Wegener’s granulomatosis

Learning objectives

Describe Wegener’s granulomatosis
Recognize the symptoms and signs of Wegener’s granulomatosis
Anesthetic management of a patient with Wegener’s granulomatosis

Definition and mechanisms

Wegener’s granulomatosis (WG) is a chronic, immunologic, systemic disease, characterized by a triad of necrotizing granulomas in the upper and lower respiratory tract, small- and medium-sized vessel vasculitis, and glomerulonephritis
Involvement of the upper respiratory tract (i.e., nose and sinuses) is seen in nearly all WG patients
Early diagnosis and treatment might lead to a full recovery
Without treatment, the condition can be fatal

Signs and symptoms

Signs and symptoms develop suddenly or over several months

Pus-like drainage with crusts from your nose, stuffiness, sinus infections, and nosebleeds
Coughing, sometimes with bloody phlegm
Shortness of breath or wheezing
Fever
Fatigue
Joint pain
Numbness in the limbs, fingers, or toes
Weight loss
Blood in urine
Skin sores, bruising, or rashes
Eye redness, burning or pain, and vision problems
Ear inflammation and hearing problems

Complications

Hearing loss
Skin scarring
Chronic kidney disease
A loss of height in the bridge of the nose (saddling) caused by weakened cartilage
Deep vein thrombosis

Treatment

Combination of corticosteroids and cytotoxic agents to induce and maintain remission

Severe disease

Induce remission with immunosuppressants (e.g., rituximab or cyclophosphamide) in combination with high-dose corticosteroids (e.g., prednisone)
Plasmapheresis if kidneys are involved
Steroid dose is tapered slowly after remission → focus shifts to maintain remission state and prevent subsequent WG flares → less toxic immunosuppressants (e.g., rituximab, methotrexate, azathioprine, leflunomide, or mycophenolate)

Limited disease

Combination of methotrexate and corticosteroids
Steroid dose is reduced after remission and methotrexate is continued as maintenance therapy