“Despite the amazing advances in medicine since the first edition, so many patients with immune-mediated inflammatory disease remain soaked in steroids!  Steroid-related toxicities continue to be a huge burden for our patients.”

The important advances made in understanding the underlying mechanisms and treatment of many inflammatory conditions over the past decade led to numerous requests for a second edition of the 2009 book, A Clinician's Pearls and Myths in Rheumatology. 

John H. Stone, MD MPH, a Professor of Medicine at Harvard Medical School and the Edward A. Fox Chair in Medicine at the Massachusetts General Hospital, developed and edited both editions and considers Pearls and Myths one of the achievements in his career, of which he is most proud. In that regard he was, “delighted to hear only recently that there were more than 7,000 downloads of the book in the first two weeks alone” after publication. Clearly, the book has tapped into a major unmet need for clinicians.

“There have been so many changes and advances in medicine, disease descriptions, and new therapeutics since the first edition, that this new edition was well overdue. 

However, despite the advances in our field, when it gets right down to it, really good medicine is based on what one can learn from history – from the art of listening to and talking with patients carefully, and an informed physical examination. As diagnosticians and doctors, we ignore those points at our peril.”

Dr Stone highlighted a key thread running throughout the entire book: the continuing overuse of glucocorticoids and their impact on patients.  

“While editing the book I was struck by how often glucocorticoids were a keyword in chapter after chapter. So despite the advances in treatment – which have been substantial even since the first edition – too many patients with immune-mediated inflammatory disease remain soaked in steroids. Consequently, steroid-toxicities continue to be a huge burden for our patients.

Providers and patients need to be alert to the reality of steroid-toxicity. I often think that steroid-toxicity is a bit like the wallpaper in the examining room. It's there while the physician and patient are having their encounter, but too often it is completely ignored, not addressed, and allowed to grow worse. 

So one of my goals for this book is to raise awareness of the possibility of steroid-toxicity and, when possible, give some insights into how this might be mitigated by greater clinician awareness and appropriate use of treatment alternatives. With the Glucocorticoid Toxicity Index, we are now able to measure steroid-toxicity both in clinical trials and in clinical practice. And that is the next key step toward being able to relieve patients of the burden from steroids."

In developing the book, Dr Stone worked with more than 200 contributors, bringing together pearls and myths from experts around the world.

“The rapid advances in medicine make it increasingly hard to be a generalist and stay up to date. One really does need to be able to reach out and access the most current information about a particular problem – there are diseases that weren't in existence (or were there but hadn’t been recognized) when many currently practicing clinicians were in training. In rheumatology alone, we have probably at least ten diseases recently described that weren’t known at the time of the first edition in 2009. I hope this book will enable clinicians to tap into experts and their hard-earned knowledge to put themselves in the best position possible to help patients.”

One new disease description that was only briefly mentioned in the 2009 book was IgG4-related disease, which was only recognized as a systemic disease in 2003. 
 
Rheumatology depends significantly upon clinical wisdom and the development of a system of ‘Pearls’ and ‘Myths’ – a ‘Pearl’ being a nugget of truth about the diagnosis or treatment of a particular disease that has been gained by dint of clinical experience and a ‘Myth’ being a commonly held belief that influences the practice of many clinicians, but is false.  
When asked about this favorite “pearl” in the book, Dr Stone related the story of a specific patient encounter from early on in his career.

“It was on meeting this patient that I became a rheumatologist, and his disease became my ‘first love’ in the field. He was a young man who was a drummer in a rock band. He had decreased hearing in one ear – which could have been related to his drumming, of course – but he also had pulmonary nodules and a positive blood test for a biomarker that was new to the medical scene at the time called an ANCA [antineutrophil cytoplasmic antibody]. After talking with him in detail and after examining him, I flipped through a textbook trying to figure out what his diagnosis was... 

And I came to this chapter on Wegener's granulomatosis, as the disease was known at the time. It turned out that Wegener’s granulomatosis can cause decreased hearing because of inflammation in the middle ear (i.e., otitis media). The book chapter was written by none other than Dr Anthony Fauci! And in reading the description of Wegener’s granulomatosis, it became very clear that this was my patient’s diagnosis. We now call that disease granulomatosis with polyangiitis (GPA). 

That encounter with that patient inspired me to pursue a career in rheumatology. I’m pleased to say that my 22 year old patient from 1992 is now a 53 year old man who has lived very well these three decades, thriving from the improvements in treatment that have happened in that time. In another era, he would likely have died within a year.

So my favorite ‘Pearl’ is from the GPA chapter. And that ‘Pearl’ is that patients with GPA sometimes develop a facial droop; a frightening sagging on one side of their face. And if one is presented with a patient like that in the clinic, one might think of a range of other diagnoses but one should also consider GPA, which causes a facial droop because of its associated otitis media. That inflammation in the middle ear can also compress the seventh cranial nerve, which runs through the middle ear on its way to innervating the muscles of one side of the face. The resulting cranial nerve compression can lead to facial palsy. Understanding that little known neuroanatomical fact can help crack a puzzling case, and it’s a neat ‘Pearl’ I enjoy passing along to trainees.” 

The second edition of A Clinician's Pearls and Myths in Rheumatology is available now from Springer.