II. Epidemiology

  1. Rare Incidence
  2. Peak Incidence during fourth decade of life

III. Pathophysiology

  1. Renamed in 2012 as Granulomatosis with Polyangiitis
    1. Previously known as Wegener's Granulomatosis
  2. Classic Triad of Involvement
    1. Interstitial Lung Disease
    2. Glomerulonephritis
    3. Upper respiratory tract (Sinus and nasal disease)
  3. Granulomatous ANCA-Associated Small Vessel Vasculitis
    1. Necrotizing Granulomas

IV. Symptoms

V. Signs

  1. Head and neck changes
    1. Nasal mucosa ulceration
    2. Septal perforation
    3. Cartilaginous destruction (Saddle nose deformity)
    4. Gingival ulceration
    5. Recurrent Sinusitis
    6. Otitis Media
    7. Hearing Loss
  2. Lung Changes
    1. See Interstitial Lung Disease
    2. Pneumonia
  3. Renal
    1. See Glomerulonephritis
  4. Eye Involvement may also occur
    1. Conjunctivitis
    2. Uveitis
    3. Retinitis
    4. Chemosis
    5. Exophthalmos
  5. Rheumatologic
    1. Polyarthritis
  6. Neurologic
    1. Neuropathy

VI. Differential Diagnosis

VII. Labs

  1. Antineutrophil Cytoplasmic Antibodies (ANCA)
    1. cANCA positive in 75-90% of patients
    2. pANCA positive in 20% of cases
    3. ANCA Test Specificity: 98%
      1. Despite Specificity, high False Positive Rate due to rare Incidence of condition
    4. Indications for ANCA testing (do not obtain solely due to recurrent Sinusitis)
      1. Pulmonary-Renal Syndrome
      2. Rapidly progressive Renal Failure
      3. Mononeuritis multiplex
      4. Pulmonary Hemorrhage
  2. Complete Blood Count
    1. Anemia
    2. Leukocytosis
    3. Eosinophilia
  3. Urinalysis
    1. Consistent with Glomerulonephritis
  4. Hyperglobulinemia

VIII. Imaging: Chest XRay

  1. See Interstitial Lung Disease
  2. Bronchopneumonic patches
  3. Multiple nodular densities (may cavitate)

IX. Diagnostics

  1. Open lung biopsy (most definitive)
  2. Renal and sinus biopsy are often non-diagnostic

X. Management

  1. Induction Therapy
    1. Cyclophosphamide (Cytoxan)
    2. Corticosteroids
      1. Consider high-dose IV Methylprednisolone for 3 days
  2. Maintenance Therapy
    1. Taper Prednisone
    2. Maintain cyclophosphamide for 12 to 18 months

XI. Coarse

  1. Mortality often associated with Renal Failure

XII. References

  1. Allen in Goldman (2000) Cecil Medicine, p. 1529-32
  2. Calabrese in Ruddy (2001) Kelley's Rheum, p. 1167-76
  3. Ali (2018) Am Fam Physician 98(3): 164-70 [PubMed]

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Related Studies

Ontology: Wegener Granulomatosis (C0043092)

Definition (MEDLINEPLUS)

Granulomatosis with polyangiitis (Wegener's), also known as GPA, is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. The inflammation limits the flow of blood to important organs, causing damage. It can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs, and kidneys.

The cause of GPA is unknown. It can affect people at any age. Men and women are equally affected. It is more common in whites. Symptoms may include joint pain, weakness, tiredness, and cold symptoms such as a runny nose that doesn't get better. Doctors use blood tests, chest X-rays, and biopsies to diagnose GPA and rule out other causes of the symptoms.

Early treatment is important. Most people improve with medicines to slow or stop the inflammation.

NIH: National Institute of Allergy and Infectious Diseases

Definition (NCI) A rare, autoimmune, systemic medium and small size vasculitis. It is characterized by the formation of necrotizing granulomas in the respiratory tract, necrotizing angiitis, and glomerulonephritis.
Definition (CSP) multisystem disease chiefly affecting males, characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts, glomerulonephritis, and variable degrees of systemic small vessel vasculitis; considered an aberrant hypersensitivity reaction to an unknown antigen.
Concepts Disease or Syndrome (T047)
MSH D014890
ICD9 446.4
ICD10 M31.3 , M31.30
SnomedCT 195353004, 155445002, 266323005, 23782005, 239934006, 390001000
LNC LA17782-6
English Granulomatosis, Wegener, Granulomatosis, Wegener's, WEGENER'S GRANULOMATOSIS, Nec respiratory granulomatos, Wegener's Granulomatosis, WEGENERS GRANULOMATOSIS, WEGENER GRANULOMATOSIS, WG, Wegener Granulomatosis, Wegener's granulomatosis (diagnosis), Wegener granulomatosis, Wegener's granulomatosis NOS, Wegener Granulomatosis [Disease/Finding], wegener's syndrome, wegener granulomatosis, wegener's granulomatosis, granulomatosis wegener, granulomatosis wegener's, granulomatosis wegeners, wegeners granulomatosis, Wegeners granulomatosis, Granulomatosis with polyangiitis, GRANULOMATOSIS WITH POLYANGITIS, Granulomatosis - Wegener's, Wegener's syndrome (disorder), Wegener syndrome, Granulomatosis with Polyangiitis (Wegener's), GPA, Wegener's granulomatosis, Necrotizing respiratory granulomatosis, Necrotising respiratory granulomatosis, Wegener's granulomatosis (disorder), Wegener; granulomatosis, Wegener, granulomatosis; Wegener, granulomatosis; necrotizing, necrotizing; granulomatosis, Wegener's granulomatosis (disorder) [Ambiguous], Wegener's syndrome
Portuguese GRANULOMATOSE DE WEGENER, Granulomatose de Wegener
Spanish GRANULOMATOSIS DE WEGENER, granulomatosis respiratoria necrosante, granulomatosis con poliangeítis, síndrome de Wegener (trastorno), granulomatosis respiratoria necrotizante, síndrome de Wegener, granulomatosis de Wegener (concepto no activo), granulomatosis de Wegener (trastorno), granulomatosis de Wegener, Granulomatosis de Wegener
Swedish Wegeners granulomatos
Czech Wegenerova granulomatóza, Granulomatóza Wegenerova
Finnish Wegenerin granulomatoosi
French GRANULOMATOSE DE WEGENER, Granulomatose avec polyangéite, GPA (Granulomatose avec PolyAngéite), Granulome rhinogène, Granulomatose de Wegener, Maladie de Wegener, Syndrome de Wegener
German WEGENERSCHE GRANULOMATOSE, Wegener Granulomatose, Granulomatose, Wegener-, Wegener-Granulomatose, Wegener-Klinger-Granulomatose
Korean 베게너 육아종증
Polish Ziarniniak Wegenera, Zespół Wegenera
Japanese Wegener肉芽腫症, Wegener肉芽腫, ウェゲナーニクゲシュショウ, ウェゲナー肉芽腫症, ウェーゲナー肉芽腫症, ヴェゲナー肉芽腫症, ヴェーゲナー肉芽腫症, 肉芽腫症-Wegener, 肉芽腫症-ウェゲナー
Norwegian Wegeners granulomatose, Granulomatose, Wegeners, Granulomatose, Wegener
Dutch Wegener-granulomatose, Wegener-granulomatosis, Wegener; granulomatose, granulomatose; Wegener, granulomatose; necrotiserend, necrotiserend; granulomatose, Granulomatose van Wegener
Hungarian Wegener-granulomatosis, Wegener granulomatosis
Italian Granulomatosi di Wegener

Ontology: Granulomatosis with polyangiitis (C3495801)

Definition (MSH) 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.
Concepts Disease or Syndrome (T047)
MSH D014890
English Granulomatous polyangiitis, Granulomatosis with polyangiitis, Polyangiitides, Granulomatosis with, Granulomatosis with Polyangiitides, Polyangiitis, Granulomatosis with, Granulomatosis with Polyangiitis, with Polyangiitis, Granulomatosis, with Polyangiitides, Granulomatosis
Czech Granulomatózní polyangiitida, Granulomatóza s polyangiitidou
Dutch granulomatose met polyangiitis, granulomateuze polyangiitis
French Polyangéite granulomateuse, Granulomatose avec polyangéite
German Granulomatose mit Polyangiitis, granulomatoese Polyangiitis
Hungarian Granulomatosus polyangiitis, Polyangiitises granulomatosis
Italian Poliangite granulomatosa, Granulomatosi con poliangioite
Japanese 多発血管炎を伴う肉芽腫症, 肉芽腫性多発血管炎, タハツケッカンエンヲトモナウニクゲシュショウ, ニクゲシュセイタハツケッカンエン
Portuguese Poliangite granulomatosa, Granulomatose com poliangite
Spanish Granulomatosis con poliangitis