The Challenges of Diagnosis and Management of Wegener’s Granulomatosis with Negative ANCA

Abstract

ABSTRACT Wegener’s granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved. Keywords: Wegener’s granulomatosis, Autoimmune vasculitis, negative ANCA