I served on the editorial board of The Yearbook of Rheumatology, Arthritis, and Musculoskeletal Disease throughout its lifetime, three years as an associate editor to John Sergent, MD, and 10 years as editor. (Dr. Pisetsky served as an associate editor.) We took pride in creating something special and thought ours was the best of the Yearbook series. We valued critical thinking, eclectic selections, and eloquent and provocative expositions. The perspectives were refreshing and erudite. Presentations were personalized, relevant, practical, entertaining, and challenging. It is that style I will try to introduce for this department in The Rheumatologist.
I welcome your comments, responses, suggestions for inclusions, or disagreements. Together we will have fun thinking and learning about rheumatology.
THERE’S NOTHING NEW UNDER THE SUN. NOT!
Whenever possible, I will make thematic selections and from literature that perhaps not all clinical rheumatologists would routinely read or be aware of. The story line for today is new diseases and syndromes of clinical and intellectual interest to rheumatologists.
When I spoke with John Stone at his poster about immunoglobin 4 (Ig 4)–related systemic disease at last fall’s ACR meeting in Philadelphia, I was embarrassed at not being familiar with this, until I realized it is a relatively new concept (Arthritis Care Res. 2010;62:316-322). Until recently, most reports were single cases, focused on pathology or immunopathology, or were reported in obscure journals. We will probably encounter this as “noninfectious” aortitis or chronic periaortitis. These patients should have elevated serum IgG4 levels and may have systemic disease such as Sjögren’s syndrome or involvement of biliary tract, liver, lung, kidney, lymph nodes, pancreas, or retroperitoneum. Aortitis responds well to glucocorticoids. This is something I will now look for.
I probably won’t look for patients with deficiency of the interleukin (IL)-1 receptor antagonist (DIRA) (N Engl J Med. 2009;360:2426-2437). These patients have neonatal sterile multifocal osteomyelitis, periostitis, pustolosis, osteopenia, lytic bone lesions, respiratory insufficiency, and thrombosis. The absence of the IL-1-receptor antagonist, caused by autosomal recessive mutations, permits unopposed action of IL-1, allowing elaboration and overproduction of proinflammatory chemokines and cytokines, leading to these devastating clinical manifestations. Patients responded to treatment with ankinra. Why, though, would this immunodeficiency be associated with these particular clinical symptoms?
I may, however, consider the syndrome of autoimmune polyendocrinopathy type I with chronic mucocutaneous candidiasis (J Exp Med. 2010;207:291-297). These patients have autoantibodies against IL-17A, IL-17F, and/or IL-22. (These cytokines are important in host defense against candida in mice, which formed the basis of the rationale for this study.)
