A Clinical Scoring System for Chronic Inflammatory Bowel Disease in Children JOHN D. LLOYD-STILL, MD, and ORVILLE C. GREEN, MD
A clinical scoring system for the assessment o f children with chronic inflammatory bowel disease has been devised. A close correlation is demonstrated between severity of disease and the level o f serum albumin. The clinical score is simple to perform, sensitive to changes in clinical status, reproducible by different observers, and specifically designed to evaluate inflammatory bowel disease in children and adolescents. The clinical score is a useful adjunct in the management o f children with chronic inflammatory bowel disease and can be used in prospective studies o f various therapeutic modalities.
Chronic inflammatory bowel disease (Crohn's disease and ulcerative colitis) may involve different areas of small or large intestine and have numerous extraintestinal complications (1). Malabsorption and inadequate caloric intake frequently lead to malnutrition and growth failure in the pediatric population (2). Despite all these manifestations, no consistent clinical or biochemical finding always correlates with the severity of the disease. Truelove and Witts (3) first devised a classification for severity of disease in their controlled trial on the effects of cortisone in ulcerative colitis. Recently the National Cooperative Crohn's Disease Study Group has developed a Crohn's disease activity index (4) that utilizes eight selected variables in assessing the activity of the disease. Whittington et al (5) utilized the physician's subjective impression of patient well-being in a study of the medical management of Crohn's disease in adolescents. The physician's subjective impression of patient wellbeing has correlated well with Crohn's disease activity index (4). In order to perform cooperative studies on the natural history or therapy of chronic inflammatory From the Department of Pediatrics, Northwestern University Medical School, Children's Memorial Hospital, Chicago, Illinois. Address for reprint requests: Dr. John D. Lloyd-Still, Division of Gastroenterology, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, Illinois 60614.
620
bowel disease, it is essential to have some modality that attempts to quantify the clinical manifestations of chronic inflammatory bowel disease in childhood into a readily applicable system. Unfortunately, none of the presently available methods of assessment satisfactorily encompasses the wide variety of clinical manifestations of chronic inflammatory bowel disease in the pediatric population. The Shwachman clinical score (6) for grading the severity of disease is of proven value in the management of patients with cystic fibrosis. We have devised a method of assessment of the child with chronic inflammatory bowel disease by a clinical scoring system modified from the Shwachman score for cystic fibrosis. All patients with ulcerative colitis, regional enteritis, and granulomatous disease of the colon can be included in this scoring system which also has specific sections related to the nutritional and growth parameters in the pediatric population. MATERIALS AND METHODS
The scoring system is divided into five major divisions with a maximum score in parentheses--general activity (10), physical examination and clinical complications (30), nutrition (20), x-rays (15), and laboratory (25). The various subdivisions of each score are shown in Table 1. All of the evaluations included in the score are easily elicited by any physician, and all the investigations, radiological and/or laboratory parameters are standard procedures in the care of these patients.
Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979) 0163-2116/79/0800-0620503.00/1 9 1979DigestiveDisease Systems, Inc.
SCORING JUVENILE
BOWEL DISEASE
Serum albumin was measured by the autoanalyzer (normal > 3.5 g/dl), and the erythrocyte sedimentation rate (ESR) determined by the Westergen method (normal < 15 mm/hr). TABLE 1. CLINICAL SCORE IN CHRONIC INFLAMMATORYBOWEL DISEASE. General activity (10) 10 Normal school attendance BM < 3 p e r d a y Lacks endurance BM 3-5 per day Misses < 4 weeks school/year Fever, home tutor BM > 5 per day Severely restricted activity Physical examination and complications (30) Abdomen 10 Normal 5 Mass 1 Distention, tenderness Proctoscopy/Perianal
l0 5 1
Normal, no fissures Friability, 1 fissure Ulcers, pseudopolyps, bleeding, multiple fissures, fistulas
Arthritis
5 3 1
Nil One joint/arthralgia Multiple joints
Skin/stomatitis/eyes
5 3 1
Normal Mild stomatitis Erythema nodosum, pyoderma, severe stomatitis, uveitis
Nutrition (20) Height 10 5
Weight
1
> 2"/year < optimal % No growth
10 5 1
Normal No gain Weight loss
RESULTS In Figure 1 the ages of 54 children with chronic inflammatory bowel disease are plotted against clinical score. There were 25 females and 29 males. All patients were seen during the last 3 years. The grading of the clinical score was done on initial presentation. The figure further differentiates those patients with Crohn's disease (21 patients) compared to those with ulcerative colitis (33 patients). The figure also demonstrates the wide variation in the severity of the disease. All the severely ill children (scores below 30) were treated with corticosteroids, whereas only about 50% of the moderately affected children (scores in the 50s and 60s) were given these drugs. An attempt is made to use corticosteroids for short periods (usually six weeks) in our clinic, and 12 of the 27 children treated with corticosteroids are on long-term therapy. All children are treated with long-term sulfasalazine, provided this is tolerated. Seven patients with scores below 40 required prolonged therapy with constant-infusion intragastric elemental diet or intravenous alimentation to correct nutritional failure. In order to assess the ability of the clinical score to demonstrate changes in an individual patient's clinical status, we have plotted the clinical scores of 5 patients with Crohn's disease (Figure 2), and 5 with ulcerative colitis (Figure 3) over a 4-year period. Both figures illustrate the highly complicated ways in which these diseases progress. Some patients with severe disease (scores in the 30s and 40s) recover into a good status (scores in the 80s), others
100
X-rays (15) 15 Normal 10 Ileitis, colitis to splenic flexure 5 Total colon or ileocolic involvement 1 Toxic megacolon, obstruction
o
1
ESR
5 3
WBC
5 3 1 10 5
1
Albumin
1
> 40 25-35 < 25 Normal 20-40 > 40 Normal < 20,000 > 20,000 Normal 3.0 g/dl < 2.5 g/dl
Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979)
o
o
o o
LJ ~o 40
o
~
9
o
o
o o
oe
o 9
o
o
oo
o
o
6O Laboratory (25) Hct. 5 3
o
oo
80
9
O%o.'/-o
.
9
9
o o
oO
~o
9
9
20 ULCERATIVE COLITIS o C R O H N S DISEASE 9 2
4 AGE
6
8
10
12
14
16
IN YEARS
Fig 1. Clinical score correlations with ages of 54 children with chronic inflammatory bowel disease. The patients are subdivided into those with ulcerative colitis and Crohn's disease.
621
LLOYD-STILL AND GREEN TABLE 2. CORRELATIONBETWEEN CLINICAL SCORE AND SURGICAL PROCEDURESIN 8 PATIENTS WITH CHRONIC INFLAMMATORYBOWEL DISEASE
Proctocolectomy and ileostomy Ileoproctostomy Ileohemicolectomy
No.
Clinical scores
3 1 4
27, 30, 33 49 42, 49, 51,53
have intermittent severe relapses followed by a good recovery, while others maintain a prolonged severe status. Figure 3 includes the severe course of two patients with ulcerative colitis who ultimately underwent total colectomy and ileostomy for failure to respond to medical therapy over a period of 2-4 years. Surgery was performed in 8 of the 54 children (Table 2). Proctocolectomy and ileostomy were performed on 3 patients, all with clinical scores below 35. Ileoproctostomy was performed on one patient. Four patients with Crohn's disease required various forms of resections either due to obstructive symptoms (2 patients) or the development of fistulae between bowel and bladder (2 patients). None of these patients were as severely ill as those requiring total proctocolectomy. This is reflected in the scores which were in the 40s and 50s. In our clinical experience the level of serum albumin is one of the best correlations with severity of disease. In Figure 4 the clinical score is plotted against serum albumin and shows an excellent correlation (r = 0.72). In order to test the sensitivity of the scoring system, we have plotted the scores of 2 patients who developed severe complications from their disease. In Figure 5 the patient developed 2 episodes of toxic megacolon that responded to therapy with corticosteroids, transfusions of blood and plasma, and elemental diet. On both occasions his clinical score dropped below 30, but in the intervening period his score was in the 90s and he remained symptom free. In Figure 6 the patient with ulcerative colitis developed recurrent relapses associated with arthritis and erythema nodosum and required three separate courses of corticosteroids. Note how on each occasion her score would drop from the 70s or 80s to the 50s. She is now doing well on continuous maintenance therapy with corticosteroids. The reproducibility of the system was compared with two observers who were familiar with the same patient. I n d e p e n d e n t a s s e s s m e n t of the clinical
622
score in 20 patients showed a correlation coefficient of 0.898. In Table 3 the clinical score in 21 patients (11 with ulcerative colitis and 10 with Crohn's disease) is compared to the Truelove et al (3) classification for ulcerative colitis, the Crohn's disease activity index (4), and the Whittington et al (5) physician's disease activity rating. There is good correlation among all scoring systems. DISCUSSION Schacter and Kirsner (7) have attempted a definition of inflammatory bowel diseases in adults. Their principal objective was to encourage the establishment of an acceptable starting point for prospective cooperative clinical studies. The scoring system we have devised is simple to perform, consistent in reproducibility by different observers, and sensitive to changes in the clinical status of the patients. We have confirmed a correlation between severity of disease as defined by the clinical score, and a decrease in the concentration of the serum albumin. The score is specifically designed for use in children and adolescents, and it is sufficiently
TABLE 3. COMPARISONOF CLINICAL SCORES OF 21 PATIENTS (NUMBERS 1-11 ULCERATIVECOLITIS, 12-21 CROHN'S DISEASE) WITH OTHER CLASSIFICATIONSOF SEVERITY OF DISEASE* Patient
Clinical score
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
27 28 30 32 33 34 40 47 58 67 72 34 36 36 39 46 47 47 56 56 65
Truelove et al (3)
Best et al (4)t
Severe Severe Severe Severe Severe Severe Severe Moderate Moderate Moderate Moderate 290/v. poor 291/v. poor 281/v. poor 231/poor 210/poor 198/poor 200/poor 158/fair to good 168/fair to good 13 i/fair to good
Whittington et al (5)
VI VI VI VI VI VI VI V V IV IV VI VI VI V IV V V IV IV IV
*IV = moderate symptoms occasionally interfering with normal activity; V = severe, requiring symptomatic or antidiarrheal medications; VI = incapacitating symptoms. t C r o h n ' s disease activity index. Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979)
SCORING JUVENILE BOWEL DISEASE 100
100 LL/
075
U
~...6.,..~....;::'.~...........
\
L.~.e::i...~...........
"- i ~.
\
u0 75
..-"
.................,,~,/
so .
Z ...% ~ 25
" b .......
O Z d 25
....o
1
2
3
I
2 YEARS
)'EARS Fig 2. T h e clinical scores of 5 patients with C r o h n ' s disease are plotted o v e r a 3-year period. Note the wide variation in the clinical course.
5.0
9
100
o~ 4.0 Z
i
6O
o ~40 3.0
~,,, 2.0
20
..
/
PREDNISONE
20
40
60
80
100
SCORE Fig 4. S e r u m albumin plotted against clinical score. Note the correlation (r = 0.72).
100
80 601
o ~40 20 PREDNISONE ~
2
4
~
4
Fig 3. The clinical scores of 5 patients with ulcerative colitis are plotted to s h o w their progress over follow-up for periods up to 4 years. The two arrows denote surgery (total colectomy and ileostomy) after failure to respond to medical therapy. Note h o w t h e clinical score reflects their severe prolonged course with frequent relapses.
80
u')
3
mm-zz:
6
8 10 12 14 16 i8 MONTHS Fig 6. Patient S K with ulcerative colitis developed 3 relapses with arthritis and e r y t h e m a n o d o s u m . She is n o w on c o n t i n u o u s therapy with corticosteroids. Note h o w the clinical score is sensitive to c h a n g e s in the clinical manifestations of the disease. Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979)
2
4
6
8
10
mm~
12 14 16
MONTHS Fig 5. Patient R F with ulcerative colitis developed 2 episodes bordering toxic megacolon that r e s p o n d e d to medical therapy, On both occasions he made an excellent recovery and was s y m p tom-free in the intervening periods.
flexible to be adapted for use in all forms of inflammatory bowel disease. Thus, for example, the development of a psychosis might severely restrict activity and would be given a low score. Assessment of the clinical score is complementary to other approaches to inflammatory bowel disease in childhood (8, 9). A clinical scoring system loses its value if it becomes too complicated, and an all-inclusive list of the possible complications of inflammatory bowel disease detracts from the simplicity of the present system. Comparisons of the clinical severity of disease by different classifications (Table 3) demonstrate that neither the Truelove et al (3) classification for ulcerative colitis nor the Crohn's disease activity index (4) can be used comprehensively for all forms of
623
LLOYD-STILL
chronic inflammatory bowel disease. The Whittington et al (5) physician's disease activity rating can be used for both conditions, although it was originally described in the management of Crohn's disease. Unfortunately, use of the Crohn's disease activity index in our pediatric population tends to result in a lower score than in the adult population for whom it was devised. One of the factors involved (variable 5) involves the administration of Lomotil or opiates for diarrhea, and adds 30 points to the score. Since none of our pediatric patients are given anticholinergics or opiates, this limits their potential disease activity index score. Other deficiencies in the Crohn's disease activity index, when this is applied to the pediatric population, include the absence of a score for height which is one of the major problems in the adolescent age group (2), and no accounting is made for the level of serum albumin which is simple to perform and correlates highly with disease activity in our patients (Figure 4). Our clinical scoring system is at present being used to monitor the effects of different therapeutic modalities in prospective studies of chronic inflammatory bowel disease. These results can then be directly correlated with an index of disease activity (the clinical score). Lastly, we believe that use of the clinical score can result in improved patient care by alerting physicians to the more important areas
624
AND GREEN
where complications may occur and therapeutic intervention is possible. ACKNOWLEDGMENTS Special thanks are due to Hans Wessel, MD, for statistical analysis. REFERENCES 1. Ament ME: Inflammatory disease of the colon: Ulcerative colitis and Crohn's colitis. J Pediatr 86:322, 1975 2. Kirschner BS, Voinchet O, Rosenberg IH: Growth retardation in inflammatory bowel disease. Gastroenterology 75:504, 1978 3. Truelove SC, Witts LJ: Cortisone in ulcerative colitis. Br Med J 2:4947, 1955 4. Best WR, Becktel JM, Singleton JW, Kern F: Development of a C r o h n ' s disease activity index. G a s t r o e n t e r o l o g y 70:439, 1976 5. Whittington PF, Barnes HV, Bayless TM: Medical management of Crohn's disease in adolescence. Gastroenterology 72:1338, 1977 6. Shwachman H, Kulczycki LL: Long-term study of 105 patients with cystic fibrosis. Am J Dis Child 96:6, 1958 7. Schachter H, Kirsner JB: Definitions of inflammatory bowel disease of unknown etiology. Gastroenterology 68:591, 1975 8. Grand RJ: Homer DR: Approaches to inflammatory bowel disease in childhood and adolescence. Ped Clin North Am 22:835, 1975 9. Gryboski JD, Spiro HM: Prognosis in children with Crohn's disease. Gastroenterology 74:807, 1978
Digestive Diseases and Sciences. Vol. 24, No. 8 (August 1979)
A clinical scoring system for the assessment o f children with chronic inflammatory bowel disease has been devised. A close correlation is demonstrated between severity of disease and the level o f serum albumin. The clinical score is simple to perform, sensitive to changes in clinical status, reproducible by different observers, and specifically designed to evaluate inflammatory bowel disease in children and adolescents. The clinical score is a useful adjunct in the management o f children with chronic inflammatory bowel disease and can be used in prospective studies o f various therapeutic modalities.
Chronic inflammatory bowel disease (Crohn's disease and ulcerative colitis) may involve different areas of small or large intestine and have numerous extraintestinal complications (1). Malabsorption and inadequate caloric intake frequently lead to malnutrition and growth failure in the pediatric population (2). Despite all these manifestations, no consistent clinical or biochemical finding always correlates with the severity of the disease. Truelove and Witts (3) first devised a classification for severity of disease in their controlled trial on the effects of cortisone in ulcerative colitis. Recently the National Cooperative Crohn's Disease Study Group has developed a Crohn's disease activity index (4) that utilizes eight selected variables in assessing the activity of the disease. Whittington et al (5) utilized the physician's subjective impression of patient well-being in a study of the medical management of Crohn's disease in adolescents. The physician's subjective impression of patient wellbeing has correlated well with Crohn's disease activity index (4). In order to perform cooperative studies on the natural history or therapy of chronic inflammatory From the Department of Pediatrics, Northwestern University Medical School, Children's Memorial Hospital, Chicago, Illinois. Address for reprint requests: Dr. John D. Lloyd-Still, Division of Gastroenterology, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, Illinois 60614.
620
bowel disease, it is essential to have some modality that attempts to quantify the clinical manifestations of chronic inflammatory bowel disease in childhood into a readily applicable system. Unfortunately, none of the presently available methods of assessment satisfactorily encompasses the wide variety of clinical manifestations of chronic inflammatory bowel disease in the pediatric population. The Shwachman clinical score (6) for grading the severity of disease is of proven value in the management of patients with cystic fibrosis. We have devised a method of assessment of the child with chronic inflammatory bowel disease by a clinical scoring system modified from the Shwachman score for cystic fibrosis. All patients with ulcerative colitis, regional enteritis, and granulomatous disease of the colon can be included in this scoring system which also has specific sections related to the nutritional and growth parameters in the pediatric population. MATERIALS AND METHODS
The scoring system is divided into five major divisions with a maximum score in parentheses--general activity (10), physical examination and clinical complications (30), nutrition (20), x-rays (15), and laboratory (25). The various subdivisions of each score are shown in Table 1. All of the evaluations included in the score are easily elicited by any physician, and all the investigations, radiological and/or laboratory parameters are standard procedures in the care of these patients.
Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979) 0163-2116/79/0800-0620503.00/1 9 1979DigestiveDisease Systems, Inc.
SCORING JUVENILE
BOWEL DISEASE
Serum albumin was measured by the autoanalyzer (normal > 3.5 g/dl), and the erythrocyte sedimentation rate (ESR) determined by the Westergen method (normal < 15 mm/hr). TABLE 1. CLINICAL SCORE IN CHRONIC INFLAMMATORYBOWEL DISEASE. General activity (10) 10 Normal school attendance BM < 3 p e r d a y Lacks endurance BM 3-5 per day Misses < 4 weeks school/year Fever, home tutor BM > 5 per day Severely restricted activity Physical examination and complications (30) Abdomen 10 Normal 5 Mass 1 Distention, tenderness Proctoscopy/Perianal
l0 5 1
Normal, no fissures Friability, 1 fissure Ulcers, pseudopolyps, bleeding, multiple fissures, fistulas
Arthritis
5 3 1
Nil One joint/arthralgia Multiple joints
Skin/stomatitis/eyes
5 3 1
Normal Mild stomatitis Erythema nodosum, pyoderma, severe stomatitis, uveitis
Nutrition (20) Height 10 5
Weight
1
> 2"/year < optimal % No growth
10 5 1
Normal No gain Weight loss
RESULTS In Figure 1 the ages of 54 children with chronic inflammatory bowel disease are plotted against clinical score. There were 25 females and 29 males. All patients were seen during the last 3 years. The grading of the clinical score was done on initial presentation. The figure further differentiates those patients with Crohn's disease (21 patients) compared to those with ulcerative colitis (33 patients). The figure also demonstrates the wide variation in the severity of the disease. All the severely ill children (scores below 30) were treated with corticosteroids, whereas only about 50% of the moderately affected children (scores in the 50s and 60s) were given these drugs. An attempt is made to use corticosteroids for short periods (usually six weeks) in our clinic, and 12 of the 27 children treated with corticosteroids are on long-term therapy. All children are treated with long-term sulfasalazine, provided this is tolerated. Seven patients with scores below 40 required prolonged therapy with constant-infusion intragastric elemental diet or intravenous alimentation to correct nutritional failure. In order to assess the ability of the clinical score to demonstrate changes in an individual patient's clinical status, we have plotted the clinical scores of 5 patients with Crohn's disease (Figure 2), and 5 with ulcerative colitis (Figure 3) over a 4-year period. Both figures illustrate the highly complicated ways in which these diseases progress. Some patients with severe disease (scores in the 30s and 40s) recover into a good status (scores in the 80s), others
100
X-rays (15) 15 Normal 10 Ileitis, colitis to splenic flexure 5 Total colon or ileocolic involvement 1 Toxic megacolon, obstruction
o
1
ESR
5 3
WBC
5 3 1 10 5
1
Albumin
1
> 40 25-35 < 25 Normal 20-40 > 40 Normal < 20,000 > 20,000 Normal 3.0 g/dl < 2.5 g/dl
Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979)
o
o
o o
LJ ~o 40
o
~
9
o
o
o o
oe
o 9
o
o
oo
o
o
6O Laboratory (25) Hct. 5 3
o
oo
80
9
O%o.'/-o
.
9
9
o o
oO
~o
9
9
20 ULCERATIVE COLITIS o C R O H N S DISEASE 9 2
4 AGE
6
8
10
12
14
16
IN YEARS
Fig 1. Clinical score correlations with ages of 54 children with chronic inflammatory bowel disease. The patients are subdivided into those with ulcerative colitis and Crohn's disease.
621
LLOYD-STILL AND GREEN TABLE 2. CORRELATIONBETWEEN CLINICAL SCORE AND SURGICAL PROCEDURESIN 8 PATIENTS WITH CHRONIC INFLAMMATORYBOWEL DISEASE
Proctocolectomy and ileostomy Ileoproctostomy Ileohemicolectomy
No.
Clinical scores
3 1 4
27, 30, 33 49 42, 49, 51,53
have intermittent severe relapses followed by a good recovery, while others maintain a prolonged severe status. Figure 3 includes the severe course of two patients with ulcerative colitis who ultimately underwent total colectomy and ileostomy for failure to respond to medical therapy over a period of 2-4 years. Surgery was performed in 8 of the 54 children (Table 2). Proctocolectomy and ileostomy were performed on 3 patients, all with clinical scores below 35. Ileoproctostomy was performed on one patient. Four patients with Crohn's disease required various forms of resections either due to obstructive symptoms (2 patients) or the development of fistulae between bowel and bladder (2 patients). None of these patients were as severely ill as those requiring total proctocolectomy. This is reflected in the scores which were in the 40s and 50s. In our clinical experience the level of serum albumin is one of the best correlations with severity of disease. In Figure 4 the clinical score is plotted against serum albumin and shows an excellent correlation (r = 0.72). In order to test the sensitivity of the scoring system, we have plotted the scores of 2 patients who developed severe complications from their disease. In Figure 5 the patient developed 2 episodes of toxic megacolon that responded to therapy with corticosteroids, transfusions of blood and plasma, and elemental diet. On both occasions his clinical score dropped below 30, but in the intervening period his score was in the 90s and he remained symptom free. In Figure 6 the patient with ulcerative colitis developed recurrent relapses associated with arthritis and erythema nodosum and required three separate courses of corticosteroids. Note how on each occasion her score would drop from the 70s or 80s to the 50s. She is now doing well on continuous maintenance therapy with corticosteroids. The reproducibility of the system was compared with two observers who were familiar with the same patient. I n d e p e n d e n t a s s e s s m e n t of the clinical
622
score in 20 patients showed a correlation coefficient of 0.898. In Table 3 the clinical score in 21 patients (11 with ulcerative colitis and 10 with Crohn's disease) is compared to the Truelove et al (3) classification for ulcerative colitis, the Crohn's disease activity index (4), and the Whittington et al (5) physician's disease activity rating. There is good correlation among all scoring systems. DISCUSSION Schacter and Kirsner (7) have attempted a definition of inflammatory bowel diseases in adults. Their principal objective was to encourage the establishment of an acceptable starting point for prospective cooperative clinical studies. The scoring system we have devised is simple to perform, consistent in reproducibility by different observers, and sensitive to changes in the clinical status of the patients. We have confirmed a correlation between severity of disease as defined by the clinical score, and a decrease in the concentration of the serum albumin. The score is specifically designed for use in children and adolescents, and it is sufficiently
TABLE 3. COMPARISONOF CLINICAL SCORES OF 21 PATIENTS (NUMBERS 1-11 ULCERATIVECOLITIS, 12-21 CROHN'S DISEASE) WITH OTHER CLASSIFICATIONSOF SEVERITY OF DISEASE* Patient
Clinical score
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
27 28 30 32 33 34 40 47 58 67 72 34 36 36 39 46 47 47 56 56 65
Truelove et al (3)
Best et al (4)t
Severe Severe Severe Severe Severe Severe Severe Moderate Moderate Moderate Moderate 290/v. poor 291/v. poor 281/v. poor 231/poor 210/poor 198/poor 200/poor 158/fair to good 168/fair to good 13 i/fair to good
Whittington et al (5)
VI VI VI VI VI VI VI V V IV IV VI VI VI V IV V V IV IV IV
*IV = moderate symptoms occasionally interfering with normal activity; V = severe, requiring symptomatic or antidiarrheal medications; VI = incapacitating symptoms. t C r o h n ' s disease activity index. Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979)
SCORING JUVENILE BOWEL DISEASE 100
100 LL/
075
U
~...6.,..~....;::'.~...........
\
L.~.e::i...~...........
"- i ~.
\
u0 75
..-"
.................,,~,/
so .
Z ...% ~ 25
" b .......
O Z d 25
....o
1
2
3
I
2 YEARS
)'EARS Fig 2. T h e clinical scores of 5 patients with C r o h n ' s disease are plotted o v e r a 3-year period. Note the wide variation in the clinical course.
5.0
9
100
o~ 4.0 Z
i
6O
o ~40 3.0
~,,, 2.0
20
..
/
PREDNISONE
20
40
60
80
100
SCORE Fig 4. S e r u m albumin plotted against clinical score. Note the correlation (r = 0.72).
100
80 601
o ~40 20 PREDNISONE ~
2
4
~
4
Fig 3. The clinical scores of 5 patients with ulcerative colitis are plotted to s h o w their progress over follow-up for periods up to 4 years. The two arrows denote surgery (total colectomy and ileostomy) after failure to respond to medical therapy. Note h o w t h e clinical score reflects their severe prolonged course with frequent relapses.
80
u')
3
mm-zz:
6
8 10 12 14 16 i8 MONTHS Fig 6. Patient S K with ulcerative colitis developed 3 relapses with arthritis and e r y t h e m a n o d o s u m . She is n o w on c o n t i n u o u s therapy with corticosteroids. Note h o w the clinical score is sensitive to c h a n g e s in the clinical manifestations of the disease. Digestive Diseases and Sciences, Vol. 24, No. 8 (August 1979)
2
4
6
8
10
mm~
12 14 16
MONTHS Fig 5. Patient R F with ulcerative colitis developed 2 episodes bordering toxic megacolon that r e s p o n d e d to medical therapy, On both occasions he made an excellent recovery and was s y m p tom-free in the intervening periods.
flexible to be adapted for use in all forms of inflammatory bowel disease. Thus, for example, the development of a psychosis might severely restrict activity and would be given a low score. Assessment of the clinical score is complementary to other approaches to inflammatory bowel disease in childhood (8, 9). A clinical scoring system loses its value if it becomes too complicated, and an all-inclusive list of the possible complications of inflammatory bowel disease detracts from the simplicity of the present system. Comparisons of the clinical severity of disease by different classifications (Table 3) demonstrate that neither the Truelove et al (3) classification for ulcerative colitis nor the Crohn's disease activity index (4) can be used comprehensively for all forms of
623
LLOYD-STILL
chronic inflammatory bowel disease. The Whittington et al (5) physician's disease activity rating can be used for both conditions, although it was originally described in the management of Crohn's disease. Unfortunately, use of the Crohn's disease activity index in our pediatric population tends to result in a lower score than in the adult population for whom it was devised. One of the factors involved (variable 5) involves the administration of Lomotil or opiates for diarrhea, and adds 30 points to the score. Since none of our pediatric patients are given anticholinergics or opiates, this limits their potential disease activity index score. Other deficiencies in the Crohn's disease activity index, when this is applied to the pediatric population, include the absence of a score for height which is one of the major problems in the adolescent age group (2), and no accounting is made for the level of serum albumin which is simple to perform and correlates highly with disease activity in our patients (Figure 4). Our clinical scoring system is at present being used to monitor the effects of different therapeutic modalities in prospective studies of chronic inflammatory bowel disease. These results can then be directly correlated with an index of disease activity (the clinical score). Lastly, we believe that use of the clinical score can result in improved patient care by alerting physicians to the more important areas
624
AND GREEN
where complications may occur and therapeutic intervention is possible. ACKNOWLEDGMENTS Special thanks are due to Hans Wessel, MD, for statistical analysis. REFERENCES 1. Ament ME: Inflammatory disease of the colon: Ulcerative colitis and Crohn's colitis. J Pediatr 86:322, 1975 2. Kirschner BS, Voinchet O, Rosenberg IH: Growth retardation in inflammatory bowel disease. Gastroenterology 75:504, 1978 3. Truelove SC, Witts LJ: Cortisone in ulcerative colitis. Br Med J 2:4947, 1955 4. Best WR, Becktel JM, Singleton JW, Kern F: Development of a C r o h n ' s disease activity index. G a s t r o e n t e r o l o g y 70:439, 1976 5. Whittington PF, Barnes HV, Bayless TM: Medical management of Crohn's disease in adolescence. Gastroenterology 72:1338, 1977 6. Shwachman H, Kulczycki LL: Long-term study of 105 patients with cystic fibrosis. Am J Dis Child 96:6, 1958 7. Schachter H, Kirsner JB: Definitions of inflammatory bowel disease of unknown etiology. Gastroenterology 68:591, 1975 8. Grand RJ: Homer DR: Approaches to inflammatory bowel disease in childhood and adolescence. Ped Clin North Am 22:835, 1975 9. Gryboski JD, Spiro HM: Prognosis in children with Crohn's disease. Gastroenterology 74:807, 1978
Digestive Diseases and Sciences. Vol. 24, No. 8 (August 1979)
