Toll of delayed diagnosis in giant cell arteritis with atypical presentations

Authors: Saerom Youn, Etienne Benard‐Seguin, Jeremy Moreau, Abdullah Al‐Ani, Fiona Costello. Division of Ophthalmology, Department of Surgery, University of Calgary.

Author Disclosures:  S. Youn:  None.  E. Benard-Seguin:  None.  J. Moreau:  None.  A. Al-Ani:  None.  F. Costello: None

Abstract Body: 

Purpose: Cell Arteritis (GCA) is a systemic vasculitis that targets medium to large vessels. Protean manifestations of GCA can make for a diagnostic challenge and can delay lifealtering treatment. We present 3 cases of atypical presentation of GCA encountered in a tertiary neuro‐ophthalmology center that led to delayed diagnosis and permanent vision loss. 

Study Design: Case series 

Methods: We retrospectively reviewed 3 cases of atypical presentations of GCA encountered in a tertiary neuro‐ophthalmology center. 

Results: Two males and one female between 62 and 78 years of age with temporal artery biopsy (TAB) proven diagnosis of GCA were included. Two patients presented with upper limb and posterior neck pain. MRI revealed posterior cervical myositis in one patient, while the other was seen by dentists and neurologists for positional
vertigo and musculoskeletal pain, and received cortisone injections without improvement. One patient with a 50‐pack‐year smoking history and lung nodules presented with bilateral intraconal inflammation on orbital imaging. Two patients experienced unintentional weight loss of 37 and 20 lbs. They had limited symptoms of cranial arterial ischemia which were not their primary complaints at initial presentation: one patient experienced mild scalp tenderness; another patient noted jaw claudication, and the last patient experienced headaches. All three patients had elevated serum Creactive
protein and sedimentation rates, thrombocytosis, and anemia prior to diagnosis. Due to their atypical presentations, all patients underwent extensive investigations to rule out infectious, rheumatologic, cerebrovascular, hematologic, and neoplastic etiologies of their clinical presentations. Unfortunately, all patients experienced permanent vision loss due to delayed diagnosis and treatment. 

Conclusions: GCA can present with unexpected inflammatory prodrome that is often hard to dissociate from other autoimmune, infectious, and malignant disorders. Failure to consider GCA may lead to unnecessary investigations, and delay treatment thus leading to preventable permanent vision loss. Clinicians should be aware of atypical presentations of GCA for timely diagnosis and management.

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