Rheumatic Fever in Minnesota 11. Evaluation of Hospitalized Patients and Utilization Of a State Rheumatic Fever Registry MARY JO RICE, MD,
Abstract: We studied the hospital records of 124 patients with a discharge diagnosis of acute rheumatic fever who were hospitalized in 21 Minneapolis-St. Paul hospitals during 1975 and 1976. After careful review of the hospital records, we found that 83 (67 per cent) of these patients did not have an acute illness. Seventeen (41 per cent) of the 41 cases with an acute illness were thought to adequately fulfill the Jones' Criteria for acute rheumatic fever. Upon review of the rheumatic fever registry of the Minnesota State Health Department, we found that less than one-half of the hospitalized patients had been reported to the registry. Cases that fulfilled and did not fulfill the Jones' Criteria A recent study' evaluating patients reported to the rheumatic fever registry at the Minnesota State Department of Health as having had acute rheumatic fever revealed that approximately 40 per cent of the cases did not fulfill the revised Jones' Criteria.2 Data from that study also suggested underreporting of acute rheumatic fever in Minnesota because there were few cases reported from some of the populous areas of the state where one might expect cases to occur.' The wide discrepancy between published estimates of the annual incidence of this disease in the United States imply that the true incidence is unknown. The Intersociety Commission for Heart Disease Resources report suggested that there are 100,000 cases annually in the United States3; yet a recent report from the National Center for Disease Control suggests many fewer cases.4 Because of this lack of reliable data, it becomes difficult, is not impossible, to determine appropriate control measures for this theoretically preventable cardiovascular disease. For example, it has been asserted that, at least in the United States and other industrialized countries, the incidence of acute rheumatic fever has decreased except among certain socially and economically disadvantaged populations. Without accurate epidemiologic data it is not possible to locate specific high-risk populations where control measures should be intensified. In addition, in some states there has recently been significant public presFrom the Department of Pediatrics, University of Minnesota Medical School. Address reprint requests to Edward L. Kaplan, MD, Dept. of Pediatrics, Box 94, Mayo Memorial Building, University of Minnesota Medical School, Minneapolis, MN 55455. This paper, submitted to the Journal November 13, 1978, was revised and accepted for publication March 21, 1979. Editor's Note: See also editorial, page 761, this issue. AJPH August 1979, Vol. 69, No. 8
EDWARD L. KAPLAN, MD
were reported with equal frequency, indicating significant underreporting and overreporting of rheumatic fever. Evaluation of secondary rheumatic fever prophylaxis, both in those patients with acute rheumatic fever as well as in those with rheumatic heart disease, indicated that many patients who, in theory, should be receiving prophylaxis were not receiving it. These studies indicate a need for more thorough evaluation of the current epidemiology of rheumatic fever and the role of a rheumatic fever registry, and imply a need for reevaluation of these programs. (Am J Public Health
sure to introduce streptococcal surveillance programs into schools as a part of school health programs. While these control measures attempt to reduce morbidity and absenteeism among school children (a theoretical benefit of such programs), the primary reason for school streptococcal control programs is the ultimate reduction of the incidence of rheumatic fever and prevention of rheumatic heart disease. Yet it is not possible to assess the impact of these programs on the incidence of acute rheumatic fever unless there are accurate epidemiologic data. The present study was undertaken to attempt to further define current underreporting of patients with acute rheumatic fever to a rheumatic fever registry. A second purpose was to determine if the registry is an accurate reflection of the characteristics of the disease in patients thought to have acute rheumatic fever in the mid 1970s.
Material and Methods Permission was sought from the medical records departments of 29 hospitals in the Minneapolis-St. Paul, Minnesota
metropolitan area to study the records of all patients discharged with a diagnosis of acute rheumatic fever during the years 1975 and 1976. The metropolitan area was chosen because it was felt that standards for medical care were at least as high there as elsewhere in the state as well as for the sake of convenience. Six of the hospitals (20 per cent) reported no patients with a discharge diagnosis of acute rheumatic fever during those two years; and two hospitals elected not to participate in the study, leaving the records from 21 hospitals for evaluation. The hospital record of each of these patients was reviewed by the authors independently and collectively 767
RICE AND KAPLAN TABLE 1-Jones' Criteria among 41 Hospitalized Patients According to Whether Criteria Were or Were Not Fuffilled
Major Criteria Arthritis Carditis Sydenham's Chorea Erythema Marginatum Subcutaneous Nodules Minor Criteria Previous Rheumatic Fever Arthralgia without arthritis Fever Positive Acute Phase Reactants (ESR and/or CRP) Prolonged PR Interval Evidence of a Preceding Streptococcal Infection Positive Throat Culture ASO (2 333 Todd Units) Anti DNase B ( . 240) Other (Scarlet Fever, other Antibody Evidence) Total Patients with Evidence of a Streptococcal Infection
Patients Fuffilling Jones' Criteria (Total N = 17)
Patients Not Fuffilling Jones' Criteria (Total N = 24)
Number of Patients 14 (82) 10 (59) 2 (12) 1 (6) 1 (6)
Number of Patients 11 (46) 1 (4) 000-
6 (35) 2 (12) 9 (53) 15 (88)
5 (21) 8 (33) 9 (38) 19 (79)
6 (35) 14 (82) 9 (53) 2 (12)
3 (13) 9 (38) 1 (4) 5 (21)
Percentages in parentheses.
with particular attention given to criteria for diagnosis. Each chart was carefully reviewed to determine whether the revised Jones' Criteria2 for making a diagnosis of acute rheumatic fever were fulfilled. If a diagnosis of rheumatic fever could not be confirmed from chart review, an attempt was made to establish the most likely diagnosis. Following review of all hospital records, the files of the Minnesota State Health Department rheumatic fever registry were searched to determine which patients had been enrolled.* The registry review was performed during the spring of 1978, allowing over 15 months for registration even of those patients hospitalized during the latter months of 1976. The method for administering secondary rheumatic fever prophylaxis was recorded during both the review of the hospital chart and of the state health department records.
Results The records of 124 patients hospitalized during the years 1975 and 1976 with a discharge diagnosis of acute rheumatic fever were evaluated. It was found that 83 (67 per cent) were patients who did not have an acute "inflammatory" illness or Sydenham's chorea at the time of hospitalization, but may have had an acute illness in the past (including rheumatoid arthritis, a history of rheumatic fever in the distant past, migratory arthritis, or pericarditis). The remaining 41 patients *The Minnesota Department of Health has had a rheumatic fever registry for over 15 years, registering over 13,000 patients since its creation. Rheumatic fever is a reportable disease in this state.5 768
(33 per cent) were all hospitalized with an acute "inflammatory" illness during the years 1975 and 1976. Of these 41 patients, only 17 (41 per cent) had sufficient documented evidence to fulfill the revised Jones' Criteria. The mean age for those patients fulfilling the revised Jones' Criteria was 14 (range, 5-28 years); for the patients not thought to fulfill the revised Jones' Criteria, the mean age was 23 (range, 7-55 years). None of the 41 patients was older than 55. The findings of these 41 patients are shown in Table 1: arthritis was the most common presenting manifestation, and all patients with arthritis had polyarthritis with involvement of at least two joints. None of the 10 patients with strong evidence for carditis were thought to have had congestive heart failure. As to minor criteria, the most common manifestation was a positive test for acute phase reactants (88 per cent); fever was the next most common manifestation (59 per cent). All 17 patients had evidence of a preceding streptococcal infection. In the group of 24 patients whose records did not reveal evidence sufficient to fulfill the revised Jones' Criteria, 11 had migratory polyarthritis and one had carditis, but there were no patients with subcutaneous nodules or erythema marginatum. As to minor criteria, five patients (21 per cent) had a previous history of acute rheumatic fever, and 19 (79 per cent) had a positive test for acute phase reactants. Fifteen of these patients (62.5 per cent) had evidence of a preceding streptococcal infection. In Figure 1, the month of onset of symptoms for all 41 patients with an acute illness is shown with those fulfilling the revised Jones' Criteria in the clear area. With the exception of the summer months, there was essentially no AJPH August 1979, Vol. 69, No. 8
RHEUMATIC FEVER REGISTRY IN MINNESOTA
Jones' Crileria not fulf/lled
TABLE 2-Antibiotic Prophylaxis Prescribed at Hospital Discharge According to Whether or Not Jones' Criteria Were Fulfilled
JAN FEB MAR APR MAY JUN JUL AUG SEP OCT NOV DEC Month of Onset
Benzathine Penicillin G, Intramuscular Oral Penicillin Erythromycin Oral Sulfadiazine TOTAL Number Receiving Prophylaxis
Patients Fuffilling Jones' Criteria (Total N = 17)
Patients Not Fulfilling Jones' Criteria (Total N = 24)
7 5 0 0
7 4 1 0
*Each indicated month includes total cases for both /975 and 1976.
FIGURE 1-Month of Admission (1975-76) to Hospital of 41 Patients with an Acute Illness Who Were Discharged with a Diagnosis of Acute Rheumatic Fever
demonstrable seasonal difference in the season of onset of the bona fide cases; more patients who did not appear to meet the revised Jones' Criteria were admitted to the hospital in October and November and in the early spring. In our review of the rheumatic fever registry at the Minnesota State Health Department we found that only 16 (40 per cent) of these 41 patients were included in the registry; eight of 17 cases (49 per cent) were from the group which fulfilled the Jones' Criteria and eight were from the 24 patients who did not meet the Jones' Criteria. It is of interest that two of the eight cases which fulfilled the revised Jones' Criteria and one case which did not fulfill the revised Jones' Criteria were in the registry only because of a "previous" episode. The registry had not been notified of these 1975-76
episodes. The criteria reported to the registry for those patients fulfilling the revised Jones' Criteria were generally also found in the hospital records, but in several cases there was important diagnostic and clinical information in the hospital records which had not been reported to the registry. For example, there was one case for which no documentation of a recent streptococcal infection was reported to the registry, yet the evidence was available in the hospital records. It should be added that there was even poorer correlation between hospital records and registry records for those patients not fulfilling the revised Jones' Criteria. Of the 83 hospitalized patients who had no evidence of a recent acute inflammatory illness, we found that 60 (72 per cent) had rheumatic heart disease. The mean age of this group was 60 (range, 26-91 years). Other diagnoses included rheumatoid arthritis, non-specific polyarthritis (in the past) and pericarditis (in the past). In this group, all patients with rheumatic heart disease and/or a history of rheumatic fever had significant valvular heart disease and/or prosthetic valves. According to their hospital records, only eight (12.5 per cent) of the 64 patients with rheumatic heart disease and/ or a past history of rheumatic fever were receiving "'secondAJPH August 1979, Vol. 69, No. 8
ary" rheumatic fever prophylaxis**; all eight were receiving oral penicillin. Only five of these 64 patients (8 per cent) were listed in the rheumatic fever registry (two patients with a history of rheumatic fever and three with rheumatic heart disease). While all five had been originally placed on oral penicillin, only one was receiving oral penicillin at the time the registry records were reviewed. The hospital records of the 41 patients who had had an acute illness were also reviewed to see whether antibiotics were prescribed to prevent recurrences of rheumatic fever (Table 2). Only 24 (59 per cent) were discharged from the hospital on "secondary" rheumatic fever prophylaxis (70 per cent of those whose hospital records fulfilled the revised Jones' Criteria and one-half of those who did not). Review of the State Health Department records revealed that all 16 patients reported to the registry (of the 41 with an acute illness) had been placed on some form of prophylaxis. Of the eight patients who fulfilled the Jones' Criteria, three were started on benzathine penicillin G, five on oral penicillin, and one on sulfadiazine. Of these eight patients, only one patient (started on benzathine penicillin G) was still receiving prophylaxis at the time the registry records were reviewed.
Discussion This study of hospitalized patients confirms and extends the observation from our previous study which dealt only with patients reported to the State of Minnesota Rheumatic Fever Registry as having rheumatic fever. ' Our data are also similar to those from a previous study from Maryland.7 The mean age of the group of 41 patients with an acute illness (19 years) was younger than that of the group with chronic illness (60 years), who, nonetheless, had a hospital discharge diagnosis of acute rheumatic fever. It is of interest that the mean age of those 17 patients who fulfilled the Jones' Criteria (14 years) was less than the mean age of those with an acute illness who failed to satisfy the Jones' Criteria (23 **(As defined by the American Heart Association, "'secondary" prophylaxis refers to continuous administration of appropriate antibiotics to prevent recurrences of rheumatic fever.6) 769
RICE AND KAPLAN
years). The ages of the patients in this report are different from those reported in our previous study,' in which we evaluated only patients 20 years of age or less who had been reported to the Minnesota registry within four months of the onset of the disease in an attempt to eliminate questionable cases from analysis. While the trends of the two reports can be compared, the exact percentages cannot be compared, since by including older patients in this present report the number of patients likely to have had acute rheumatic fever in 1975-76 has been decreased. The list of 124 patients with "acute rheumatic fever" was obtained by requesting from 21 Twin Cities hospitals those cases with a discharge diagnosis of acute rheumatic fever hospitalized during the years 1975 and 1976. Following review of the charts it was determined that 67 per cent had a chronic disease (some with an acute illness compatible with rheumatic fever in the distant past) but only 33 per cent had an "acute" illness when hospitalized. According to the hospital records, only 41 per cent of those with an acute illness in 1975 and 1976 fulfilled the Jones' Criteria. Thus, in only 15 per cent of all the 124 cases reviewed could the discharge diagnosis of acute rheumatic fever be confirmed on the basis of information present in their hospital records. Therefore, determining the incidence of acute rheumatic fever by requesting information from hospital medical records departments may be inaccurate. It could not be determined with certainty whether this is due to the lack of accurate recording in the chart or actual absence of the findings. It is especially noteworthy that only eight of the 17 patients who fulfilled the revised Jones' Criteria according to hospital records were ever reported to the rheumatic fever registry at the Minnesota State Department of Health. Since two of these cases were only included in the registry because of a previous episode of rheumatic fever (with no report of the episode occurring during 1975 or 1976), only about one third of the cases of documented acute rheumatic fever hospitalized in the 21 hospitals during our study period were reported to the registry. Furthermore, one-third of the cases with an acute illness not fulfilling the revised Jones' Criteria were reported to the registry. These data demonstrate both overreporting and underreporting of rheumatic fever, confirming our previous observations. ' We also found that information present in the hospital records was not always reported to the registry. These observations lead one to question the validity of the registry's records in assessing the incidence and the manifestations of acute rheumatic fever. The incidence of individual major and minor criteria is not significantly different from that found in previous studies.8 9 Three patients did not fulfill the revised Jones' Criteria because they lacked evidence of a preceding streptococcal infection. However, even if these patients are considered to have fulfilled the Jones' Criteria, less than 50 per cent of the 41 patients with an acute illness had bona fide acute rheumatic fever. It should be noted that although the ASO test appears more sensitive than the anti-DNase B test, antiDNase B tests were not performed on all sera. The regimen of choice for "secondary" rheumatic fever prophylaxis, according to the American Heart Association,6 is intramuscular benzathine penicillin G, 1,200,000 units 770
once a month unless the patient is allergic to penicillin. Of those cases of acute rheumatic fever fulfilling the revised Jones' Criteria, only 71 per cent were discharged from the hospital on "secondary" rheumatic fever prophylaxis. Most disturbing is that none of these patients were reported to be receiving any form of rheumatic fever prophylaxis at the time of this study, and only four of the five discharged on oral penicillin prophylaxis were still reported to be taking the medication less than two years later. Whether this means that those patients are no longer receiving prophylaxis or that they are obtaining their antibiotics from another source could not be verified. However, in either case, one is left with the impression that the accuracy of the registry and its usefulness as a means of evaluating rheumatic fever in Minnesota are questionable. According to hospital records, only 12 per cent of the 83 patients with rheumatic heart disease or a history of rheumatic fever were on any prophylaxis in 1975 or 1976 although all should have been on secondary rheumatic fever prophylaxis according to the recommendations of the American Heart Association.6 Our data suggest that little is actually known about the current epidemiology of acute rheumatic fever, at least in one geographic area, the State of Minnesota. If it can be assumed that standards for medical care and standards for reporting reportable diseases are not appreciably different in other parts of the United States, then our data may reflect a broader problem and suggest that a rheumatic fever registry is not an accurate method for assessing the incidence of this
disease. That both the incidence and severity of acute rheumatic fever have decreased during the past several decades is probably true; however, the slope of the descent curve may not actually be as sharp as some have described because of errors, in both diagnosis and in reporting.10 Our experience also indicates the necessity for re-evaluating current rheumatic fever control programs in Minnesota, and perhaps in other areas of the United States and other countries as well. This is especially true if those with public health responsibilities are to be able to make valid judgements about streptococcal and rheumatic fever control programs. This need was evident in a recent report from the Center for Disease Control, demonstrating the wide variability of control programs for streptococcal infections and their sequelae in the United States."I REFERENCES 1. Dahl D, Bessinger FB and Kaplan EL: Rheumatic fever in Minnesota, current assessment of reported cases. Minn Med 61:249-254, 1978. 2. Committee to Revise the Jones' Criteria: Jones' criteria (revised) for guidance in the diagnosis of rheumatic fever. Circula-
tion 32:664-668, 1965. 3. Intersociety Commission for Heart Disease Resources: Prevention of rheumatic fever and rheumatic heart disease. Circulation 41:Al, 1970. 4. Reported Morbidity and Mortality in the United States, 1976. Morbidity and Mortality Weekly Report, Annual Summary, 1976, CDC, 25:2-10, 1977. 5. Minnesota State Department of Health, Regulation 316, revised June 1975. AJPH August 1979, Vol. 69, No. 8
RHEUMATIC FEVER REGISTRY IN MINNESOTA 6. Committee on Prevention of Rheumatic Fever and Bacterial Endocarditis of the American Heart Association. Prevention of rheumatic fever. Circulation 55:1-4, 1977. 7. Gordis L, Lilienfeld A and Rodriguez R: An evaluation of the Maryland rheumatic fever registry. Public Health Reports 84:333-339, 1969. 8. Perlman LV, Ellison J, Kleinman H and Fleming DS: Rheumatic fever, Minnesota secondary prevention program. Minn Med 47:1551-1557, 1964. 9. Finkelstein S: Minnesota rheumatic fever secondary prevention program, 1960-1969. Minnesota Department of Health, Minneapolis, MN, 1973. 10. Kaplan EL: Acute rheumatic fever. Pediatr Clin North Am 25:817-829, 1978.
11. McCormick JB and Fraser DW: Disease control programs in the United States-control of streptococcal and poststreptococcal disease. JAMA 239:2359-2361, 1978.
ACKNOWLEDGMENTS We would like to thank the medical records librarians at the participating hospitals for their cooperation and assistance. We also appreciate the help provided by the Minnesota State Health Department in allowing us to review the registry records. This study was supported in part by the James F. Zagaria Memorial Fellowship in Cardiology through the Minnesota Medical Foundation (Dr. Rice); and in part by the Dwan Family Fund.
4th National Conference on Child Abuse and Neglect The Fourth National Conference on Child Abuse and Neglect, to be held October 7-10, 1979, at the Los Angeles Hilton Hotel, will provide a national forum to address policy, economic, political and social concerns impacting on the quality of life for children and families. The Conference is hosted by Region IX Child Abuse and Neglect Resource Center in cooperation with the U.S. Department of Health, Education, and Welfare. The newest concepts, research, techniques and models pertinent to the field of child abuse and neglect and the welfare of children and families will be presented. Special emphasis will be given to workshops designed to provide both practical and theoretical information relevant to those working in the area of child abuse and neglect and related fields. Continuing Education credit is being arranged. Intensive training sessions will include units for educators, law enforcement personnel, physicians, nurses, and social workers. Advance registration by September 1 is $60; on-site registration is $75; students with valid identification may register for $25; daily registration fee is $35. The Hilton Hotel is offering special conference room rates, effective October 4-13: Singles: $34; Double/Twins: $42; Triples: $50. Hotel registration requests may be made through your local Hilton Hotel Reservation Service by September 29. For further information, contact Shelley Brazier, Assistant Director, Region IX Child Abuse and Neglect Resource Center, California State University, 5151 State University Drive, Los Angeles, CA 90032, (213) 224-3283.
AJPH August 1979, Vol. 69, No. 8