REVISTA BRASILEIRA DE MEDICINA INTERNA

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1 REVISTA BRASILEIRA DE MEDICINA INTERNA Case Report Systemic Giant Cell Arteritis: unusual clinical manifestation and challenges in management Arterite de células gigantes sistêmica: manifestação clínica incomum e difícil manejo Autores: Cesar Roberto Busato, Cintia Doná Busato, Maria Fernanda Gauer, Marcelo Derbli Schafranski, Marcelo de Oliveira Dreweck, Mário Rodrigues Montemor Netto Instituição Proponente: Serviço de Angiologia e Cirurgia Vascular do Hospital Universitário Regional dos Campos Gerais (HURCG) RESUMO Ataxia como sintoma predominante em arterite de células gigantes (ACG) é uma ocorrência incomum. Homem de 67 anos, com fraqueza progressiva em MMII, rigidez articular em mãos, punhos e dor cervical, desenvolve cefaléia temporo-parietal persistente e ataxia. Este caso de arterite de células gigantes sistêmica mostra ao PET SCAN comprometimento difuso da aorta ascendente, subclávias, artérias carótidas comuns, femorais e poplíteas. Biópsia de artéria temporal com trombose, infiltrado linfo-plasmocitário e presença de células gigantes. Dificuldade na descontinuação do tratamento com corticosteróide. PALAVRAS-CHAVE: arterite de células gigantes; ataxia; artéria temporal. Artigo recebido em: 02/10/2014 Aceito para publicação: 02/12/2014 Autor para correspondência: crbusato@brturbo.com.br (Cesar R Busato)

2 ABSTRACT Ataxia as a predominant symptom in giant cell arteritis (GCA) is uncommon. A 67 year old man with progressive weakness in the lower limbs, stiff joints in the hands and wrists and neck pain developed a temporal-parietal headache and persistent ataxia. This case of systemic giant cell arteritis showed diffuse involvement of the ascending aorta and subclavian, common carotid, femoral and popliteal arteries in PET SCAN. Biopsy of the temporal artery with thrombosis showed lymphoplasmacytic infiltration and giant cells. Discontinuation of treatment with corticosteroids was unsuccessful. KEYWORDS: prednisone; side effects; corticoid therapy CASE REPORT A 67 year old man presented with a history of five months of progressive lower limb weakness associated with joint stiffness in the hands and wrists and neck pain radiating to the shoulders. The patient lost 8 kg during this time. His condition worsened five days prior to seek treatment with development of a persistent left temporal-parietal headache, including difficulty walking and maintaining balance. He presented with a wide-based ataxic gait. He was treated for carpal tunnel syndrome and depression. There were no focal neurological deficits noted on physical examination. The left temporal artery was pulseless, thickened and sensitive to palpation. Allen s test was positive with compromised bilateral flow in the radial arteries. Femoral and popliteal pulses were bilaterally palpable. Duplex scanning showed monophasic bilateral flow in the posterior tibial arteries and lack of flow in the right dorsalis pedis. The velocity of hemosedimentation (VHS) was 75 mm/h. and C reactive protein(crp) was 4,80 mg/dl. PET SCAN showed metabolic hyperactivity in the pathways of the ascending aorta and subclavian (Figure 1A.), common carotid (Figure 1B.), femoral and popliteal arteries (Figure 1C.). A clinical diagnosis of systemic GCA was established and management with prednisone (60 mg/day) and aspirin (100 mg/day) was initiated. The patient was referred for a temporal left artery biopsy, which showed an occluded artery (Figure 2A.) with an intense inflammatory lymphoplasmacytic reaction and giant cells (Figure 2B). The patient showed total clinical regression of symptoms and normal gait and balance after one week of treatment. Cervical spine and shoulder (rheumatic polymyalgia) pain resolved. The Romberg test was negative and Allen s test showed normal flow in the radial arteries. Dorsal pedis and posterior tibial pulses were palpable. After two weeks of treatment, VHS was 19 and CRP was Chest radiographs and abdominal ultrasound did not show vascular dilatation of the aorta and its branches. DISCUSSION GCA is a chronic granulomatous systemic inflammatory arterial disease that involves the medium and large arteries 1-2. The clinical course is severe with nonspecific initial manifestations that hinder diagnosis and delay the start of treatment 3-4. Although intracranial involvement is responsible for more severe clinical disease and causes neurological deficits and visual impairments, including blindness, ataxia is an uncommon symptom that is rarely observed as the predominant manifestation 5-6. Prednisone (60 mg/day) was prescribed, following significant symptomatic improvement. Resolution of both myalgia and ataxia was seen as early as one week after the beginning of therapy. Fifteen days after starting treatment, the patient had normal VHS and CRP and showed normal peripheral pulses in the upper and lower limbs. Prednisone at 40 mg/day maintained the VHS and CRP at normal levels.

3 REV. BRAS. MED. INTERNA 2014; 1(1):60-64 Figure 1A Metabolic hyperactivity in the pathways of the ascending aorta and common carotid. Figure 1B Femoral and popliteal hyperactivity. Figure 1C Subclavian hyperactivity.

4 REV. BRAS. MED. INTERNA 2014; 1(1):60-64 Figure 2A Temoral artery biopsy: Left temporal artery occluded Figure 2B Intense inflammatory lymphoplasmacytic reaction and giant cells. Two further attempts to reduce prednisone dosage to levels lesser than 30 mg/day resulted in an increase in VHS and CRP. As a steroid-sparing agent, methotrexate was initiated at a dosage of 15 mg/week orally, later increased to 20 mg/week, with no benefit. So, three monthly infusions of tocilizumab 8 mg/kg were administered, resulting in adequate symptomatic control and inflammatory markers normalization. Unfortunately, the drug has to be withdrawn due to recurring infectious complications (pneumonia and erysipelas). At the moment, leflunomide 20 mg/day was initiated as a second-line steroid-sparing agent and the patient awaits further evaluation. Conclusion This report represents a case of GCA with an unusual clinical presentation and multiple challenges in management.

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