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

Abstract

ABSTRAK Penyakit Granulomatosis Wegener merupakan suatu penyakit autoimun yang mengenai dinding pembuluh darah terutama pembuluh darah kecil dan sedang akibat adanya reaksi komplek imun. Sampai saat ini etiologi penyakit Granulomatosis Wegener belum diketahui secara pasti. Diagnosis Granulosis Wegener salah satunya adalah terdeteksinya antibodi cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), akan tetapi pemeriksaan ANCA negatif atau tidak ditemukan pada kasWegener’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.us ini sangat jarang terjadi. Artikel ini membahas mengenai kasus laki-laki 33 tahun datang dengan keluhan luka pada kedua tungkai kaki yang sulit sembuh disertai nyeri pada sendi, demam pada malam hari, penurunan berat badan, rambut rontok dan mimisan berulang yang tidak diketahui penyebabnya. Pada pemeriksaan fisik ditemukan saddle nose, terdapat bercak kehitaman dari kedua pangkal paha kanan dan kiri sampai ke punggung kedua kaki.  Pada regio cruris dan dorsum pedis didapatkan adanya ulkus multiple, ukuran bervariasi, irregular. Hasil pemeriksaan laboratorium menunjukkan Hb 8,7 gr/dl, LED 130 mm/jam, Hapusan darah tepi: anemia hipokromik-mikrositik suspek et causa proses kronis bersamaan dengan proses perdarahan. ANA-IF pola speckled 1:320, ANCA tes hasil negatif (-). Hasil pemeriksaan urin rutin makroskopis darah +4, dan mikroskopis ditemukan leukosituria 2-10 LPB, eritrosituria 8-21 LPB. Hasil USG doppler pada regio ekstremitas inferior sinistra menunjukkan kesan: swelling jaringan lunak pedis sinistra dengan arteritis perifer pada area lesi kutis. Hasil pemeriksaan Patologi Anatomi menunjukkan peradangan kronis non spesifik et regio cruris dan pedis. Tatalaksana yang dilakukan debridement luka oleh spesialis bedah, transfusi pack red cell (PRC), metylprednisolon  mg, azathioprine, cefixime. Hasilnya mimisan sudah tidak dirasakan, nyeri sendi berkurang tetapi masih ada, dan demam membaik. Kata Kunci: Granulomatosis wegener, Vaskulitis autoimun, ANCA negatif  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