Rheumatic masqueraders: mimics of primary vasculitis - a case-based review

ABSTRACT

Introduction

Vasculitis conditions are often serious and sometimes fatal diseases, therefore it is paramount to diagnose correctly and treat appropriately. Mimics of primary vasculitis can include either non-inflammatory syndromes or secondary vasculitis where the underlying etiology of the vasculitis is being driven by infection, malignancy, drug-effect or other.

Areas covered

This review comprises six individual cases of vasculitis mimics. Each case is presented and the clinical, radiographic, and histological features that distinguish the case from primary vasculitis are highlighted. Key mimics in large, medium and small vessel vasculitis are outlined.

Expert opinion

The diagnosis of vasculitis requires a comprehensive assessment of clinical, radiographic, and histologic features. Clinicians should be familiar with mimics of primary vasculitis conditions. In the case of non-inflammatory mimics, it is important to differentiate from primary vasculitides in order to avoid unnecessary and potentially harmful immunosuppression. For cases of secondary vasculitis, identification of the correct etiologic cause is critical because treatment of the underlying stimulus is necessary for successful management and outcomes.

KEYWORDS:

• Vasculitis
• mimics
• infection
• histiocytosis
• vasculopathy

Article highlights

• Diagnosis of vasculitis requires a comprehensive assessment of clinical, radiologic and histologic features and the exclusion of mimicking conditions.

• Histiocytic disorders, such as Erdheim Chester Disease, are hematologic malignancies that can mimic large vessel vasculitis and require treatment with targeted therapeutics.

• Non-inflammatory vasculopathies such as fibromuscular dysplasia, segmental arterial mediolysis and vascular Ehlers Danlos syndrome should be considered in a patient with multifocal dissection and aneurysm.

• Evaluation of small vessel vasculitis should include consideration of etiologic triggers such as infection, malignancy, and illicit drug use.

Declaration of interest

K Warrington provides clinical trail support to Eli Lilly, GlaxoSmithKline and Kiniksa. K Warrington consults ChemoCentryx. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Ethics Statement

This study was reviewed by the Institutional Review Board and deemed exempt. All patients have provided written consent.

Additional information

Funding

This paper was not funded.