19.2.5 Wegener’s Granulomatosis

Wegener’s granulomatosis is a form of vasculitis that affects the lungs, kidneys, and other organs. Due to its end-organ damage, it can be a serious disease that requires long-term immune suppression. It is named after Dr. Friedrich Wegener, who described the disease in 1936 [34].

19.2.5.1 Pathogenesis

Wegener’s granulomatosis is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) and affect smalland medium-sized blood vessels. On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation (clumps of typically arranged white blood cells) on microscopy. These granulomas are the main reason for the appellation of “Wegener’s granulomatosis,” although it is not an essential feature. Unfortunately, many biopsies can be nonspecific, and 50% provide too little information for the diagnosis of Wegener’s.

19.2.5.2 Ocular Manifestation

Fifty percent to 60% have ophthalmologic manifestations, which can be a presenting feature in a minority of patients. Orbital disease is the most common manifestation and may result in proptosis, restrictive ophthalmopathy, chronic orbital pain, and, in chronic cases, orbital retraction syndrome and intractable socket pain. Wegener’s may also cause injury to the optic nerve, ophthalmoplegia, conjunctivitis, keratitis, scleritis, episcleritis, dacryocystitis, nasolacrimal duct obstruction, dacryoadenitis, uveitis, and retinal vasculitis. Initial treatment is generally with corticosteroids and oral cyclophosphamide. Once remission has been achieved, azathioprine and steroids can be used to maintain remission.

19.2.5.3 Diagnosis

In 1990, the American College of Rheumatology accepted the classification criteria for Wegener’s. These criteria were not intended for diagnosis but for inclusion in randomized controlled trials. Two or more positive criteria have a sensitivity of 88.2% and

a specificity of 92.0% of describing Wegener’s http:// emedicine.medscape.com/article/332622-overview.

Nasal or oral inflammation:

•Painful or painless oral ulcers or purulent or bloody nasal discharge

Lungs: abnormal chest x-ray with:

•Nodules, infiltrates, or cavities Kidneys: urinary sediment with:

•Microhematuria or red cell casts Biopsy:

•Granulomatous inflammation within the arterial wall or in the perivascular area

19.2.6 Kawasaki Disease

Kawasaki disease is a poorly understood selflimited vasculitis that affects many organs. Usually in children (age < 4), it affects large, medium, and small vessels, prominently the coronary arteries, and is associated with mucocutaneous lymph node syndrome [5].

19.2.6.1 Clinical Characteristics

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of paracetamol (acetaminophen) or ibuprofen. The fever may persist steadily for up to 2 weeks andisnormallyaccompaniedbyirritability.Affected children develop red eyes, red mucous membranes in the mouth, red cracked lips, a “strawberry tongue,” iritis, keratic precipitates (detectable by an ophthalmologist but usually too small to be seen by the unaided eye), and swollen lymph nodes. Skin rashes occur early in the disease, and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness.

19.2.6.2 Diagnosis

Classically, 5 days of fever [5] plus at least four out of five criteria:

•Bilateral conjunctival injection

•Injected or fissured lips, injected pharynx, or strawberry tongue

•Erythema of palms/soles, edema of hands/ feet, periungual desquamation