An unusual case of ELKS (E – ear, nose and throat, L – lungs, K – Kidneys, S – skin and mucus membranes) constellation with gastrointestinal vasculitis in a patient with Wegener’s granulomatosis
Omotayo Olatinwo, Aduse Opoku-Agyeman, Uma Jamched, Chinedu Ivonye, Balsam Elhammali
Am J Case Rep 2008; 9:224-228
Background: Wegener’s Granulomatosis (WG) is a rare systemic vasculitis syndrome which can affect any system of the body. The prevalence varies from 24 to 154 cases per million. The condition can occur at any age, but peaks between the ages of 35 to 55 years. The male to female ratio is approximately1:1-2.1. The ELKS (E – ear, nose and throat, L – lungs, K – Kidneys, S – skin and mucus membranes) involvement along with gastrointestinal vasculitis presenting as gastrointestinal bleeding is a rare phenomenon and we present a Caucasian female with this presentation.
Case Report: A 59-year-old female presented with painful ulcers in the oral pharynx and multiple areas of the skin. Her exam was otherwise negative. But laboratory tests showed renal impairment. Chest CT revealed cavitary lung lesions. C-ANCA and anti-proteinase 3 antibodies were elevated. During the hospital stay she had an episode of gastrointestinal bleeding. She eventually had a lung and kidney biopsy with colonoscopy which all confirmed the diagnosis of vasculitis consistent with Wegener’s Granulomatosis.
Conclusions: If untreated Wegener’s granulomatosis has a poor prognosis, with most patients dying within two years. The use of cyclophosphamide has dramatically increased the longevity of these patients hence prompt diagnosis and treatment of this condition is extremely important, especially in atypical presentations as seen in our patient. Since gastrointestinal vasculitis presenting with gastrointestinal bleeding is rare in patients with WG, confirmatory endoscopic biopsies should be done before attributing this to steroids used commonly in the management of these patients.
Keywords: systemic vasculitis, Wegener ganulomatosis, Gastrointestinal Hemorrhage - blood