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Vasculitis in Patients With Sarcoidosis: A Single-Institution Case Series of 17 Patients
Bradly A Kimbrough1, Kenneth J Warrington1, Hannah E Langenfeld2
1From the Division of Rheumatology.
Insights
Vasculitis can affect any blood vessel size in patients with sarcoidosis. Most patients achieved remission with various treatments, regardless of vessel size, highlighting the importance of recognizing this association.
Area of Science:
- Rheumatology
- Internal Medicine
- Vascular Medicine
Background:
- Vasculitis is a rare complication in patients diagnosed with sarcoidosis.
- Limited data exists on the clinical characteristics and outcomes of vasculitis associated with sarcoidosis.
Purpose of the Study:
- To describe the clinical features, treatments, and outcomes of vasculitis in patients with sarcoidosis.
- To compare patients with large/medium vessel vasculitis (L/MVV) versus small vessel vasculitis (SVV).
Main Methods:
- A single-institution retrospective review of medical records from January 1998 to December 2019.
- Inclusion criteria: diagnosis codes for both sarcoidosis and vasculitis.
- Vasculitis diagnosis confirmed by biopsy and/or arterial imaging.
Main Results:
- Seventeen patients were identified: 9 with L/MVV and 8 with SVV.
- Sarcoidosis onset preceded vasculitis in 44% of L/MVV and 38% of SVV cases.
- Complete remission of vasculitis was achieved in most patients (8/9 L/MVV, 7/8 SVV) with diverse therapies.
Conclusions:
- Sarcoidosis-associated vasculitis presents with comparable frequencies of L/MVV and SVV.
- Effective remission of vasculitis is achievable with various treatments, irrespective of the affected vessel size.
- Clinicians should maintain awareness of the potential co-occurrence of sarcoidosis and vasculitis.
Objectives:
Vasculitis in patients with sarcoidosis is rare and can affect any sized blood vessel. Limited information describing this association is available.
Methods:
A single-institution medical records review study was performed reviewing all patients with a diagnosis code for sarcoidosis and vasculitis between January 1, 1998, and December 31, 2019. Data were abstracted regarding diagnosis, treatment, and outcomes from medical records. Patients were diagnosed with vasculitis based on biopsy and/or arterial imaging. Comparison between patients presenting with large and/or medium vessel vasculitis (L/MVV) versus patients with only small vessel vasculitis (SVV) was performed.
Results:
Seventeen patients were identified during the study period. Nine patients (56% female) had L/MVV, and 8 (50% female) had SVV. Sarcoidosis preceded vasculitis in 4 (44%) L/MVV and 3 (38%) SVV. The mean ± SD age at sarcoidosis diagnosis was 53.2 ± 17.8 and 51.9 ± 11.4 years, and the mean ± SD age at vasculitis diagnosis was 57.4 ± 19.6 and 59.0 ± 13.4 years in L/MVV and SVV, respectively. Number of organ systems involved by sarcoidosis was similar (median [interquartile range], 3 [1-4] L/MVV vs 2.5 [1.75-3.25] SVV). The mean length of follow-up was 11.5 ± 12.8 in L/MVV and 13.1 ± 14.3 years in SVV. Complete response to therapy for vasculitis was observed in 8 of 9 with L/MVV and 7 of 8 with SVV. Four patients with SVV were able to stop all immunosuppression as compared with only 1 patient with L/MVV at the last follow-up.
Conclusions:
This series observed a comparable number of patients with L/MVV and SVV. Although a variety of treatments were used, most patients achieved remission regardless of vessel size affected. Clinicians should be aware of the overlap between sarcoidosis and vasculitis.
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