BACKGROUND: Giant cell arteritis (GCA) or temporal arteritis (TA) is a necrotizing medium to large vessel arteritis of unknown etiology that was described in 1932 by Horton. It commonly afflicts elderly people and may present with diverse manifestations such as severe headache, impairment of vision, polymyalgia rheumatica, tenderness of the temporal arteries, a highly elevated erythrocyte sedimentation rate (ESR) and a characteristic abnormal temporal artery biopsy. It is rare in individuals less than 50 yrs old. Age of more than 50 yrs old is the first of five clinical criteria formulated by the American College of Rheumatology. In the largest series reported, all patients were ?50 years, and 95% of them were >60 years. In the last three decades, only 13 patients <40 years old have been reported with TA. We report here a case of a relatively young patient presenting with clinical features of TA with a negative temporal artery biopsy.
SETTING: St. Luke's Medical Center- a tertiary care hospital
KEYWORDS: Giant cell arteritis; temporal arteritis
CASE REPORT: Patient is a 41 y/o male who presented with a 3 week history of severe left sided frontal headache with intermittent episode of moderate grade fever, blurring of vision, diplopia on the left eye and left jaw claudication. This was followed by an onset of moderate pain over the right shoulder with limited range of motion. Pertinent physical examination revealed a pulsating, warm and tender to touch left temporal artery, with scalp tenderness. The right temporal artery was essentially normal. There was no Brundzinksi's and Kernig's signs. Other neurologic examination were unremarkable. Laboratory investigations revealed: Complete blood count (CBC) Hgb 11.6 g/dl, Hct 33.5%, RBC 4.06 mil/mm3, white blood count at 6,240 mm3, neutrophils 68%, lymphocytes 22%,monocytes 9% and platelet count of 144,000/mm3 ,ESR 90 mm/hr., CRP 34.9 mg/dl. Brain CT scan showed normal results as well as cerebrospinal fluid analysis. A left temporal artery biopsy with length of 2cm was obtained. It was grossly palpably normal but histopathology revealed intimal fibrosis of the tunica media devoid of any inflammatory infiltrates. He was managed as a case of temporal arteritis and was started on prednisone at 1mg/kg/day with remarkable improvement and had follow-up 3 months later free of complications on tapering dose of corticosteroids.

Human ; Male ; Adult ; Adrenal Cortex Hormones ; Diplopia ; Giant Cell Arteritis ; Lymphocytes ; Neutrophils ; Polymyalgia Rheumatica ; Rheumatology ; Temporal Arteries ; Tunica Media ;

Background: Antiphospholipid syndrome (APS) is an autoimmune, pro-thrombotic disorder in the presence of circulating antiphospholipid antibodies. EvanÊs syndrome is defined as the presence of autoimmune hemolytic anemia and autoimmune thrombocytopenia is strongly associated with APS and presents a difficult management challenge. Although experience remains limited and uncontrolled mainly due to the prohibitive costs of the drug, the use of intravenous immunoglobulin (IVIg) ???? in combination with high dose steroids - may be beneficial - with reported decline in anticardiolipin antibodies - in the treatment of APSrelated manifestations including EvanÊs syndrome, especially when anti-coagulation is contra-indicated. Setting: St. Luke's Medical Center - a tertiary care hospital

Antiphospholipid Syndrome ; EvansÊ Syndrome ; Intravenous Immunoglobulin (ivig)