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Rheumatic Fever and Streptococcal Infection
  1. University Department of Rheumatology, Basle, Switzerland

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    Rheumatic Fever and Streptococcal Infection.Unravelling the Mysteries of a Dread Disease. By Benedict F Massell. (Pp 394; $25). Cambridge, MA: Harvard University Press, 1997. ISBN 0-674-76877-9.

    For most physicians reading this book in the developed world, rheumatic fever is a condition studied at medical school, rarely encountered in clinical practice and not considered a “dread disease” in comparison with, for example AIDS. Armed with the Jones criteria and bountiful antibiotics we feel confident in dealing with rheumatic fever, sometimes forgetting that in the developing world it remains a major problem, and that mini epidemics still occur in the USA and Europe.

    This extraordinary work from Dr Benedict Massel is a timely reminder that rheumatogenic group A haemolytic streptococci are still present and still defying an effective vaccine development.

    Dr Massel has accomplished in one concise and readable book (286 pages of text and 107 of notes and references) a comprehensive clinical and scientific review of rheumatic fever that could only be written by one who was there caring for and performing research with patients suffering from this then truly “dread disease”. His style is clear and precise but in no way dry. In the early historical review, the painstaking clinical and postmortem observations of workers in the 17th and 18th centuries are described with generous original quotations from their work. The major manifestations were well known by the end of the 19th century including the observations by Barlow that in children, joint manifestations were less severe than in adults, in contrast with the cardiac features. This is still of relevance to rheumatologists today dealing with so called post-streptococcal reactive arthritis.

    The narrative proceeds into the 20th century with early epidemiological observations, Schottmüller’s identification of the haemolytic streptococcus (1903), Lancefield’s laborious classification of haemolytic streptococcal subtypes in 1928, and Schlessinger’s observation that the throat infection and lag period was aetiological, rather than another manifestation of rheumatic fever. The final link between a necessary haemolytic streptococcal throat infection and rheumatic fever was made independently by the English physician Collis and the American Coburn in 1931. These landmark developments are fully described.

    Chapters 8, 9, and 10 deal with the development of antibiotics from sulphonamide (Domagk 1932), to penicillin (Fleming 1929, further developed by Chain and Flory in 1940). These chapters stand alone as a coherent description of this extraordinary period of the human conquest over the pathogen, and we learn that Massel himself was the first to apply sulphonamide (sulphanilamide or “Prontylin”) to treat streptococcal throat infection in patients with rheumatic fever. Although this was unsuccessful in preventing rheumatic fever, later studies, also involving Massel, showed the now established fact that prevention of the primary infection rather than treatment was necessary.

    We learn somewhat surprisingly that Duckett Jones, enshrined in the medical literature for his diagnostic criteria of rheumatic fever (1944) continued to doubt the unique and obligatory association with haemolytic streptococcal infection until his death in 1954. Massel allows himself a gentle criticism of Jones’ lack of accreditation to some other key workers in the field concerning this.

    The later chapters deal with successful prevention programmes in military institutions in the second world war (later reflected in disquieting mini epidemics in US military institutions in the late 1980s after ceasing prophylactic penicillin programmes) and the evolving understanding of the immunogenetics (Zabriskie) and immunopathology. The section on emerging knowledge of T cell mediated autoimmune events is somewhat scanty, but does not detract from the overall relevance of this exhaustive review.

    The final chapters on vaccine development, especially dealing with non-type specific engineered vaccines containing conserved regions of the M molecule (Fischetti) take us into the next millennium with an optimistic tone. This is especially relevant for developing countries for which expensive antibiotics and improved housing and social circumstances are unfortunately not immediate options.

    The lessons learned from reading this book concerning the linking of careful, valid, clinical, and pathological observations to laboratory findings and eventually logical clinical trials could apply to any field of medicine today. Since acquiring this book to review, I have already used it several times as a reference text (the indexing and referencing are excellent) and strongly recommended it to anybody concerned with the relation between microbes and rheumatic disease despite the fact that it deals exclusively with rheumatic fever and the haemolytic streptococcus.

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