Wegener’s granulomatosis(redirected from Wegener syndrome)
Wegener’s granulomatosisA rare condition characterised by granulomatous inflammation of the respiratory tract, antineutrophil cytoplasmic antibodies (ANCAs) and necrotising vasculitis of small-to-medium-sized vessels (e.g., capillaries, arteries, arterioles, and venules).
Male:female ratio 2:1; middle-aged population.
Hypersensitivity and/or autoimmunity.
Anorexia, weight loss, fatigue, malaise, prostration, persistent fever, night sweats, cough, haemoptysis, splenomegaly, migratory polyarthritis, granulomas of oral cavity and periorbital regions.
Aseptic meningitis, nonhealing granulomas of CNS, peripheral neuropathy.
Nasolacrimal duct obstruction, episcleritis, conjunctivitis, proptosis.
Chondritis of external ear, serous or purulent otitis media, hearing loss.
Myocardial infarction due to vasculitis.
From asymptomic to focal glomerulitis; may progress to progressive necrotising crescentic glomerulonephritis with hypertension uremia, and end-stage renal failure.
Upper-respiratory tract complaints—ulcers, pneumonia, pulmonary lesions with cavitation, pleuritis, progressive dyspnoea requiring O2.
Granulomas, paranasal sinusitis; nasal mucosa is red, raised, granular, friable and ulcerated, with 2º bacterial infection, and may perforate; haemorrhagic rhinorrhea.
Granulomatous, eventually; a disseminated vascular phase may develop and is associated with lesions, diffuse leukocytoclastic vasculitis and necrotising skin lesions.
Renal biopsy determines extent of renal involvement, which may include necrotising glomerulonephritis; open-lung biopsy of a solid or cavitating lesion.
Granulomatous disease, polyarteritis, renovascular phase of SBE, progressive glomerulonephritis, SLE, lethal midline granuloma (i.e., lymphoma).
Anaemia may be profound, normal/increased complement, increased ESR, leukocytosis, increased ANCAs.
Proteinuria, hematuria, RBC casts.
Corticosteroids (e.g., prednisone); methotrexate, azathioprine, cyclophosphamide induce remission in 75%; long-term prophylactic trimethoprim-sulphamethoxazole may be effective for upper-respiratory tract lesions; kidney transplant if renal failure.
Management side effects
Cyclophosphamide—leukopenia, ergo infections; haemorrhagic cystitis; gonadal dysfunction; hair loss, which is reversible on discontinuing the drug.
Dramatically improved by immunosuppressants; there is an increased risk of solid tumors post-high-dose cyclophosphamide; complete Wegener’s granulomatosis usually progresses rapidly to renal failure once diffuse-vascular phase begins; patients with the limited Wegener’s granulomatosis may have nasal and pulmonary lesions, with little or no systemic involvement.