Rheumatology (Oxford) 2015 Mar 5;54(3):463-70. Epub 2014 Sep 5.
Division of Rheumatology, Department of Medicine, Arcispedale S. Maria Nuova IRCCS, Reggio Emilia, Italy, Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, Division of Rheumatology, Department of Medicine, Division of Biostatistics, Department of Health Sciences Research and Division of Epidemiology, Department of Health Sciences Research, Mayo Clinic, Rochester, MN, USA.
Objective: The aim of this study was to compare baseline variables, treatment and outcomes in patients with large-vessel GCA (LV-GCA), primarily of the upper extremities, with those with cranial disease (C-GCA).
Methods: All patients >50 years of age with radiographic evidence of subclavian LV-GCA diagnosed between 1 January 1999 and 31 December 2008 were identified and compared with those with biopsy-positive C-GCA diagnosed in the same period.
Results: The study included 120 LV-GCA patients and 212 C-GCA patients. Compared with C-GCA, patients with LV-GCA were younger [68.2 years (s.d. 7.5) vs 75.7 (7.4), P < 0.001] and had longer duration of symptoms at GCA diagnosis (median 3.5 vs 2.2 months, P < 0.001). A history of PMR was more common in LV-GCA patients (26% vs 15%, P = 0.012), but a smaller proportion had cranial symptoms (41% vs 83%, P < 0.001) and vision loss (4% vs 11%, P = 0.035). ACR classification criteria for GCA were satisfied in 39% of LV-GCA patients and 95% of C-GCA patients (P < 0.001). Compared with C-GCA, patients with LV-GCA had more relapses (4.9 vs 3.0/10 person-years, P < 0.001), higher cumulative corticosteroid (CS) doses at 1 year [11.4 g (s.d. 5.9) vs 9.1 (s.d. 3.7), P < 0.001] and required longer treatment (median 4.5 vs 2.2 years, P < 0.001).
Conclusion: Although patients with LV-GCA had a lower rate of vision loss, they had a higher relapse rate and greater CS requirements. The ACR criteria for GCA are inadequate for the classification of patients with LV-GCA.