Granulomatosis with Polyangiitis (GPA) formerly known as Wegener’s Granulomatosis

Granulomatosis with Polyangiitis (GPA) is a vasculitis (inflammation of blood vessels) characterized by involvement of small to medium sized vessels and necrotizing granulomas (scattered spherical areas of tissue destruction).  The cause is unknown although there are several theories currently under investigation.  It is thought to be a type of auto-immune disease, where the patient’s own immune cells mistakenly begin attacking blood vessels and surrounding tissues.  This disease was formerly known as Wegeners Granulomatosis prior to the American College of Rheumatology nomenclature change in 2011.  The disease is known to affect blood vessels and adjacent tissue primarily in the ears, nose, throat, lungs and kidneys.  One in 33,000 individuals are affected by this disease.  The mean age at onset is 55 years old.  Most of those affected (> 90%) are white.  This disease can be fatal if untreated.  Two main forms of GPA exist:  1) Limited (involving only the ears, nose and throat) and 2) Generalized (involving the ears, nose, throat, lungs and kidneys).

Most patients with GPA present to an Otolaryngologist (ENT) physician (often times before they are diagnosed with GPA) because of ear, nose or throat symptoms.  The most common symptoms GPA patients present with include nasal congestion, diminished sense of smell, runny nose, nose bleeds, excessive tearing from the eye and noticing a flattening of the bridge of the nose. When GPA patients are examined in the ENT office or clinic they may have such exam findings as 1) saddle nose deformity (flattening of the bridge of the nose due to destruction of the cartilage in the nose), 2) septal perforation (whole in the middle wall of the inside of the nose, due to cartilage destruction), 3) bloody crust formation in the nose, 4) granulation tissue (thick, red, bleeding tissue lining part of the inside of the nose) and 5) purulent sinusitis (pus draining from the sinuses and running from the nose or down the throat).  To determine if a patient has GPA the ENT physician will perform a thorough ear, nose and throat exam, nasal endoscopy and order or perform tests looking for specific markers of GPA.  These tests may include a chest X-ray, sinus CT scan, urinalysis, blood draw and possible biopsy of the inflamed tissues inside the nose.  GPA can be difficult to accurately diagnose due to the fact that the blood test and biopsy markers we look for to make the diagnosis may only be present 50% of the time when the disease is present.

Treatment of GPA usually requires both a Rheumatologist (specialist in inflammatory and autoimmune diseases) and ENT physician.  If the ENT physician can make the diagnosis (or is strongly suspicious of the diagnosis), referral to a Rheumatologist is made.  Patients are often treated with drugs that suppress the immune system so that the immune cells in the bloodstream slow down their attack on the patient’s blood vessels and tissues.  These may include cyclophosphamide, methotrexate, prednisone and rituximab.  Long term use of a suppressive dose of the antibiotic, trimethoprim-sulfamethoxazole has also shown benefit for patients with GPA.  ENT physicians often employ saline irrigations, topical nasal steroids, topical nasal antibiotics and periodic cleaning of the infected mucous and crusts from the nose to alleviate nasal and sinus symptoms in GPA.  Placement of ear tubes, tear duct stents or procedures on the airway may also be beneficial in some patients.  GPA is a chronic disease without a known cure.  Symptoms can be treated, and often times controlled, but require a long term relationship with a Rheumatologist and Otolaryngologist.

References

1. Erickson V, Hwang, P.  Current Opinion in Otolaryngology & Head and Neck Surgery 2007, 15:170 – 176
2. Borner et al.  American Journal of Rhinology & Allergy. 26(6):475-80, 2012 Nov-Dec.
3. Wenig B.  Atlas of Head and Neck Pathology; 2nd Ed.

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Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a vasculitis characterized by eosinophilic inflammation of small to medium sized vessels.  It is similar in nature to Granulomatosis with Polyangiitis (GPA) but the main white blood cell involved in the inflammation is the eosinophil.  EGPA is much less common than GPA and EGPA is associated with allergies, asthma and nasal polyps.

1990 American College of Rheumatology criteria for Diagnosis of EGPA
4 of the 6 following criteria should be met before making a diagnosis of EGPA

1. Asthma
   
2. Blood eosinophilia > 10%
   
3. Paranasal sinus abnormality (most commonly seen on sinus CT scan)
   
4. Pulmonary infiltrates (most commonly seen on chest X-ray or chest CT scan)
   
5. Mono-neuritis or polyneuropathy
   
6. Tissue biopsy showing vasculitis with extravascular eosinophils