A form of necrotizing vasculitis with involvement of lungs, kidneys and sinuses.
Pulmonary granulomatous polyangiitis.
Also called granulomatosis with polyangitis.
Positive antineutrophilic cytoplasmic (ANCA) test present.
Close correlation between c-ANCA test and disease activity.
80-90% have a positive PR-3 ANCA test.
60-80% of patients have flare of their disease.
4-25% of patients have evidence of cardiac disease during the course of their illness.
6% have pericarditis.
High frequency of echocardiographic abnormalities exist and is associated with higher mortality rate.
Prevalence in Europe 5 per 100,000.
The disease occurs more frequently in northern latitudes.
Most patients have initial symptoms of nasal discharge, sinusitis, or epistaxis.
Patients typically present with nonspecific symptoms, such as fever, lethargy, malaise, and arthralgias.
Patients commonly present with persistent rhinorrhea, oral and nasal ulcers polyarthritis and myalgias.
Chest x-rays often show hilar adenopathy, lung nodules, and opacities.
May cause destruction of nasal septum with a saddle nose deformity.
Lung parenchyma, trachea and bronchi may be affected.
May be associated with a cavitary process in the lung.
As the disease matures to a more vasculitic stage arthralgias, cutaneous vasculitis, mononeuritis and polyneuritis may manifest.
Patients may have active urinary sediment.
More than 70% will have renal involvement.
Commonly affects CNS.
Orbits affected in approximately 50% of cases and can be associated with pain, tenderness of the orbits along with proptosis.
Cranial nerve palsies affecting primarily the second, sixth, and seventh nerves.
Can be associated with pachymeningitis.
May be associated with thrombocytosis.
ANCA positive in 80% of patients with renal involvement.
Renal biopsy demonstrates focal, segmental narcotizing glomerulonephritis with occasional crescent formation.
Unlike respiratory tract biopsy material renal biopsy rarely shows granuloma.
Complement levels are normal.
No immunoglobulin or complement on renal immunofluoresence and anti glomerular basement membrane antibodies are negative.
Most patients have a remission with cyclophosphamide and glucocorticoids.
Between 70 and 90% of patients attain clinical remission with initial immunosuppressive treatment.
Long-term management with corticosteroid sparing agents should be considered to for the induction of remission but potential need for repeated courses of therapy, as relapses are common.