European Journal of
Pediatrics
Eur. J. Pediatr. 132, 115-118 (1979)
9 by Springer-Verlag 1979
Case Reports Rheumatoid Arthritis During the First Year of Life A Case Report and Review of the Literature
N. Gadoth 1 and Y. Hershkovitch 2 *The Department of Neurology, Beilinson Medical Center, Petah Tiqva, Tel-Aviv University Sackler School of Medicine, and 2Department of Pediatrics "B" Soroka Medical Center, Beer-Sheba, Israel
Abstract. A 20-week-old female, first examined at 14 weeks of age for daily "spiking" fever and skin rash, subsequently developed signs of rheumatoid arthritis. The onset of rheumatoid arthritis during the first weeks of life is rare and its diagnosis may be difficult. It seems that onset during the later months of the first year is more frequently encountered. A review of the relevant publications in English disclosed that onset during first year was noticed in 1.02 percent of the patients, while in a study from Germany 6 to 9.9 percent of cases started during their first year. Key words: Early juvenile rheumatoid arthritis - Fever of unknown origin.
Although rheumatoid arthritis may present at any age throughout childhood, the onset of symptoms during the first year of life is uncommon. In a review of ten large series of cases of juvenile rheumatoid arthritis comprising a total of 2143 children, only 22 such cases are documented (1.02 percent). It seems that early onset is seen more frequently in Germany and these early cases comprise 6.5 to 9.9 percent of all children affected. A case is reported in which fever and extensive skin rash appeared at 14 weeks of age, with the subsequent evolution of classical rheumatoid arthritis. The young age Of the patient and the unusual, extensive edematous facial rash (which mimicked facial diplegia) prompted this report.
Case History A female infant, aged 20 weeks, was admitted with the following history: her birth weight was 3.1 kg after a normal delivery and uneventful pregnancy. Until 14 weeks of age, her general health and psychomotor development were normal, when restlessness, insomnia and daily spikes of fever up to 39~ first .became apparent. Address for offprint requests: N. Gadoth, M.D., Department of Neurology, Beilinson Medical Center, Petah Tiqva, Israel
0340-6199/79/0132/0115/$ 01.00
116
N. Gadoth and Y. Hershkovitch
,411.2
.40.S -40.0
E
"39.5 102.
938.9
101,
-38.4
100'
.37.8
99
.37.3
Sill.
.36.7
r/,
9
91i,
'35.6
4)
F'
C~ 1
2
3
4
fi
6
'
7
[ 8
9
'10
' 11
' 12
' 13 ' 114 ' 15
16
' 1"/ ' 18
DIA Y S IOOmg/K9 ASA Fig. 1. Fever pattern showing two single quotidian peaks, and a dramatic response to salicylate administration
Fig. 2. Spindle-shaped swelling of the interphalangeal joints in a 20-week old infant
Treatment with a variety of antibiotics had proved ineffective, and three weeks prior to admission a skin rash appeared. Her admission to hospital was prompted by the mother's concern about her daughter's inability to smile or grimace while crying9 On examination she appeared well-nourished, febrile, pale and irritable 9 The skin was warm and puffy and covered with a scaly, brownish macular rash over the face, trunk and extremities, but sparing the palms and soles. The face was expressionless due to lack of the normal skin creases, reminiscent of facial diplegia. This was so striking that an urgent neurological consultation was requested to rule out "Parkinsonism" or bilateral facial nerve paralysis.
Rheumatoid Arthritis During the First Year of Life
117
The neurological as well as the physical examinations were entirely normal. Daily fever reaching 38.5 centigrade was evident, but only on two occasions were single quotidian peaks present (Fig. 1). On the seventh hospital day spindle-shaped warm, red and tender swellings appeared at the proximal interphalangeal joints of the index and middle fingers of both hands, spreading later to all the interphalangeaI joints (Fig. 2). The rest of her joints were not affected. The liver and spleen were normal and no lymphadenopathy was present. A dramatic improvement resulted from treatment with aspirin (100 mg/kg of body weight) and prior to her discharge she was afebrile, symptom free, smiling and crying with the appropriate facial expression. Extensive laboratory work-up showed the following abnormal findings; Hemoglobin 8 g/dl, hematocrit 25%, white cell count 24,000 per mm 3, erythrocyte sedimentation rate 130 mm/h. Biopsy of involved skin showed non-specific inflammatory changes. No other manifestations of juvenile rheumatoid arthritis were observed over a two year follow-up period; however, discontinuance of aspirin medication promptly led to a relapse of arthritis and skin rash.
Discussion This infant p r e s e n t e d with the acute febrile (systemic) form o f j e v e n i l e r h e u m a t o i d arthritis (JRA). The characteristic f o r m o f fever seen in older children which is " s p i k y " a n d c o n t a i n s single a n d d o u b l e q u o t i d i a n p e a k s was n o t evident. Skin a n d small j o i n t s were affected while the liver, spleen o f l y m p h nodes were n o t enlarged. The y o u n g age o f onset p r o m p t e d a search for cases starting very early in life. In a review o f p u b l i c a t i o n s written in English only 22 such ~ases could be f o u n d a m o n g s t 2143 children (1.02%) in 10 large series (Table 1). A higher f r e q u e n c y o f onset d u r i n g the first y e a r was r e p o r t e d in G e r m a n y [7]. In this s t u d y 36 out o f 362 children with systemic J R A (9.9%) a n d 34 out o f 510 n o n - s y s t e m i c cases (6.5%) h a d the onset d u r i n g the first y e a r o f life.
Table 1. Onset during the first year of life in 10 large series of juvenile rheumatoid arthritis cases Series
No. of patients
Patients under 12 months
Kelley (1960) Schlesinger et al. (1961) Ansell and Bywaters (1963) Lindjberg (1964) Laaksonen (1966) Calabro and Marchesano (1968) Brewer (1970) Schaller and Wedgwood (1972) Goel and Shanks (1974) Grossman and Mukhopadhyay (1975)
100 100 316 75 544 100 100 124 100 584
1 6 3 1 6 1 1 0 2 1
2143
22
Total
118
N. Gadoth and Y. Hershkovitch
U n f o r t u n a t e l y , d e t a i l e d clinical d e s c r i p t i o n s of such y o u n g patients are not given in the m e n t i o n e d reports. This w o u l d suggest t h a t the m o d e o f onset in these infants is similar to t h a t in o l d e r children. H o w e v e r , the presence o f fever, l e u k o c y t o s i s , a n e m i a a n d increased s e d i m e n t a t i o n rate in a very y o u n g infant is most a l a r m i n g . In the absence o f a specific l a b o r a t o r y test to diagnose J R A , the c o m b i n a t i o n o f skin rash, m o d e r a t e l e u k o c y t o s i s , a n e m i a a n d increased s e d i m e n t a t i o n rate, s h o u l d alert the p e d i a t r i c i a n to the possibility o f J R A even in the first few weeks o f life.
Acknowledgement. We wish to thank M. Cohen, M.D. for his helpful comments.
References 1. Ansell, B. M., Bywaters, E. G. L.: Rheumatoid arthritis (Stitl's Disease). Pediatr. Clin. North Am. 10, 931 (1963) 2. Brewer, E. L. Jr.: Juvenile rhemnatoid arthritis. Major problems in clinical pediatrics, Vol. VI. Philadelphia: Saunders 1970 3. Calabro, J. J., Marchesano, J. M.: The early natural history of juvenile rheumatoid arthritis. A 10-year follow-up study of 100 cases. Med. Clin. North Am. 52, 567 (1968) 4. Goel, K. M., Shanks, R. A.: Follow-up study of 100 cases of juvenile rheumatoid arthritis. Ann. Rheum. Dis. 33, 25 (1974) 5. Grossman, B. J., Mukhopadhyay, D.: Juvenile rheumatoid arthritis. Current problems in pediatrics. Chicago: Year book of Medical Publishers 1975 6. Kelley, V. C.: Rheumatoid disease in childhood. Pediatr. Clin. North Am. 7, 435 (1960) 7. K6ile, G.: Die juvenile rheumatoide Arthritis (juvenile chronische Polyarthritis) und das Still-Syndrom. Rheumaforum 4. Karlsruhe: Braun 1975 8. Laaksonen, A. L.: A prognostic study of juvenile rheumatoid arthritis. Analysis of 544 cases. Acta Pediatr. Scand. (Suppl.) 166, 1 (1966) 9. Lindjberg, J. F.: Juvenile rheumatoid arthritis, a follow-up of 75 cases. Arcg. Dis.Child. 39, 576 (1964) 10. Schaller, J., Wedgwood, R. J.: Juvenile rheumatoid arthritis: A review. Pediatrics 50, 940 (1972) 11. Schlesinger, B. E., Forsyth, C. C., White, R. H., Smellie, J. M., Stroud, C. E.: Observations on the clinical course and treatment of one hundred cases of Still's Disease. Arch. Dis. Child. 36, 65 (1961) Received February 24, 1979
Pediatrics
Eur. J. Pediatr. 132, 115-118 (1979)
9 by Springer-Verlag 1979
Case Reports Rheumatoid Arthritis During the First Year of Life A Case Report and Review of the Literature
N. Gadoth 1 and Y. Hershkovitch 2 *The Department of Neurology, Beilinson Medical Center, Petah Tiqva, Tel-Aviv University Sackler School of Medicine, and 2Department of Pediatrics "B" Soroka Medical Center, Beer-Sheba, Israel
Abstract. A 20-week-old female, first examined at 14 weeks of age for daily "spiking" fever and skin rash, subsequently developed signs of rheumatoid arthritis. The onset of rheumatoid arthritis during the first weeks of life is rare and its diagnosis may be difficult. It seems that onset during the later months of the first year is more frequently encountered. A review of the relevant publications in English disclosed that onset during first year was noticed in 1.02 percent of the patients, while in a study from Germany 6 to 9.9 percent of cases started during their first year. Key words: Early juvenile rheumatoid arthritis - Fever of unknown origin.
Although rheumatoid arthritis may present at any age throughout childhood, the onset of symptoms during the first year of life is uncommon. In a review of ten large series of cases of juvenile rheumatoid arthritis comprising a total of 2143 children, only 22 such cases are documented (1.02 percent). It seems that early onset is seen more frequently in Germany and these early cases comprise 6.5 to 9.9 percent of all children affected. A case is reported in which fever and extensive skin rash appeared at 14 weeks of age, with the subsequent evolution of classical rheumatoid arthritis. The young age Of the patient and the unusual, extensive edematous facial rash (which mimicked facial diplegia) prompted this report.
Case History A female infant, aged 20 weeks, was admitted with the following history: her birth weight was 3.1 kg after a normal delivery and uneventful pregnancy. Until 14 weeks of age, her general health and psychomotor development were normal, when restlessness, insomnia and daily spikes of fever up to 39~ first .became apparent. Address for offprint requests: N. Gadoth, M.D., Department of Neurology, Beilinson Medical Center, Petah Tiqva, Israel
0340-6199/79/0132/0115/$ 01.00
116
N. Gadoth and Y. Hershkovitch
,411.2
.40.S -40.0
E
"39.5 102.
938.9
101,
-38.4
100'
.37.8
99
.37.3
Sill.
.36.7
r/,
9
91i,
'35.6
4)
F'
C~ 1
2
3
4
fi
6
'
7
[ 8
9
'10
' 11
' 12
' 13 ' 114 ' 15
16
' 1"/ ' 18
DIA Y S IOOmg/K9 ASA Fig. 1. Fever pattern showing two single quotidian peaks, and a dramatic response to salicylate administration
Fig. 2. Spindle-shaped swelling of the interphalangeal joints in a 20-week old infant
Treatment with a variety of antibiotics had proved ineffective, and three weeks prior to admission a skin rash appeared. Her admission to hospital was prompted by the mother's concern about her daughter's inability to smile or grimace while crying9 On examination she appeared well-nourished, febrile, pale and irritable 9 The skin was warm and puffy and covered with a scaly, brownish macular rash over the face, trunk and extremities, but sparing the palms and soles. The face was expressionless due to lack of the normal skin creases, reminiscent of facial diplegia. This was so striking that an urgent neurological consultation was requested to rule out "Parkinsonism" or bilateral facial nerve paralysis.
Rheumatoid Arthritis During the First Year of Life
117
The neurological as well as the physical examinations were entirely normal. Daily fever reaching 38.5 centigrade was evident, but only on two occasions were single quotidian peaks present (Fig. 1). On the seventh hospital day spindle-shaped warm, red and tender swellings appeared at the proximal interphalangeal joints of the index and middle fingers of both hands, spreading later to all the interphalangeaI joints (Fig. 2). The rest of her joints were not affected. The liver and spleen were normal and no lymphadenopathy was present. A dramatic improvement resulted from treatment with aspirin (100 mg/kg of body weight) and prior to her discharge she was afebrile, symptom free, smiling and crying with the appropriate facial expression. Extensive laboratory work-up showed the following abnormal findings; Hemoglobin 8 g/dl, hematocrit 25%, white cell count 24,000 per mm 3, erythrocyte sedimentation rate 130 mm/h. Biopsy of involved skin showed non-specific inflammatory changes. No other manifestations of juvenile rheumatoid arthritis were observed over a two year follow-up period; however, discontinuance of aspirin medication promptly led to a relapse of arthritis and skin rash.
Discussion This infant p r e s e n t e d with the acute febrile (systemic) form o f j e v e n i l e r h e u m a t o i d arthritis (JRA). The characteristic f o r m o f fever seen in older children which is " s p i k y " a n d c o n t a i n s single a n d d o u b l e q u o t i d i a n p e a k s was n o t evident. Skin a n d small j o i n t s were affected while the liver, spleen o f l y m p h nodes were n o t enlarged. The y o u n g age o f onset p r o m p t e d a search for cases starting very early in life. In a review o f p u b l i c a t i o n s written in English only 22 such ~ases could be f o u n d a m o n g s t 2143 children (1.02%) in 10 large series (Table 1). A higher f r e q u e n c y o f onset d u r i n g the first y e a r was r e p o r t e d in G e r m a n y [7]. In this s t u d y 36 out o f 362 children with systemic J R A (9.9%) a n d 34 out o f 510 n o n - s y s t e m i c cases (6.5%) h a d the onset d u r i n g the first y e a r o f life.
Table 1. Onset during the first year of life in 10 large series of juvenile rheumatoid arthritis cases Series
No. of patients
Patients under 12 months
Kelley (1960) Schlesinger et al. (1961) Ansell and Bywaters (1963) Lindjberg (1964) Laaksonen (1966) Calabro and Marchesano (1968) Brewer (1970) Schaller and Wedgwood (1972) Goel and Shanks (1974) Grossman and Mukhopadhyay (1975)
100 100 316 75 544 100 100 124 100 584
1 6 3 1 6 1 1 0 2 1
2143
22
Total
118
N. Gadoth and Y. Hershkovitch
U n f o r t u n a t e l y , d e t a i l e d clinical d e s c r i p t i o n s of such y o u n g patients are not given in the m e n t i o n e d reports. This w o u l d suggest t h a t the m o d e o f onset in these infants is similar to t h a t in o l d e r children. H o w e v e r , the presence o f fever, l e u k o c y t o s i s , a n e m i a a n d increased s e d i m e n t a t i o n rate in a very y o u n g infant is most a l a r m i n g . In the absence o f a specific l a b o r a t o r y test to diagnose J R A , the c o m b i n a t i o n o f skin rash, m o d e r a t e l e u k o c y t o s i s , a n e m i a a n d increased s e d i m e n t a t i o n rate, s h o u l d alert the p e d i a t r i c i a n to the possibility o f J R A even in the first few weeks o f life.
Acknowledgement. We wish to thank M. Cohen, M.D. for his helpful comments.
References 1. Ansell, B. M., Bywaters, E. G. L.: Rheumatoid arthritis (Stitl's Disease). Pediatr. Clin. North Am. 10, 931 (1963) 2. Brewer, E. L. Jr.: Juvenile rhemnatoid arthritis. Major problems in clinical pediatrics, Vol. VI. Philadelphia: Saunders 1970 3. Calabro, J. J., Marchesano, J. M.: The early natural history of juvenile rheumatoid arthritis. A 10-year follow-up study of 100 cases. Med. Clin. North Am. 52, 567 (1968) 4. Goel, K. M., Shanks, R. A.: Follow-up study of 100 cases of juvenile rheumatoid arthritis. Ann. Rheum. Dis. 33, 25 (1974) 5. Grossman, B. J., Mukhopadhyay, D.: Juvenile rheumatoid arthritis. Current problems in pediatrics. Chicago: Year book of Medical Publishers 1975 6. Kelley, V. C.: Rheumatoid disease in childhood. Pediatr. Clin. North Am. 7, 435 (1960) 7. K6ile, G.: Die juvenile rheumatoide Arthritis (juvenile chronische Polyarthritis) und das Still-Syndrom. Rheumaforum 4. Karlsruhe: Braun 1975 8. Laaksonen, A. L.: A prognostic study of juvenile rheumatoid arthritis. Analysis of 544 cases. Acta Pediatr. Scand. (Suppl.) 166, 1 (1966) 9. Lindjberg, J. F.: Juvenile rheumatoid arthritis, a follow-up of 75 cases. Arcg. Dis.Child. 39, 576 (1964) 10. Schaller, J., Wedgwood, R. J.: Juvenile rheumatoid arthritis: A review. Pediatrics 50, 940 (1972) 11. Schlesinger, B. E., Forsyth, C. C., White, R. H., Smellie, J. M., Stroud, C. E.: Observations on the clinical course and treatment of one hundred cases of Still's Disease. Arch. Dis. Child. 36, 65 (1961) Received February 24, 1979
