Arch Dis Child: first published as 10.1136/adc.33.170.350 on 1 August 1958. Downloaded from THE DIFFERENTIAL DIAGNOSIS OF RHEUMATIC FEVER BY K. A. HALLIDIE-SMITH and E. G. L. BYWATERS From the Rheumatism Research Unit (Medical Research Council), Canadian Red Cross Memorial Hospital, Taplow (RECEIVED FOR PUBLICATION JANUARY 29, 1958) Since the efficacy of continuous daily sulphona- Jones, save that chorea was excluded as a major mide or penicillin administration in the prevention criterion and past rheumatic fever as a minor of attacks of rheumatic fever has now been estab- criterion. In addition, 42 cases were discharged with lished, and the measure recommended not only a diagnosis of 'possible rheumatic fever' and 202 with nationally but by international committees (World a diagnosis of Still's disease, the remaining 1,011 Health Organization, 1957), it has become increas- cases being various other miscellaneous disorders. ingly important to recognize even mild cases of rheumatic fever. At the same time it is equally Group A. Erroneous Initial Diagnoses in Rheumatic essential to avoid diagnosing this erroneously, since Fever Cases the resulting continuous daily prophylaxis is A one-in-six sample of these patients ultimately undesirable from the point of view both of the diagnosed as rheumatic fever was taken, consisting patient and of the tax-payer. We thought therefore of those with surnames beginning with the initials that an account of our observations at this National A, B and C admitted between July, 1947, and June, copyright. Centre for Juvenile Rheumatism over the last nine 1956. In this group of 160 rheumatic fever patients, years would be useful, since over this period there 12 were found definitely to have been initially other- has accumulated considerable experience not only wise diagnosed, though subsequently all satisfied of rheumatic fever, but also of many conditions Duckett Jones' criteria for the diagnosis of rheumatic which masquerade as such. fever. All were children between the ages of 6 and Out of such prolonged experience at the House of 12 years, apart from the patient initially thought to the Good Samaritan in Boston, Duckett Jones (1944) have sub-acute bacterial endocarditis, and only in was able to formulate a set of diagnostic criteria that patient and the child thought to be a case of which have proved extremely useful in a modified Still's disease was the ultimate diagnosis not made http://adc.bmj.com/ form (Rheumatic Fever Committee Joint Report, before transfer here. All patients have been regularly 1955) for ensuring in a large therapeutic trial that followed up since discharge, for periods between cases from many different centres were of the same 18 months and seven years. general type. He realized that cases of other diseases were as far as possible to be ruled out. It was realized Poliomyelitis. These two children complained of fever and limb pains and one had neck stiffness. Normal further that many cases of genuine rheumatic fever, cerebrospinal fluid was found and in both significant if first observed late in the course of the disease, or heart murmurs became audible subsequently. if mild, could not be fitted in and would have to be on October 1, 2021 by guest. Protected called 'probable rheumatic fever'. Furthermore, Pneumonia. This 9-year-old girl gave a history of some general malaise and limb pains. On admission to the of the criteria depend upon continuous clinical referring hospital, she was febrile and was found to have observation or laboratory tests usually possible in impaired percussion note and crepitations in the right hospital, but only comparatively rarely made in this mid-zone. Following subsidence of the chest signs she country in the average general practice. In this centre developed significant heart murmurs, and later rheumatic then, a discharge diagnosis of rheumatic fever has nodules. been made on unformulated but rather wider grounds Meningitis. This child had a sore throat followed by than those of the Duckett Jones criteria, so that of limb pain and headache. Normal cerebrospinal fluid was 2,214 patients admitted to the unit over the nine-year obtained and she subsequently developed polyarthritis period, 959 were discharged with the diagnosis of and heart block. rheumatic fever. Nearly two thirds of these satisfied Rubella. This 7-year-old girl had a sudden onset of diagnostic criteria comparable to those of Duckett abdominal pain and a rubella-like rash. There was, how- 350 Arch Dis Child: first published as 10.1136/adc.33.170.350 on 1 August 1958. Downloaded from DIFFERENTIAL DIAGNOSIS OF RHEUMATIC FEVER 351 ever, a normal white count and no accompanying TABLE 1 lymphadenopathy; she subsequently developed peri- FINAL DIAGNOSIS OF 196 CASES REFERRED AS RHEU- and endocarditis. MATIC FEVER IN WHOM THIS DIAGNOSIS WAS NOT carditis CONFIRMED Functional (8): Dysentery. This girl complained of abdominal pain, Physiological tachycardia 3 diarrhoea and limb pains. Stool culture was negative. Hysteria. 3 Neurosis and depression 2 She complained of limb pains after the diarrhoea settled Traumatic and Postural (11): and an organic mitral pansystolic murmur developed. Sprains 5 Slipped femoral epiphysis 2 Torn cartilage, osteochondritis dissecans, chest deformity and Subacute Bacterial Endocarditis. This young adult back ache-1 each 4 female with known rheumatic heart disease gave a five-day Infections (91): Upper respiratory tract 45 history of general malaise, generalized aches and pains Lower respiratory tract 10 and fever. She subsequently developed polyarthritis and Bone andl joint 10 Blood strlamr 4 a raised antistreptolysin-0 titre; repeated blood cultures Usinary -act 4 were negative and there were no embolic manifestations. Pericarditis 2 Specific fevers 16 She responded to bed rest and salicylates. Connective rissue Disorders (42): Still's and rheumatoid arthritis 24 Appendicitis. These two children both complained of Schonlein-Henoch purpura 8 Erythema nodosum 5 abdominal pain. Normal appendices were removed, but Visceral Itipus erythematosus 2 they both subsequently developed polyarthritis and Periarteritis nodosum. Penicillin sensitivity organic heart murmurs. Granuloma annulare Rheumatic and Congenital Heart Disease (9): Suppurative Arthritis of Hip. This 9-year-old boy had Congenital Rheumatic a four weeks' history of pain in one hip with inguinal Blood Dyscrasias (2): adenitis and fever. He subsequently developed poly- Iron deficiency anaemia Aleukaemic stem-celled leukaemia 3 arthritis and a mitral pansystolic murmur. Others (33): Arthritis. 5 Still's Disease. This boy presented with a three months' Arthralgia 9 Limb pains 1519 history of polyarthritis, fever, rash and general malaise. Fever It was not until after 10 weeks in this hospital that he Gout and syphilis 1 heart lesions, by which time the arthritis had developed Total copyright. settled leaving no residua. Fallen Arches. This 6-year-old boy had complained of I. Functional. This group comprised six children pain in his feet for four weeks and had been treated with and two young adults. The three patients with remedial exercises. He developed polyarthritis, and had a physiological tachycardia were all children. The first raised E.S.R. and A.S.O. titre. had a history of recent sore throat and the referring doctor thought he had detected clinical signs of Group B. Unsubstantiated Admission Diagnosis of cardiac enlargement with persistent tachycardia. Rheumatic Fever The second child had complained of limb pains for This group consisted of all patients admitted six weeks and was noted to have an evening tempera- http://adc.bmj.com/ between July, 1947, and October 31, 1956, with the ture up to 98 8°F. and persistent tachycardia of presumed diagnosis of rheumatic fever, but in whom 130-140 per minute. The third child had a recent this diagnosis was not substantiated. Only those history ofcold and cough with persistent tachycardia. were included in whom there was fairly certain Nothing abnormal could be found in any of these evidence from the referring letter or history on entry patients, and, although the pulse rate was elevated that they were thought to have rheumatic fever; they by day, the sleeping pulse was within the normal numbered 196. range (Nelson, 1954). on October 1, 2021 by guest. Protected These 196 admissions consisted of 171 children The two young female adults complained of limb under the age of 17 years, and 25 adults. Follow-up pains and general ill-health; one had had rheumatic has varied from none in a small minority to several fever as a child; she showed hysterical manifestations years in some instances. There has been no evident such as hyperventilation and no organic signs were rheumatic activity at these check-ups, and no patient found. The other patient also had nothing organic has developed organic heart disease under out- demonstrable, and was referred to a psychiatrist patient observation. None of the patients either on who diagnosed neurosis and depression. long or short term follow-up has been re-admitted The other three children all complained of pains with rheumatic fever. They may be divided into seven in joints or limbs for which no organic cause was broad groups which can be further subdivided as found. One had a functional wrist drop, the second shown in Table 1. The various groups will now be hysterical gait, and the third, with an unhappy home considered in more detail: background, admitted to exaggerating his symptoms Arch Dis Child: first published as 10.1136/adc.33.170.350 on 1 August 1958. Downloaded from 352 ARCHIVES OF DISEASE IN CHILDHOOD because of desire for sympathy. All were helped by III. Infections. (a) UPPER RESPIRATORY TRACT psychiatric treatment. INFECTIONS. Of this large group, 45 (43 children and two young adults) were diagnosed as upper respira- ll. Traumatic and Postural. All the patients in this tory tract infections. The presumptive diagnosis of group were children, and all complained of joint rheumatic fever was based on preceding upper pains; three patients had a history of rheumatic fever.