Granulomatosis with polyangiitis

Granulomatosis with polyangiitis' (formerly Wegener's granulomatosis' ) is a necrotizing vasculitis of small vessels that affects:
 * respiratory system' by the formation of granulomas,
 * kidneys necrotizing glomerulonephritis.

Clinical picture

 * General symptoms: febrile, weight loss, fatigue;
 * ORL area: inflammation (up to necrotizing) of the upper respiratory tract with the formation of ulcers, epistaxis, sinusitis, destruction of the nasal cartilages with the formation of the so-called saddle nose, subglottic stenosis of the trachea manifested by stridor with danger of acute asphyxia, chronic carrier of Staphylococus aureus;
 * otitis media, conductive hearing disorders;

Gastrointestinal tract: diarrhoea, enterorrhagia, abdominal pain, endoscopically demonstrable haemorrhages and ulcerations; Locomotor system: arthralgia, myalgia, erosive arthritis.
 * Lower respiratory tract: cough, chest pain, hemoptysis from typical necrotizing granulomas in the bronchi;
 * Kidney: rapidly progressing glomerulonephritis (ANCA-positive glomerulonephritis) with an acutely developing picture renal insufficiency;
 * Peripheral nerves: mononeuritis multiplex;
 * Eyes: inflammation of the cornea with the formation of ulcers, danger of blindness;

Diagnostics


Laboratory findings: X-ray of lungs:
 * ↑ PAF, ↑ FW, hypergammaglobulinemia;
 * anemia, leukocytosis, thrombocytopenia;
 * proteinuria, erythrocyturia
 * c-ANCA antibodies (positivity recedes as disease activity subsides);
 * "rheumatoid factors" are demonstrated in some patients;
 * granulomatous inflammation' in a biopsy specimen.
 * Butterfly shading or nodal shading.
 * Renal biopsy:
 * Focal to focal-segmental glomerulonephritis;
 * histologically, sometimes demonstrable vasculitis of small vessels.

Therapy

 * Active forms: combined pulse treatment with methylprednisolone + cyclophosphamide as in PAN;
 * in case of positivity of ANCA antibodies and kidney involvement or hemoptysis: immediate plasmapheresis.

Prognosis

 * Depends on the degree of kidney involvement, 90% of patients survive an average of five years;
 * untreated disease has a poor prognosis: up to 70% of patients die;
 * has a tendency to "relapses", they are frequent, they appear in up to 50% of patients even several years after diagnosis, often in connection with infection or reduction of corticosteroid doses, "large relapses" are treated with pulsed application of methylprednisone and cyclophosphamide, 'minor relapses'' by increasing the maintenance dose.

Related Articles

 * Autoimmune disease
 * Glomerulonephritis
 * Rapidly progressive glomerulonephritis
 * Systemic vasculitides