Wegner’s granulomatosis

What is granulomatosis with polyangiitis (GPA)?

Granulomatosis with polyangiitis (GPA) is an uncommon type of inflammation of small arteries and veins (vasculitis). It classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages(sinuses), and the kidneys. "Incomplete" forms exist that only involve one of these areas. When both lungs and kidneys are affected, the condition is sometimes referred to as generalized granulomatosis with polyangiitis. When only the lungs are involved, the condition is sometimes referred to as limited granulomatosis with polyangiitis (GPA). Doctors typically avoid the older name "Wegener's granulomatosis" because the German physician Friedrich Wegener, the disease's former namesake, was a member of the Nazi party and allegedly linked to Nazi war crimes. The terminology "granulomatosis with polyangiitis" highlights the two central pathologic features of the disease, granuloma formation and inflammation of blood vessels (angiitis or vasculitis).

GPA usually affects young or middle-aged adults. Although it is uncommon in children, it can affect people at any age. The cause of GPA is not known.

What are symptoms of granulomatosis with polyangiitis (GPA)?

• bloody sputum,
• fevers,
• fatigue,
• joint pains,
• shortness of breath,
• sinus inflammation (sinusitis), and
• weight loss.

Nasal ulcerations and even bloody nasal discharge can occur. Other areas of the body that can also become inflamed in patients with GPA include the eyes, the nerves (neuropathy), the middle ear (otitis media), and the skin resulting in skin nodules or ulcers.

How is granulomatosis with polyangiitis (GPA) diagnosed?

Abnormal lab findings in patients with granulomatosis with polyangiitis (GPA) include urine tests that detect protein and red blood cells in the urine (not visible to the naked eye) and x-ray tests of the chest and sinuses which detect abnormalities resulting from lung and sinus inflammation. Blood tests that detect the abnormal inflammation include the sedimentation rate(sed rate) and C-reactive protein. A more specific blood test used to diagnose and monitor Wegener's granulomatosis is the antineutrophil cytoplasmic antibody (ANCA test), which is commonly elevated when the disease is active.

The diagnosis of GPA is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis in a biopsy of tissue involved with the inflammatory process. For examples, an open lung biopsy or a kidney biopsy are commonly used in making a diagnosis of granulomatosis with polyangiitis.