, dyspnea, stridor, and wheezing may be associated with tracheobronchial disease.
    • Stridor may also occur with subglottic or tracheal stenosis, which may prove fatal if untreated.
    • Hemoptysis may result from cavitated pulmonary parenchymal lesions, DAH, or bronchiectasis.
  • Cardiac: Pericardial rub may occur with pericarditis.
  • Gastrointestinal: Abdominal pain may be present with splanchnic vasculitis.13
  • Musculoskeletal: Large joint arthritis, polyarticular symmetric small and medium joint arthralgias, and myalgias may be appreciated.
  • Neurologic: Examination of the nervous system may confirm a pattern of mononeuritis multiplex or cranial nerve paralysis.
  • Integument
    • Cutaneous findings are variable and nonspecific and usually affect the lower extremities.
    • Palpable purpura, papules, subcutaneous nodules, and ulcerations are the most common findings.
    • Ulcerations may resemble pyoderma gangrenosum.
    • Petechiae, vesicles, pustules, hemorrhagic bullae, livedo reticularis, digital necrosis, subungual splinter hemorrhages, and genital ulcers resembling squamous cell carcinoma have been reported.

More on Wegener Granulomatosis

Overview: Wegener Granulomatosis
Differential Diagnoses & Workup: Wegener Granulomatosis
Treatment & Medication: Wegener Granulomatosis
Follow-up: Wegener Granulomatosis
Multimedia: Wegener Granulomatosis
References
Further Reading
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Keywords

Wegener granulomatosis, WG, Wegener's granulomatosis, Wegener's disease, Wegener disease, systemic vasculitis, systemic necrotizing angiitis, necrotizing glomerulonephritis, necrotizing granulomatous inflammation of the respiratory tract, rapidly progressive glomerulonephritis, ANCA-associated vasculitis, ANCA, AAV, PR3-ANCA, alveolar capillaritis, diffuse alveolar hemorrhage, pauci-immune complex, glomerulonephritis

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Contributor Information and Disclosures

Author

Patricia J Papadopoulos, MD, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Patricia J Papadopoulos, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert John O'Brian, MD, Staff Rheumatologist, National Naval Medical Center
Robert John O'Brian, MD is a member of the following medical societies: American College of Rheumatology
Disclosure: Nothing to disclose.

Medical Editor

Robert E Wolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport
Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, and Society for Leukocyte Biology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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