Lesions of Sarcoidosis: A Problem Solving Approach Om P Sharma, Violeta Mihailovic-Vucinic
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1CLINICAL FOCUS SERIES: Lesions of Sarcoidosis A Problem Solving Approach2
3CLINICAL FOCUS SERIES: Lesions of Sarcoidosis A Problem Solving Approach
Late Om P Sharma MD FRCP Master FCCP Violeta Mihailovic-Vucinic MD PhD Professor of Internal Medicine and Pulmonology Medical Faculty, University of Belgrade Serbia Head of Department of Sarcoidosis and Other Granulomatous Diseases Clinic of Pulmonology, Clinical Center Belgrade Serbia Founder of the Serbian Association of Sarcoidosis Foreword Robert P Baughman Elyse E Lower
4
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Lesions of Sarcoidosis: A Problem Solving Approach
First Edition: 2014
9789350904237
Printed at
5Dedication
Professor Om P Sharma (1936–2012) was born in India, graduated from Victoria College, and attended G.R. Medical College—both in his native land. He then studied tropical medicine at the London School of Hygiene and Tropical Medicine. He served internships in medicine in India, Scotland, and England and a residency at Albert Einstein Medical College, Bronx, New York, where he also completed the fellowship in cardiopulmonary medicine.
Dr Sharma was Professor of Medicine at the Keck School of Medicine at the University of Southern California, Los Angeles, for over 40 years. Dr Sharma was also Professor of Medicine in Residence at Kyoto University Medical School, Kyoto, Japan, 1987–88. He was emeritus President of the World Association of Sarcoidosis and other Granulomatous Disorders (WASOG) and Editor of Current Opinion in Pulmonary Medicine.
During his lifetime, Professor Sharma authored or edited eight books, two medical biographies, eight monographs, and has written over 500 articles, short papers, editorials and reviews on the topics of sarcoidosis, tropical medicine, hypersensitivity pneumonitis, interstitial lung disease, other pulmonary disorders, as well as the history of medicine.
This is the last book Professor Sharma wrote. His original idea for the book, Lesions of Sarcoidosis: Problem Solving Approach rose in the summer of 2011. Although suffering with a devastating disease, he had brilliant ideas about how this book should look and how it would contribute both to doctors and patients.
His list of chapters was outstanding but demanding, as was always his scientific approach.
“There are many lesions of sarcoidosis”, Professor Sharma wrote in discussing this book with me, and how he imagined our whole project from cover to cover: “We need to make a list of these lesions and then write a chapter on each. Each chapter should be not large; it should be easily readable, contain nice image(s) of the lesion 6(one, maybe two), a table of differential diagnosis, and brief clinical importance. I envision this book as a clinical book for trainees, general practitioners, and internists…. Of course it will be useful to specialists as well.”
Enthusiasm, an endless enthusiasm, is the only way to describe his work on this book—chapter upon chapter, piles of text with such an ease and simplicity. Professor Sharma was an exceptional writer.
“Variety is a spice of life”, he used to say, trying to find different cases for each chapter. None are similar to the previous ones. “Sarcoidosis has many faces…”
Professor Sharma wrote while on chemotherapy, he wrote from the bed recovering, he wrote from everywhere. Unfortunately, he did not manage to see this book published, although it was almost finished a couple of weeks before his death.
“In nothing do men more nearly approach the gods than in giving health to men…” These ever inspiring words of Cicero accurately represent the entire life path and the dedication of Professor Sharma whose driving purpose was to give health to his patients.
This book, the last one, is only a small contribution to his life—s work.
Violeta Mihailovic-Vucinic
9PREFACE
Sarcoidosis is a multisystem granulomatous disease of unknown cause and unpredictable course. Since the first description of the mysterious skin lesions of Lady Mortimer by Jonathan Hutchinson, more than one century ago, sarcoidosis remains a puzzling disease in many aspects. Although remarkable advances in investigations of etiology, pathogenesis, and management of sarcoidosis have been made, it still remains inexplicable in many clinical points.
The book “Lesions of Sarcoidosis: A Problem Solving Approach“ is the last one written by Professor Sharma. His original idea was that this book would be of use not only to pulmonologists and internists, but also for practitioners of other specialists because of multisystem nature of sarcoidosis.
Professor Sharma sincerely hoped that this book should be a picturesque guide for all doctors who ever intended to explore numerous manifestations of this mysterious disease.
The main purpose of the book is to provide medical fraternity a comprehensive review of possible clinical presentations of sarcoidosis.
Unfortunately, Professor Sharma did not manage to see the book finally published, although he spent his last days busy working on it. Working on this book with him, I was truly amazed with his enormous energy and enthusiasm he enriched each single chapter with.
Writing this preface instead of Professor Sharma is heartbreaking and hard task.
I try to remember how he envisioned the whole book; at the same time I try not to forget all the persons who contributed in each conceivable way to the final look of this book as certainly he would do.
The book “Lesions of Sarcoidosis” was supported by our sarcoidosis patients as Professor Sharma used to say: ”who are permanently teaching us about many faces of this mysterious disease”.
The support of our families was of the utmost value: Margaret Sharma who helped with all her heart in all necessary details about the final look of this book; my sons Dr Nikola Vucinic and Strahinja-Laza, were supporting me in all difficult moments.
I am particularly grateful to the whole medical staff of the Vth Clinical Department-Clinic of Pulmonary Diseases, University Clinical Center Belgrade, who helped me with this work. My appreciations to the efforts of my Chief of Staff—the 10Main Nurse of the Vth Clinical Department, Mrs Ruzica Nikodijevic on helping me in every possible way.
Kind appreciations to my dear colleagues Professor Elyse E Lower and Professor Robert P Baughman from the University of Cincinnati for the breathtaking foreword.
The final look of this book was made by painstaking efforts of Dr Madhu Choudhary, Senior Editor Jaypee Brothers Medical Publishers team and my most sincere appreciations belongs to her.
Authors appreciate all sarcoidosis patients from every conceivable corner of the world for their assistance. They are permanently teaching us about many faces of this mysterious disease.
Violeta Mihailovic-Vucinic
11FOREWORD
Robert P Baughman MD
Professor of Medicine, Department of Medicine
University of Cincinnati Medical Center, Cincinnati, OH, USA
Elyse E Lower MD
Professor of Medicine, Department of Medicine
University of Cincinnati Medical Center, Cincinnati, OH, USA
Professor Jacques Chretien once said: “If you have seen five cases of sarcoidosis, you know nothing about sarcoidosis. If you have seen 50 cases of sarcoidosis, you know everything about sarcoidosis. If you have seen 500 cases of sarcoidosis, you know nothing about sarcoidosis.” For many clinicians, sarcoidosis remains an elusive disease without a clear-cut etiology and no specific test which confirms the diagnosis in all cases. Although the disease can affect virtually all organ systems, there is no area of the body, including the lung, which is always involved. Additionally, radiographic and blood tests frequently fail to correlate with clinical symptoms. Some patients will be asymptomatic but display markedly abnormal chest X-rays; whereas, other patients may suffer from extreme cough, dyspnea, and fatigue but have normal chest imaging.
The multiorgan nature of sarcoidosis can both be a blessing and a curse. The finding of granulomas in two or more organs can be a blessing for confirming the diagnosis of sarcoidosis. Because the combination of eye and lung disease supports the diagnosis of sarcoidosis, a patient with mediastinal adenopathy and active uveitis is more likely to have sarcoidosis and less likely to have Hodgkin—s lymphoma. However, this multisystem involvement may also be a curse when a patient presents to a neurologist or cardiologist who infrequently treats sarcoidosis and hence fails to recognize this granulomatous process.
“Lessons of sarcoidosis” provides a unique approach to assist clinicians in better understanding the multifaceted nature of this disease. In contrast to most standard textbooks which summarize tables and figures for the “average” sarcoidosis patient, Professors Sharma and Mihailovic-Vucinic have provided clinical scenarios that may be encountered by practicing clinicians faced with diagnosing and treating sarcoidosis. This practical venue enables the clinician to appreciate the vast 12differences in patient presentation, organ involvement, and outcomes seen in sarcoidosis. Clinical vignettes examine both focused questions important for some specific patients, such as lupus pernio, as well as more generalized issues common to most sarcoidosis patients, such as pain, dyspnea, and fatigue.
The prestigious authors of this book have successfully managed thousands of patients from two international sarcoidosis clinics for many years. Because of this extensive combined expertise, this book reflects a shared international experience for both the usual and the unusual aspects of the disease.
We believe that after reading this book one will be not only as enthralled with sarcoidosis as we are but also have a better understanding of this chameleon like disease.