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Wegener with polyangiitis (Wegener's Granulomatosis)

image14Wegener with polyangiitis (GPA or with an old terminology Wegener's Granulomatosis) is a rare Vasculitis affecting small and medium-sized vessels that may involve many organs with predilection for the upper and lower Airways and for kidneys.
Has a prevalence of 2.4-15.7 people each 100000, has a slight predilection for males (♂ : ♀ = 1,5 : 1), the first diagnosis is generally has between 40-55 years. Current treatments allow excellent survival with a good symptom control.
The first symptoms are very often at the level of the upper airways and is therefore often the Otolaryngologist physician who sees patients first and must suspect this disease and confirm the diagnosis and then follow with the patient to an immunologist.

The manifestations may be the most diverse and sometimes nuanced.
Chronic rhinitis cause nasal crusted level that may cause septal perforations even with possible nose deformity (saddle nose = saddle nose). Nasal respiratory problems can be very important and the accumulation of mucus and the presence of crusts may promote infections (sinusitis). You can also view epistaxis and epiphora (leakage of tears) for obstruction/inflammation of the lacrimal.
Ear-level you may have chronic otitis poorly responsive to common therapies.
Other rare and unusual manifestation of granulomatous polyangiitis is the subglottic stenosis which may cause shortness of breath.
Other signs that may identify ’ Otolaryngologist are l ’ gingival hypertrophy, cough and proptosis.
Often patients are treated with limited success for individual manifestations of the disease without curing it perceives a Vasculitis.
While traditional therapies have limited results, immunosuppressive therapies have an important role.
To clarify the diagnosis is always required a full assessment fiberoptic ENT, an evaluation of renal function, a study and evaluation of chest radilogico ’ CBC, VES, PCR and antibody testing, cANCA and bench. It is also often required a mucosal biopsy of an area affected by disease.