Abstract Sarcoidosis is a systemic inflammatory granulomatous disease for which rheumatologists are uniquely trained and qualified to treat. Historically, sarcoidosis has been managed within silos of medical subspecialties, but with…
Abstract Relapsing polychondritis (RP) is a very rare autoimmune disease characterised by a relapsing inflammation of the cartilaginous tissues (joints, ears, nose, intervertebral discs, larynx, trachea and cartilaginous bronchi), which…
Abstract Multicentric reticulohistiocytosis (MRH) is a rare, multisystemic non-Langerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. There are no specific laboratory findings for…
Abstract Uveitis may exist as a clinical manifestation of an underlying systemic disease or may represent an idiopathic entity, sometimes with a very characteristic pattern. Different forms of uveitis have…
Abstract Familial Mediterranean fever (FMF) is the most common form of monogenic autoinflammatory conditions, and response to colchicine has been considered as one of its distinctive features among other hereditary…
Abstract Behçet’s disease is a multisystem disorder with unknown etiology and a unique geographic distribution. The disease is characterized by recurrent skin–mucosa lesions and sight-threatening panuveitis. Vascular involvement, which is…
Abstract IgG4-related disease is a rare immune-mediated systemic disease with the capability of involving essentially any organ. Although the presenting clinical features vary substantially according to the speciality to which…
Abstract The current recommendation for catastrophic antiphospholipid syndrome (CAPS) management is the standard triple therapy with anticoagulation (AC), glucocorticoids (GCs), plasma exchange (PE), and/or intravenous immunoglobulins (IVIGs). Of note, only…
Abstract Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder mainly characterized by persistent high spiking fevers, evanescent rash, and joint involvement. The pathogenesis of AOSD is only partially…
This issue of Best Practice & Research Clinical Rheumatology is about a variety of systemic diseases or subgroups of systemic diseases that are relatively rare, clinically important, and can create…
