Henoch-Schönlein Purpura (HSP) – What can we Learn from Biomedical and Clinical Anatomy Practice on this Multidisciplinary Disease?

Review Article

Austin J Anat. 2018; 5(2): 1083.

Henoch-Schönlein Purpura (HSP) – What can we Learn from Biomedical and Clinical Anatomy Practice on this Multidisciplinary Disease?

Muhammad SN1,2* and Abdel Meguid E3

1The University of the West of England (UWE), Department of Health and Applied Sciences (HAS), UK

2The Renal Patient Support Group (RPSG), UK

3Queen’s University Belfast, School of Medicine and Dentistry and Biomedical Sciences, UK

*Corresponding author: Muhammad SN, The University of the West of England (UWE), Department of Health and Applied Sciences (HAS), The Renal Patient Support Group (RPSG), UK

Received: April 06, 2018; Accepted: April 25, 2018; Published: May 02, 2018

Abstract

Background: Henoch-Schönlein Purpura (HSP) is the most common vasculitis in children, characterized by triad of symptoms; 1) palpable purpura without thrombocytopenia, 2) abdominal pain, and 3) arthritis. Renal involvement also often occurs in children with HSP. Henoch-Schönlein Purpura (HSP) is the most common vasculitis in children, occurring in 8 to 20.4 per 100,000 children per year.

Epidemiology: HSP usually occurs in children aged between 2-10 years, with 50% of all cases occurred in children aged <5 years, mostly in children aged 4-6 years and occurs more frequently in male. Although it is generally a self-limiting condition, HSP can cause renal manifestations with various incidences. The guidelines and treatment for managing care in HSP patients ranges between centers.

Aims: 1) To provide practical understanding of Henoch-Schonlein Purpura (HSP), 2) Highlight the importance of multidisciplinary laboratory practice for patients with HSP, and 3) Highlight what laboratory practices could be enhanced to support the development of HSP guidelines.

Practice: HSP is a multi-specialty disease wherein a patient’s care plan will have laboratory involvement from a variety of disciplines. Laboratory investigations suggest that HSP is not a self-limited disease and patients eventually develop CKD.

Research Stance: In a retrospective study of 141 patients with HSP, Zhao et al. 2015 (9) demonstrated that abdominal pain was not related to HSP Nephritis (HSPN). However, 45% of the patients were complicated with obesity and 29.8% of them had a long disease course. Multidisciplinary laboratory perspectives are paramount in disease follow-up and to help inform clinical decision-making.

Discussion: Future biomedical/ laboratory practices can help tighter clinical decision-making in the care of young people with HSP. More case and longitudinal studies would be helpful to understand whether patients would benefit different care plan options.

Conclusion: Evidence informs that male gender, age greater than 10 years, have symptoms of severe gastrointestinal involvement, arthritis/arthralgia. So, what can we learn from this multidisciplinary disease? Certainly, well-designed and conventionally reported studies in histology, microbiology and hematology laboratory collaborations are important to identify HSP disease development and progression.

Keywords: Henoch-Schönlein Purpura (HSP); Hematology; Anatomy; Histology; Microbiology; Renal

Introduction

Henoch-Schönlein Purpura (HSP) is, characterized by triad of symptoms; 1) palpable purpura without thrombocytopenia, 2) abdominal pain, and 3) arthritis. Renal involvement also often occurs in children with HSP [1]. It is more frequently in male [1,2]. In spite of its being a self-limiting condition, HSP can cause renal manifestations with various incidences [3-9].

The guidelines and treatment for managing care in such patient’s ranges between centres [2,3-9]. Long-term prognosis depends mainly on the severity of renal involvement, which may manifest as persistent haematuria, proteinuria, nephritic or nephrotic syndrome, or even renal failure [10]. To prevent or delay end-stage renal disease, identification of early-stage nephritis is crucial. The risk factors associated with renal involvement in HSP are not clear although epidemiologic and clinical features as well as some abnormal laboratory findings have been suggested to have a predictive role [11].

Aims

The aims of this review are: 1) To provide practical understanding of HSP, 2) To provide an understanding of what further multidisciplinary care is required for patients with HSP and 3) To highlight how laboratory practices could be enhanced to support the development of HSP care guidelines.

Epidemiology

Henoch-Schönlein Purpura (HSP) is the most common vasculitis in children, occurring in 8 to 20.4 per 100,000 children per year [5-7]. HSP usually occurs in children aged between 2–10 years, with 50% of all cases occurred in children aged <6 years, mostly in children aged 4–6 years [5,6]. Although it is generally a self-limiting condition, HSP can cause acute gastrointestinal manifestations (bleeding, intussusception), and/or renal manifestations with various incidence [7-8].

Age of onset is a suspect risk factor for renal manifestations in HSP [8]. Aetiology of HSP is unknown, although a variety of antigens such as infections, vaccinations, drugs, foods and insect bites can trigger the onset of HSP [9-11].

The disease is characterized by deposits of Immunoglobulin A (IgA) which contained immune complexes and complement components in small blood vessel walls [12]. It is generally agreed that the incidence of HSP decreases with age [12]. The proportion of children presenting with renal involvement reported in studies varies from 20-10 percent.

Biomedical Markers

There are no specific diagnostic markers of HSP. If present, anaemia is usually a result of gastrointestinal bleeding or associated with acute nephritis. Thrombocytosis is a feature that may be related to disease severity. Renal involvement results in haematuria with or without proteinuria, which may be in the nephrotic range. A rapidly progressive nephritis may result in renal insufficiency and requires histological assessment. Von-Willebrand factor antigen is synthesized by and is present in endothelial cells. High concentrations have been detected in patients with HSP as with other vasculitic diseases probably because of vascular endothelial damage [13-15].

An association with disease activity has a been noted. This may be a useful marker of disease severity. Soluble thrombomodulin is derived from damaged endothelium which may reflect endothelial injury. Raised serum thrombomodulin concentrations have been demonstrated in patients with HSP nephritis [16,17]. Routine coagulation tests are normal while factor XIII activity has been reported to be low, especially in patients with severe gastrointestinal disease [18].

Chronic Kidney Disease (CKD)

Renal involvement rarely precedes the appearance of the purpura and usually occurs within the first 3-weeks of the illness. It ranges from isolated microscopic haematuria, proteinuria, or nephritic-nephrotic syndrome (> 3 g/24 h in adults and > 40 mg/m2/h in children) to acute, rapidly progressive glomerulonephritis. It can be associated with either high blood pressure or renal failure, or both. Depending on the diagnostic criteria, the frequency of renal involvement varies from 20% to 100% [19]. Overall, an estimated 2% of children with HSP experience renal failure.

In one study, the frequency of chronic kidney disease in adults was estimated to be 1% and that of end-stage renal disease to be < 1%. The frequency of renal failure was reported to be 10% to 20% in adults and 1% in children [19].

Retrospective cohort studies revealed some degree of initial renal involvement in about 20–54% of HSP patients, ranging from isolated haematuria and/or proteinuria (without any abnormality in renal function and blood pressure) to acute nephropathy with renal impairment [20]. Table 1 below summarizes the defining features of HSP. Renal manifestations generally occur over a period of 28 days after the initial presentation of HSP. Gross or microscopic haematuria, proteinuria or nephritic syndrome may occur [7,20]. Table 2 below highlights HSP criteria for hospitalization.