Granulomatosis with polyangiitis

Polyangiitis granulomatosis (also known as Wegener's granulomatosis) is a necrotizing small vessel vasculitis that affects:


 * respiratory system by formation of granulomas
 * kidney necrotizing glomerulonephritis

Clinical manifestation
inflammation (up to necrotizing) of the upper respiratory tract with ulceration, epistaxis, sinusitis, destruction of nasal cartilage with the formation of the so-called "saddle nose", subglottic tracheal stenosis with a stridor with the risk of acute asphyxia, chronic infections caused by Staphylococus aureus ; otitis media, hearing impairment ;
 * General symptoms: fever, weight loss, fatigue;
 * ORL


 * Lower respiratory tract: cough, chest pain, hemoptysis of typical necrotizing granulomas in the bronchi;


 * Kidneys: rapidly progressing glomerulonephritis (ANCA-positive glomerulonephritis) with an acutely developing pattern of renal insufficiency ;


 * Gastrointestinal tract: diarrhea, enterorrhagia, abdominal pain, endoscopically detectable haemorrhage to ulceration;


 * Peripheral nerves: mononeuritis multiplex


 * Eyes: corneal inflammation with ulceration, danger of blindness;
 * Musculoskeletal system:arthralgia, myalgia, erosive artritis.

Diagnostics


Lung X-ray
 * Lab diagnostics
 * ↑ Acute phase reactants, ↑ FW, hypergamaglobulinemia;
 * anemia, leukocytosis, thrombocytopenia;
 * proteinuria, erythrocyturia
 * c-ANCA antibodies (positivity subsides with loss of disease activity)
 * rheumatoid factors are detected in some patients ;
 * granulomatous inflammation in a biopsy specimen.

Lung X-ray
 * Butterfly obscuration or nodular shading.

Renal biopsy
 * Focal to focal-segmental glomerulonephritis;
 * sometimes histologically demonstrable vasculitis of small vessels.

Therapy

 * Active forms: combined pulse treatment with methylprednisolone + cyclophosphamide as in Polyarteriitis nodosa;
 * in case of ANCA antibody positivity and renal impairment or haemoptysis: immediate plasmapheresis.

Prognosis

 * Depends on the degree of kidney damage, 90% of patients survive for five years;
 * Untreated disease has a poor prognosis: up to 70% of patients die;
 * Relapses are common, they occur in up to 50% of patients several years after diagnosis, often in connection with infection or dose reduction of corticosteroids, large relapses are treated by pulse application of methylprednisone and cyclophosphamide, small relapses by increasing the dose of maintenance treatment.

Related Articles

 * Autoimmune Diseases
 * Granulomatous lung processes

Reference

 * 1) GEETHA, D and A EIRIN, et al. Kidney transplantation in patients with ANCA vasculitis. Postgraduate nephrology [online] . 2011, vol. 9, no. 3, pp. 46-47, also available from < http://www.transplant.cz/vzdelavani/2011/2011_03_08.pdf >. St. Translation with commentary. Comment author: Viklický O. ISSN 1214-178X. PMID: 21508899 .DOI: 10.1097 / TP.0b013e31821ab9aa.