Table of Contents

HK J Paediatr (New Series)
Vol 1. No. 1, 1996

HK J Paediatr (New Series) 1996;1:74-76

Case Report

Henoch-Schönlein Purpura Associated with Infection due to Coxsackie B Virus

WLM Robson, AKC Leung, DD Horne


Abstract

Henoch-Schönlein purpura (HSP) developed in a 6 year old boy following a respiratory infection. Acute and convalescent viral serology demonstrated a rising titre to coxsackie B virus. Coxsackie B virus should be added to the list of possible precipitating agents that may be involved in the pathogenesis of HSP.

Keyword : Coxsackie virus; Henoch-Schönlein purpura


Introduction

Henoch-Schönlein purpura (HSP) is a vasculitic disease with an immunological pathogenesis that commonly involves the kidneys.1 We report a six year old boy who developed HSP in association with a respiratory infection due to coxsackie B virus.

Case Report

TB was born after an uncomplicated pregnancy, labour, and delivery. His birth weight was 3.38 kg. At the age of five weeks, he developed projective vomiting. Pyloric stenosis was diagnosed and the child underwent a pyloromyotomy (Ramstedt's operation).

At the age of six years, he developed a nasal discharge, cough, and fever. This was followed approximately one to two weeks later by abdominal pain, vomiting, aching in the legs, and a rash over the feet. On admission to hospital, his weight was 20 kg (20th percentile), height 118.5 cm (35th percentile), blood pressure 98/50 mm Hg, heart rate 120 beats per minute, respiratory rate 28 per minute, and temperature 37.6°C. He had mild anterior cervical lymphadenopathy, a functional grade 1 to 2 ejection systolic murmur at the left sternal border, tenderness and guarding to palpation of the lower abdomen, swelling of the ankles and feet, and a petechial and purpuric rash over the feet and the right knee. Blood tests on admission revealed a haemoglobin of 115 g/L, white blood cell (WBC) count of 20 x 109/L, with 63% neutrophils, 18% bands, 6% lymphocytes, 11% monocytes, and 2% atypical lymphocytes, platelet count 393 x 109/L, sodium 128 mmol/L, potassium 3.8 mmol/L, chloride 96 mmol/L, total carbon dioxide 28 mmol/L, urea 6.5 mmol/L, creatinine 55 umol/L, total protein 31 g/L, albumin 14 g/L, glucose 7.1 mmol/L, calcium 1.86 mmol/L, and phosphorus 1.43 mmol/L. A throat swab culture was normal. The anti-streptolysin O titre (ASOT) was 166 Todd units. Acute viral serology by complement fixation using a pool of coxsackie B1 virus antigen revealed a titre to coxsackie B of 1:64, 2 weeks after the onset of the illness. Two weeks later, the titre increased to 1:256, and 4 weeks later, the titre fell to 1:64. The prothrombin time and partial thromboplastin time were normal. The third and fourth components of the serum complement were 1.30 g/L and 0.30 g/L, respectively. The total haemolytic complement was 46,000 units per litre. The serum immunoglobulin G (IgG) was 5.96 g/L, immunoglobulin A (IgA) 2.62 g/L, and immunoglobulin M (IgM) 0.64 g/L. The urinalysis revealed a random specific gravity of 1.035, 3+ protein by dipstick, and greater than 100 red blood cells (RBC), 10 to 15 WBC, 5 to 7 WBC casts, 3 to 5 granular casts, and 8 to 10 hyaline casts per high power field. A diagnosis of HSP was established and the child was treated with prednisone 2 mg/kg/day for one month.

During his hospital admission, the patient also developed protein-losing enteropathy requiring intravenous hyperalimentation, renal colic due to ureteritis, bruising of the left scrotum and tenderness of the left testicle, focal seizures with twitching of the left hand, haematochezia, anaemia (haemoglobin of 6.6 g/L) requiring transfusion, hypertension requiring treatment with anti-hypertensive medications, nephrotic syndrome, and pulmonary oedema. The patient developed renal insufficiency with the serum creatinine and urea increasing to a maximum of 156 umol/L and 24.1 mmol/L, respectively. The patient was hospitalized for two months. On discharge, the blood creatinine and urea were 66 umol/L and 21 mmol/L, respectively, and the total serum protein and albumin were 48 g/L and 29 g/L, respectively.

One year after the initial presentation, the BP was 110/ 60 mm Hg, and the urinalysis revealed 2+ protein by dipstick and 10 to 20 RBC and 2 to 5 WBC per high power field. The blood creatinine was 71 umol/L, urea 5.9 mmol/L, creatinine clearance 131 ml/min/1.73m2, and 24 hour urine collection for protein 707 mg. Fifteen months after the initial presentation, the patient developed painless gross haematuria and generalized oedema in association with pharyngitis. The patient did not develop abdominal pain, joint pain or swelling, or a rash. The throat swab grew group A beta-haemolytic streptococci and the ASOT was 833 Todd units, falling to 625 Todd units two months later. The blood creatinine and urea were 78 umol/L and 19.8 mmol/L, respectively. A 24 hour urine collection revealed a creatinine clearance of 98 ml/mm/1.73m2 and 3845 mg of protein. The serum total protein and albumin were 50 umol/L and 26 mmol/L, respectively. The patient did not experience any further relapses and, 11 years after the acute presentation, the creatinine clearance was 85 ml/mm/1.73m2 and the 24 hour urine protein was 438 mg.

Discussion

HSP is considered to be a vasculitic disorder with an immune pathogenesis.1 An immunopathogenesis involving immunoglobulin A (IgA) is suggested based on the presence of an elevated serum IgA concentration, circulating IgA-immune complexes, IgA-rheumatoid factor, IgA-fibronectin complexes, IgA-anti-alpha-galactosyl antibody, an increased percentage of IgA-bearing peripheral blood lymphocytes, and immunoregulatory abnormalities involving IgA production.1,2 Elevated levels of serum IgG and IgM have also been noted.1,2

HSP develops in response to a variety of common, usually infective, mucosal stimuli.2 Mucosal B-cells are stimulated to synthesise dimeric IgA, which circulates in the blood. Normal T-cell control of immunoglobulin synthesis is impaired. The IgA interacts with IgG, and perhaps some other proteins, and forms high molecular weight macromolecules (immune complexes), which activate the alternate pathway of the complement system. Whether by virtue of specific physical properties such as size, adhesiveness, or electrical charge, or due to specificity characteristics such as cross-reactivity or lectin binding, or by some inadequacy of the clearance mechanisms, a percentage of the circulating IgA is deposited in the affected organs, and precipitates an inflammatory process.2 A leucocytoclastic vasculitis is the final immunopathological result.1,2 In the majority of cases, normal control mechanisms reassert themselves and complete resolution of the process ensues.2

Respiratory infections commonly precede the onset of HSP. Schönlein was the first to note that a respiratory infection may precede the onset of HSP.3 Infection with group A beta-haemolytic streptococcus (GABHS) is the most frequent organism reported to precede HSP. We reviewed 16 studies that reported on the frequency of preceding streptococcal infection in patients with HSP.4 A history of preceding respiratory infection was found in 566 (62%) of 913 patients. A throat swab was positive for beta-haemolytic streptococci in 193 (24%) of 797 patients. The ASOT was elevated in 167 (35%) of 482 patients. The frequency of preceding infection with beta-haemolytic streptococci in the 16 studies varied from 7% to 47%.4 In a prospective study that we performed, evidence of preceding infection with GABHS was found in 18 (60%) of 30 patients with HSP.4 Infections with parvovirus, adenovirus, Mycoplasma pneumoniae, and varicella-zoster virus, and measles vaccination8,9 have also been reported to precede HSP.5-9

The patient that we report had evidence of infection with coxsackie B virus. Infection with this virus may have provided the immunological trigger for the HSP. To our knowledge, this is the first report of a patient who developed HSP in association with infection with coxsackie B virus. Coxsackie B virus has been reported to cause a variety of clinical syndromes including nonspecific febrile illnesses, upper respiratory tract infections, gastroenteritis, roseoliform exanthems, orchitis, aseptic meningitis, myo-pericarditis, and epidemic pleurodynia. In infants, coxsackie B viruses are known to cause an often lethal encephalo-myocarditis and chronic viral meningitis in children with hypogammaglobulinaemia. Coxsackie B viruses have also been associated with the haemolytic-uraemic syndrome and have caused some cases of oliguric renal failure in adults.10,11

Although the serological evidence of a recent coxsackie B virus infection is not definite proof of the aetiology of a temporally associated illness, it is strong presumptive evidence of an aetiologic association. The most definitive aetiologic evidence is provided by virus isolation from normally sterile sites such as cerebrospinal fluid, pericardial fluid, or blood. In this case, virus isolation was unsuccessful and unlikely to be successful because HSP is thought to be due to immunologic response to a triggering agent which has disappeared by the time the disease is fully developed. Since a viral isolate was not obtained in this case, coxsackie virus subtyping could not be done with certainty.

Acknowledgement

The authors would like to thank Mrs Heather Sol for expert secretarial assistance, and Mr Sulakhan Chopra of the University of Calgary medical library for help in the preparation of this manuscript.


References

1. Robson WLM, Leung AKC. Henoch-Schönlein purpura. Adv Pediatr 1994;41:168-94.

2. Knight JF. The rheumatic poison a survey of some published investigations of the immunopathogenesis of Henoch-Schönlein purpura. Pediatr Nephrol 1990;4:533-41.

3. Schönlein JL. Allgemeine und specielle Pathologie und Therapie, 3rd ed., Wurzburg: Herisau 1837;p48.

4. Robson WLM, Leung AKC. The frequency of preceding infection with group A beta-hemolytic streptococcus in patients with Henoch-Schönlein purpura. J Singapore Paediatr Soc 1993;35:168-72.

5. Laffère JJ, Couroucé AM, Soulier JP, et at. Henoch-Schönlein purpura and human parvovirus infection. Pediatrics 1986;78:183-4.

6. Meadow SR, Scott DG. Berger disease: Henoch-Schönlein syndrome without the rash. J Pediatr 1985;106:27-32.

7. Sussman M, Jones JH, Almeida JD, et al. Deficiency of the second component of complement associated with anaphylactoid purpura and presence of mycoplasma in the serum. Clin Exp Immunol 1973;14:531-9.

8. Leonardi S, Fischer A, Arcidiacono G, et al. Chickenpox and Schönlein-Henoch purpura: a report of a case with nephropathy. Pediatr Med Chir 1992;5:535-7.

9. Ozaki T, Miwata H, Kodama H, et al. Henoch-Schönlein purpura after measles immunization. Acta Paediatr Jpn 1989;4:484-6.

10. Ray CG, Tucker VL, Harris DJ, et al. Enteroviruses associated with the hemolytic-uremic syndrome. Pediatrics 1970;46:378-80.

11. Aronson MD, Phillips CA. Coxsackie B5 infections in acute oliguric renal failure. J Infect Dis 1975;132:303-4.

 
 

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