S A. AL RASHEED ET AL
Henoch-Schonlein Syndrome in Saudi Arabia by Saud A. Al Rasheed, FRCPC, Murtala B. Abdurrahman, FRCPC, Mohamed M. Al Mugciren, FRCPC and Ibrahim M. Al Fawaz, FRCPC Department of Paediatrics, College of Medicine and King Khalid University Hospital, Riyadh, Saudi Arabia
Introduction Henoch-Schonlein syndrome is a systemic small vessel vasculitis of unknown cause. It is characterized by purpuric skin rash, abdominal pain, and arthritis. The condition was first described by Heberden in 1801. Schonlein described the arthritic component in 1837,' and Henoch reported on the association with gastrointestinal manifestation and the renal abnormalities.2 For a disease with such dramatic and characteristic clinical features there are surprisingly few epidemiological data, 3 ' 4 and many of the published series have an inherent bias toward more severe cases or towards the special interests of the authors. To our knowledge there have been no epidemiological data about the condition reported from Saudi Arabia. The purpose of this retrospective study was to define the clinicopathological features and prognosis of Henoch-Schonlein syndrome in children in Saudi Arabia. Materials and methods We reviewed the case files of all children with Henoch-Schonlein syndrome seen in King Khalid University Hospital (KKUH), Riyadh from April 1982 to December 1988. Diagnosis of Henoch-Schonlein syndrome was based on the typical clinical picture of non-thrombocytopenic purpura associated with one or more of the following: arthritis and arthralgia, abdominal pain, gastrointestinal haemorrhage, and nephritis. The information extracted from the case files included: 1. Clinical features: age of onset; season; history of drug ingestion or upper respiratory tract infection for 2 weeks prior to presentation; symptoms and signs. Correspondence: Dr S A Al Rasheed, Department of Paediatrics, College of Medicine, P.O. Box 2925, Riyadh 11461, Saudi Arabia. © Oxford University Press 1991
2. Investigations: haemogram; urinalysis; blood chemistry; prothrombin and partial thrombotoplastin times; antinuclear antibodies; C-reactive protein; serum immunoglobulins; antistreptolysin O titre; throat culture and histological findings in those who had renal biopsy. 3. Management and outcome. 4. Follow-up data. Follow-up data included information on blood sure, presence or absence of skin rash, abdominal and renal impairment as well as the results of dipsticks for haematuria and proteineuria, and microscopic examination.
prespain, urine urine
Results During a 6j-year period from April 1982 to December 1988 there were 40 cases with Henoch-Schonlein syndrome. There were 23 boys and 17 girls: 34 of them were Saudi Arabs and 6 were non-Saudi Arabs. The age of onset of Henoch-Schonlein syndrome ranged from 5 months to 13 years, with a mean of 6.9 years. There were more cases during the winter months as shown in Fig. 1. There was a history of preceding upper respiratory tract infection in 20 patients (50 per cent), and a history of drug ingestion in 14 patients (35 per cent). A summary of the frequency of other symptoms and signs is shown in Table 1. The skin rash was purpuric in 70 per cent and macular in 30 per cent. The arthritis involved mainly the knees and ankles. At admission seven patients had hypertension, but this was only transient in four patients. Renal biopsy was done in three patients. One had focal proliferative glomerulonephritis, the second patient had a picture of end stage kidney disease, and the biopsy of the third one was inconclusive because it contained only medullary tissue. The first patient improved, but the other two developed end stage renal failure requiring dialysis
Journal of Tropical Pediatrics
Vol.37
June 1991
127
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
Summary A retrospective study was carried out on 40 children from Saudi Arabia with Henoch-Schdnlein syndrome to delineate its clinical pattern. More than 50 per cent of the cases occurred in winter. There was no apparent causal relationship with B-haemolyric streptococcal infection. All patients had skin rash. Fiftyeight per cent had gastrointestinal manifestations, 58 per cent had joint manifestations and 38 per cent had renal manifestations. The main clinical features of Henoch-Schonlein syndrome in Saudi children are comparable to those reported elsewhere.
S. A AL RASHEED ET AL
2
5
& 4
Feb
Mar
Apr
May
Jun
Jul
Aug
Sep
Oct
Nov
Dec
Months
FIG. 1. Seasonal occurrence of Henoch-Schonlein Syndrome. TABLE 1
Frequency of symptoms and signs in 40 Saudi children with Henoch-Schonlein syndrome Symptom/sign Skin rash Joint symptom/sign Abdominal pain Fever Melena Hacmatemesis Bloody diarrhoea Massive GI bleeding Haematuria Proteinuria Nephrotic syndrome Renal failure
No. of patients
%
40 23 23 18 13 8 7 3 15 13 4 2
100 57.5 57.5 45 32.5 20 17.5 7.5 37.5 32.5 10 5
therapy. Four patients had haemoglobin below 10 g/dl with normocytic normochromic picture. All patients had a normal platelet count, C 3 , measured in 17 patients, was normal. Antistreptolysin O titre was measured in 21 patients: four of them had a value of > 300 units. C-reactive protein was measured in six patients and all were positive. Throat culture was done in 33 patients; only one had a positive growth of group A streptococci. Serum IgG level was measured in 10 patients; six of them had a high level. Serum IgA level was measured in 13 patients only and all had normal values. Supportive therapy was offered to all patients. Ten patients had a short course of prednisolone 2 mg/kg/day for 7-10 days because of severe abdominal pain and massive gastrointestinal haemorrhage. The steriods seem to alleviate the symptoms within 2-3 128
days. Twenty-six patients attended more than one follow-up visit. The findings during the first and the last visit is summarized in Table 2. Discussion
The main clinicopathological features of HenochSchonlein syndrome in Saudi Arabian children, as described in the present study, are similar to those reported from other parts of the world (Table 3). Although the disease is considered rare in young children, three of our patients were under 1 year of age, the youngest being 5 months old. In a large series of patients reported by Allen et a/.3 the youngest patient was 6 months of age. In our study there were no cases occurring in the same family, but his has been reported in rare instances.3-7 The disease occurred throughout the year with a peak incidence during the winter months from October to January, as reported by others. 3 7 A preceding upper respiratory tract infection occurred in 50 per cent of our patients. Greater percentage has been reported in other series3-4-8 Several microorganisms have been implicated ranging from hemolytic streptococci 9 and Mycoplasma pneumonia 10 to human parvovirus." We did not find a strong causal relationship between B-haemolytic streptococcal infection and the development of Henoch-Schonlein syndrome which support the conclusion of Atkinson and Barker, 4 and Ayoub and Hoyer.8 Treatment of Henoch-Schonlein syndrome is mainly conservative. A short course of corticosteroids has been found useful in treating severe colicky abdominal pain and gastrointestinal haemorrhage. 3 1 3 Ten of our patients had a short course of steroids for the same indication and had a favourable Journal of Tropical Pediatrics
Vol. 37
June 1991
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
Jan
S A AL RASHEED ET AL
TABLE 2
Follow-up results in 40 Saudi children with Henoch-Schonlein syndrome Number of patients Finding
First visit
Last visit
20 6 3 3 7 2 3 4
13 7 0 3 3 2 4 3
Total number of patients Normal Recurrent abdominal pain Recurrent skin rash Miscroscopic haematuria Renal failure Hypertension Urine dipstick for haematuria or urine microscopy was not done
Frequency of symptom/sign No. of patients
Rash
Joint swelling or arthritis
Gastrointestinal
Renal
Reference
131 76 43 40
100 98.7 100 100
70 89.5 79 57.5
69 59.2 62.8 57.5
33 30.3 37.2 37.5
3 4 12
Allen el al. (I960) 3 Atkinson and Barker (1976)* Emory et al. (1977) 13 Present study
response. The justification for the use of corticosteroids has been questioned recently, because of the selflimited nature of the abdominal pain.1* The prognosis of Henoch-Scholein syndrome is generally excellent. 13 ' 15 Nephritis is the most important determinant of outcome. Five per cent of children who develop HSP progress to end-stage renal failure. Two of our patients developed end-stage renal failure, both of them had nephrotic range proteinuria and both were more than 7 years of age. Only half of our patients attended at least one follow-up visit after discharge from hospital, and even smaller number came for subsequent visits. For this reason it is not possible to comment on the prognosis of HenochSchonlein syndrome in Saudi children. Children with nephritic picture at presentation should be followed closely for at least five years." Others may not need such a prolonged follow up period." It may be concluded that the nature of HenochSchonlein syndrome in Saudi Arabia is the same as reported from other parts of the world. The importance of long-term follow-up of at least those patients manifesting with nephritis at the time of presentation should be stressed. References 1. Marx K. Henoch purpura revisited. Am J Dis Childh 1974; 129: 74. Journal of Tropical Pediatrics
Vol. 37
June 1991
2. Henoch EHH. About a peculiar form of purpura. Am J Dis Childh 1974; 128: 78-9. 3. Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome). Am J Dis Childh 1960; 99: 833-54. 4. Atkinson SR, Barker DJP. Seasonal distribution of Henoch-Schonlein purpura. Br J Prev Soc Med 1976; 30: 22-5. 5. Meadow R, Schonlein-Henoch Syndrome. In: Edelman CM (ed.) Pediatric renal disease, Boston: Little Brown, 1978; 2: 788-96. 6. Habib R, Broyer M, Levy M. Schonlein-Henoch purpura glomerulonephritis in children. In: Strause J (ed.) Pediatric nephrology. New York: Plenum Press, 1976; 155-95. 7. Meadow SR, Glasgow EF, White RHR, MoncrieffMW, Cameron JS, Ogg CS. Schonlein-Henoch nephritis. Q J Med 1972; 163: 241-58. 8. Ayoub EM, Hoyer J. Anaphylactoid purpura: streptococcai antibody titers and Blc-globulin levels. J Pediat 1969; 73: 193-201. 9. Gardner D. The Schonlein-Henoch Syndrome (anaphylactoid purpura). Q J Med 1948; 17: 95-122. 10. Liew SW, Kessel I. Mycoplasma pneumonia preceding Henoch-Schonlein purpura. Arch Dis Childh 1974; 912-14. 11. Lefrere JJ, Courouce AM, Soulier JP, Cordier MP, Guesne Girault MC, Polonovski C, Bensman A. Henoch-Schonlein purpura and human parvovirus infection. Pediatrics 1986; 78: 183-4. 12. Emory H, Larter W, Schaller JG. Henoch-Schonlein vasculitis. Arthritis Rheum 1977; 20: 385-8. 129
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
TABLE 3
Major clinicalfindingsin children with Henoch-Schonlein syndrome
S. A. AL RASHEED ET AL
13. Barrett TM, Drummond KN. The vasculitis syndromes: Henoch-Schonlein syndrome or anaphylactoid purpura. In Kelly VC (ed.): Practice of pediatrics. Philadelphia: Harper and Row, 1983; Vol 8, 1-13. 14. Rosenblum ND, Winter HS. Steriod effects on the course of abdominal pain in children with Henoch-Schonlein purpura. Pediatrics 1987; 79: 1018-21.
15. Cassidy JT. Systemic vasculitis. In Cassidy JT (ed.) Textbook of pediatric rheumatology. New York: John Wiley and Sons, 1982; 496-503. 16. Coakley JC, Chambers TL. Should we follow up children with Henoch-Schonlein syndrome? Arch Dis Childh 1979; 54: 903-4.
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
130
Journal of Tropical Pediatrics
Vol.37
June 1991
Henoch-Schonlein Syndrome in Saudi Arabia by Saud A. Al Rasheed, FRCPC, Murtala B. Abdurrahman, FRCPC, Mohamed M. Al Mugciren, FRCPC and Ibrahim M. Al Fawaz, FRCPC Department of Paediatrics, College of Medicine and King Khalid University Hospital, Riyadh, Saudi Arabia
Introduction Henoch-Schonlein syndrome is a systemic small vessel vasculitis of unknown cause. It is characterized by purpuric skin rash, abdominal pain, and arthritis. The condition was first described by Heberden in 1801. Schonlein described the arthritic component in 1837,' and Henoch reported on the association with gastrointestinal manifestation and the renal abnormalities.2 For a disease with such dramatic and characteristic clinical features there are surprisingly few epidemiological data, 3 ' 4 and many of the published series have an inherent bias toward more severe cases or towards the special interests of the authors. To our knowledge there have been no epidemiological data about the condition reported from Saudi Arabia. The purpose of this retrospective study was to define the clinicopathological features and prognosis of Henoch-Schonlein syndrome in children in Saudi Arabia. Materials and methods We reviewed the case files of all children with Henoch-Schonlein syndrome seen in King Khalid University Hospital (KKUH), Riyadh from April 1982 to December 1988. Diagnosis of Henoch-Schonlein syndrome was based on the typical clinical picture of non-thrombocytopenic purpura associated with one or more of the following: arthritis and arthralgia, abdominal pain, gastrointestinal haemorrhage, and nephritis. The information extracted from the case files included: 1. Clinical features: age of onset; season; history of drug ingestion or upper respiratory tract infection for 2 weeks prior to presentation; symptoms and signs. Correspondence: Dr S A Al Rasheed, Department of Paediatrics, College of Medicine, P.O. Box 2925, Riyadh 11461, Saudi Arabia. © Oxford University Press 1991
2. Investigations: haemogram; urinalysis; blood chemistry; prothrombin and partial thrombotoplastin times; antinuclear antibodies; C-reactive protein; serum immunoglobulins; antistreptolysin O titre; throat culture and histological findings in those who had renal biopsy. 3. Management and outcome. 4. Follow-up data. Follow-up data included information on blood sure, presence or absence of skin rash, abdominal and renal impairment as well as the results of dipsticks for haematuria and proteineuria, and microscopic examination.
prespain, urine urine
Results During a 6j-year period from April 1982 to December 1988 there were 40 cases with Henoch-Schonlein syndrome. There were 23 boys and 17 girls: 34 of them were Saudi Arabs and 6 were non-Saudi Arabs. The age of onset of Henoch-Schonlein syndrome ranged from 5 months to 13 years, with a mean of 6.9 years. There were more cases during the winter months as shown in Fig. 1. There was a history of preceding upper respiratory tract infection in 20 patients (50 per cent), and a history of drug ingestion in 14 patients (35 per cent). A summary of the frequency of other symptoms and signs is shown in Table 1. The skin rash was purpuric in 70 per cent and macular in 30 per cent. The arthritis involved mainly the knees and ankles. At admission seven patients had hypertension, but this was only transient in four patients. Renal biopsy was done in three patients. One had focal proliferative glomerulonephritis, the second patient had a picture of end stage kidney disease, and the biopsy of the third one was inconclusive because it contained only medullary tissue. The first patient improved, but the other two developed end stage renal failure requiring dialysis
Journal of Tropical Pediatrics
Vol.37
June 1991
127
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
Summary A retrospective study was carried out on 40 children from Saudi Arabia with Henoch-Schdnlein syndrome to delineate its clinical pattern. More than 50 per cent of the cases occurred in winter. There was no apparent causal relationship with B-haemolyric streptococcal infection. All patients had skin rash. Fiftyeight per cent had gastrointestinal manifestations, 58 per cent had joint manifestations and 38 per cent had renal manifestations. The main clinical features of Henoch-Schonlein syndrome in Saudi children are comparable to those reported elsewhere.
S. A AL RASHEED ET AL
2
5
& 4
Feb
Mar
Apr
May
Jun
Jul
Aug
Sep
Oct
Nov
Dec
Months
FIG. 1. Seasonal occurrence of Henoch-Schonlein Syndrome. TABLE 1
Frequency of symptoms and signs in 40 Saudi children with Henoch-Schonlein syndrome Symptom/sign Skin rash Joint symptom/sign Abdominal pain Fever Melena Hacmatemesis Bloody diarrhoea Massive GI bleeding Haematuria Proteinuria Nephrotic syndrome Renal failure
No. of patients
%
40 23 23 18 13 8 7 3 15 13 4 2
100 57.5 57.5 45 32.5 20 17.5 7.5 37.5 32.5 10 5
therapy. Four patients had haemoglobin below 10 g/dl with normocytic normochromic picture. All patients had a normal platelet count, C 3 , measured in 17 patients, was normal. Antistreptolysin O titre was measured in 21 patients: four of them had a value of > 300 units. C-reactive protein was measured in six patients and all were positive. Throat culture was done in 33 patients; only one had a positive growth of group A streptococci. Serum IgG level was measured in 10 patients; six of them had a high level. Serum IgA level was measured in 13 patients only and all had normal values. Supportive therapy was offered to all patients. Ten patients had a short course of prednisolone 2 mg/kg/day for 7-10 days because of severe abdominal pain and massive gastrointestinal haemorrhage. The steriods seem to alleviate the symptoms within 2-3 128
days. Twenty-six patients attended more than one follow-up visit. The findings during the first and the last visit is summarized in Table 2. Discussion
The main clinicopathological features of HenochSchonlein syndrome in Saudi Arabian children, as described in the present study, are similar to those reported from other parts of the world (Table 3). Although the disease is considered rare in young children, three of our patients were under 1 year of age, the youngest being 5 months old. In a large series of patients reported by Allen et a/.3 the youngest patient was 6 months of age. In our study there were no cases occurring in the same family, but his has been reported in rare instances.3-7 The disease occurred throughout the year with a peak incidence during the winter months from October to January, as reported by others. 3 7 A preceding upper respiratory tract infection occurred in 50 per cent of our patients. Greater percentage has been reported in other series3-4-8 Several microorganisms have been implicated ranging from hemolytic streptococci 9 and Mycoplasma pneumonia 10 to human parvovirus." We did not find a strong causal relationship between B-haemolytic streptococcal infection and the development of Henoch-Schonlein syndrome which support the conclusion of Atkinson and Barker, 4 and Ayoub and Hoyer.8 Treatment of Henoch-Schonlein syndrome is mainly conservative. A short course of corticosteroids has been found useful in treating severe colicky abdominal pain and gastrointestinal haemorrhage. 3 1 3 Ten of our patients had a short course of steroids for the same indication and had a favourable Journal of Tropical Pediatrics
Vol. 37
June 1991
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
Jan
S A AL RASHEED ET AL
TABLE 2
Follow-up results in 40 Saudi children with Henoch-Schonlein syndrome Number of patients Finding
First visit
Last visit
20 6 3 3 7 2 3 4
13 7 0 3 3 2 4 3
Total number of patients Normal Recurrent abdominal pain Recurrent skin rash Miscroscopic haematuria Renal failure Hypertension Urine dipstick for haematuria or urine microscopy was not done
Frequency of symptom/sign No. of patients
Rash
Joint swelling or arthritis
Gastrointestinal
Renal
Reference
131 76 43 40
100 98.7 100 100
70 89.5 79 57.5
69 59.2 62.8 57.5
33 30.3 37.2 37.5
3 4 12
Allen el al. (I960) 3 Atkinson and Barker (1976)* Emory et al. (1977) 13 Present study
response. The justification for the use of corticosteroids has been questioned recently, because of the selflimited nature of the abdominal pain.1* The prognosis of Henoch-Scholein syndrome is generally excellent. 13 ' 15 Nephritis is the most important determinant of outcome. Five per cent of children who develop HSP progress to end-stage renal failure. Two of our patients developed end-stage renal failure, both of them had nephrotic range proteinuria and both were more than 7 years of age. Only half of our patients attended at least one follow-up visit after discharge from hospital, and even smaller number came for subsequent visits. For this reason it is not possible to comment on the prognosis of HenochSchonlein syndrome in Saudi children. Children with nephritic picture at presentation should be followed closely for at least five years." Others may not need such a prolonged follow up period." It may be concluded that the nature of HenochSchonlein syndrome in Saudi Arabia is the same as reported from other parts of the world. The importance of long-term follow-up of at least those patients manifesting with nephritis at the time of presentation should be stressed. References 1. Marx K. Henoch purpura revisited. Am J Dis Childh 1974; 129: 74. Journal of Tropical Pediatrics
Vol. 37
June 1991
2. Henoch EHH. About a peculiar form of purpura. Am J Dis Childh 1974; 128: 78-9. 3. Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome). Am J Dis Childh 1960; 99: 833-54. 4. Atkinson SR, Barker DJP. Seasonal distribution of Henoch-Schonlein purpura. Br J Prev Soc Med 1976; 30: 22-5. 5. Meadow R, Schonlein-Henoch Syndrome. In: Edelman CM (ed.) Pediatric renal disease, Boston: Little Brown, 1978; 2: 788-96. 6. Habib R, Broyer M, Levy M. Schonlein-Henoch purpura glomerulonephritis in children. In: Strause J (ed.) Pediatric nephrology. New York: Plenum Press, 1976; 155-95. 7. Meadow SR, Glasgow EF, White RHR, MoncrieffMW, Cameron JS, Ogg CS. Schonlein-Henoch nephritis. Q J Med 1972; 163: 241-58. 8. Ayoub EM, Hoyer J. Anaphylactoid purpura: streptococcai antibody titers and Blc-globulin levels. J Pediat 1969; 73: 193-201. 9. Gardner D. The Schonlein-Henoch Syndrome (anaphylactoid purpura). Q J Med 1948; 17: 95-122. 10. Liew SW, Kessel I. Mycoplasma pneumonia preceding Henoch-Schonlein purpura. Arch Dis Childh 1974; 912-14. 11. Lefrere JJ, Courouce AM, Soulier JP, Cordier MP, Guesne Girault MC, Polonovski C, Bensman A. Henoch-Schonlein purpura and human parvovirus infection. Pediatrics 1986; 78: 183-4. 12. Emory H, Larter W, Schaller JG. Henoch-Schonlein vasculitis. Arthritis Rheum 1977; 20: 385-8. 129
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
TABLE 3
Major clinicalfindingsin children with Henoch-Schonlein syndrome
S. A. AL RASHEED ET AL
13. Barrett TM, Drummond KN. The vasculitis syndromes: Henoch-Schonlein syndrome or anaphylactoid purpura. In Kelly VC (ed.): Practice of pediatrics. Philadelphia: Harper and Row, 1983; Vol 8, 1-13. 14. Rosenblum ND, Winter HS. Steriod effects on the course of abdominal pain in children with Henoch-Schonlein purpura. Pediatrics 1987; 79: 1018-21.
15. Cassidy JT. Systemic vasculitis. In Cassidy JT (ed.) Textbook of pediatric rheumatology. New York: John Wiley and Sons, 1982; 496-503. 16. Coakley JC, Chambers TL. Should we follow up children with Henoch-Schonlein syndrome? Arch Dis Childh 1979; 54: 903-4.
Downloaded from http://tropej.oxfordjournals.org/ at Harvard University on June 26, 2015
130
Journal of Tropical Pediatrics
Vol.37
June 1991
