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Granulomatosis with Polyangiitis (Wegeners) Symptoms

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Granulomatosis with Polyangiitis (Wegeners) Symptoms

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Wegener’s granulomatosis, more recently termed granulomatosis with polyangiitis, is a small and medium vessel necrotizing vasculitis that typically affects the nose, lungs and kidneys. This form of vasculitis is characterized by granulomatous inflammation against a nonspecific inflammatory background, and is thought to be caused by the presence of C-ANCA, or cytoplasmic anti-neutrophil cytoplasmic antibodies. These antibodies react with proteinase 3, an enzyme within neutrophil granulocytes. The lungs and upper airways are classically involved, and patients develop chronic sinusitis, otitis media, perforation of the nasal septum and saddle-nose deformities. Individuals also commonly complain of cough and hemoptysis. The kidneys are classically involved, and this disease is a well-known culprit of rapid progressive glomerulonephritis, which presents with hematuria. Cyclophosphamide and corticosteroids can be used as immunosuppressants in the treatment of granulomatosis with polyangiitis.

9 KEY FACTS

The lungs and respiratory tract are classically involved in granulomatosis with polyangiitis, and patients can present with pulmonary nodules (coin lesions), pulmonary infiltrates and cavitary lesions. This can lead to symptoms, such as cough, while hemoptysis can occur if pulmonary hemorrhage is present.

Cough is common in granulomatosis with polyangiitis, due to involvement of the lungs and lower respiratory tract.

Hemoptysis refers to the coughing up of blood or blood stained sputum from the respiratory tract, and is a common symptom of lung involvement in granulomatosis with polyangiitis.

Chronic sinusitis, which, by definition, is a sinusitis that lasts longer than three months, is common in granulomatosis with polyangiitis. This is due to involvement of blood vessels in the upper respiratory tract.

This term refers to a loss of height of the nose, caused by collapse of the nasal bridge. Saddle-nose deformities can be caused by chronic inflammation of the nasal septum in the nose, along with sinuses in granulomatosis with polyangiitis. Other causes include congenital syphilis, cocaine abuse and leprosy.

Chronic inflammation of the nose can lead to perforation of the nasal septum. This nasal septum damage can lead to the physical deformity, referred to as a saddle-nose deformity.

Otitis media refers to inflammation of the middle ear between the tympanic membrane and the inner ear, and this is often seen in granulomatosis with polyangiitis. Patients can also develop auditory tube dysfunction, which can lead to conductive hearing loss.

The kidneys are classically involved in granulomatosis with polyangiitis, and this disease is associated with rapid progressive glomerulonephritis with hematuria. These patients usually develop chronic kidney failure over time.

Hematuria refers to blood in the urine, and is a common presenting symptom in patients, occurring as a result of renal involvement in this disease.

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