Giant cell arteritis (GCA) is a systemic vasculitis that commonly co-occurs with polymyalgia rheumatica (PMR) in elderly patients. Pericardial disease is an unusual manifestation of these inflammatory conditions, which has been reported only in case reports and small observational studies. However, no extensive research has been performed to study the demographics and clinical history of GCA or PMR patients with concomitant pericardial features. As a result, the medical evidence to help guide the physicians when evaluating such individuals is limited.
To perform a systematic review of the medical literature in order to summarize the epidemiological and clinicopathological aspects of this unique association.
We conducted an extensive search of PubMed, Cochrane Library, Ovid, Google Scholar, and gray literature to identify all the cases of GCA and PMR with pericardial involvement. The demographics, clinical features, and outcomes of the final cohort were reviewed and analyzed.
The analysis comprised 52 clinical cases (51 identified from 46 articles and 1 from the residents' clinic). These included 44 patients with GCA and 8 with PMR. The mean age at presentation was 69.5 years, with only 46% of patients older than 70 years. The most common abnormality was pericardial effusion (85%), and in 37%, the pericardial event was the initial disease manifestation. Although a significant proportion of the patients were symptomatic (69%), the classic cranial symptoms were present in only 40%. Overall, the outcome was good even in the presence of large-vessel disease, which is usually a poor prognostic factor in classic GCA. On group analysis, patients with PMR were more likely to develop cardiac tamponade (37.5%; odds ratio, 25.8; confidence interval, 2.2-297.5; p = 0.01), whereas those with GCA were more likely to have large-vessel vasculitis (43%; odds ratio, 5.18; confidence interval, 0.58-252.1; p = 0.04).
This study illustrates that patients with pericardial involvement represent a clinical phenotype of GCA (and possibly PMR), which is quite different from the cranial or large-vessel forms. These patients have a better prognosis likely due to younger age and presence of more overt symptoms resulting in early diagnosis.