Children & Young People s Directorate. Paediatric-Neonatal Guidelines Checklist & Version Control Sheet

Transcription

1 Children & Young People s Directorate Paediatric-Neonatal Guidelines Checklist & Version Control Sheet 1 Name of Guideline / Policy/ Procedure Management of Hench Schonlein Purpura (HSP) Purpose of Procedure/ Guidelines/ Protocol: Guideline for the Management of Hench Schonlein Purpura (HSP 3 Replaces: 4 Professionals consulted during development Dr Shilpa Shah, Paediatric Consultant Dr Ben McNaughten ST3 Paediatrics Dr Micheal Taylor ST1 Paediatrics Circulated to all Consultant Paediatricians 5 Applicable to which staff: Consultant Paediatricians/Paediatricians/Nursing Staff/Clinical Staff 6 Name & Title of Author: Dr Shilpa Shah, Paediatric Consultant Dr Ben McNaughten ST3 Paediatrics Dr Micheal Taylor ST1 Paediatrics 7 Proposals for dissemination: Southern Trust Web Portal 8 Proposals for implementation: n/a 9 Training Implications: n/a 10 Date Procedure/Guideline/ Protocol submitted to Procedures Committee: 11 Outcome: Approved Approved/Minor amendments Not approved Deferred Approved by Dr Khan, AMD, Consultant Paediatrician on behalf of the CYP Clinical Governance Oversight Committee Date of CYP SMT approval Comments: 13 Date of approval by Trust SMT (if required): n/a 14 Date for further review (3 year default) February Date added to repository: (Note: Guideline author to complete parts 1-10)

2 CLINICAL GUIDELINES ID TAG Title: Management of Henoch Schonlein Purpura (HSP) Dr Shilpa Shah Paediatric consultant Author: Dr Ben Mcnaughten ST3 Paediatrics Dr Micheal Taylor ST1 Paediatrics Speciality / Division: Directorate: Paediatrics CYPD Date Uploaded: 6/3/18 Review Date Clinical Guideline ID February 2021 CG0539

3 Contents 1. Clinical Features 2. Management of HSP 3. Letter for community children s nurses 4. Appendices Appendix 1: Blood Pressure Centile Charts Appendix 2: Indication for Steroid Appendix 3: Parent Advice Leaflet 5. References This guideline is for use in Southern Health and Social Care Board (CAH/DHH) By the following: PAEDITRICS: ST trainees, GP trainees, F2 trainees, APNP, ward nurses, SAS doctors, Consultants A/E: trainees, nurses, SAS doctors, consultants GP: in southern trust area Peer review and Nephrology opinion has been obtained-draft stage 1

4 CLINICAL FEATURES OF HENOCH SCHONLEIN PURPURA (HSP) SKIN: The typical rash is noted in 95% of the cases. It is classically described as palpable purpuric lesions symmetrically distributed over the lower limbs and buttocks (figures below). It may involve the arms, face and ears but usually spares the trunk. The rash can be preceded by maculopapular or urticarial looking rashes. GASTROINTESTINAL : GI involvement is noted in as many as 50-75% of cases. The most common manifestation of this is a colicky abdominal pain but may present as hematemesis, frank PR bleeding or even occult faecal blood loss. Intussusception is a rare complication JOINTS: Arthralgia presents in approximately 75% of cases and for the most part is non-destructive in nature. RENAL: Renal involvement in HSP occurs in as many as 60% of cases. It generally presents as haematuria, proteinuria, nephrotic syndrome, renal impairment and hypertension. In 97% of cases this presents in the first 3 months. There are rare cases where it may manifest years after the initial presentation. If the renal manifestations do not resolve general Paediatric referral is mandated as well as involvement of the Paediatric Nephrologists. UROLOGICAL: Orchiditis is a relatively common finding reported in up to 24%, HOWEVER IF TORSION CANNOT BE RULED OUT ON CLINICAL GROUNDS UROLOGY INVOLVEMNT IS ESSENTIAL NEUROLOGICAL AND PULMONARY are rare DIFFERENTIAL DIAGNOSIS 1. Meningococcal Disease 2. Immune Thrombocytopenic Purpura 3. Clotting disorders (everything from Von Willebrand to Factor IX,Wegners Granulomatosis) 4. ANCA Positive Vasculitis 5. Acute Haemorrhagic Oedema of infancy 6. SLE

5 MANAGEMENT OF HSP Clinical suspicion of HSP Investigations 1. Bloods a. FBP b. U&E c. CRP d. Bone profile e. Coag Screen 2. Blood Pressure 3. Urinalysis Please provide 1 bottle of urine dipsticks to patient for CCN team as appropriate 1) Inform the named consultant No Renal Involvement - discharge 2) Arrange Community children s nurse/ssau/apu/paw follow up as follows if normal results Week 1 to 4: weekly blood pressure and urinary dipsticks. Week 5 to 12: fortnightly blood pressures and urinary dipsticks Week 12 onwards: six monthly Microscopic haematuria can persist for many years. Seek paediatric advice If required repeat the initial investigations and consider the following Refer to general paediatrician if hypertension/macroscopic haematuria/2+ or more proteinuria on any two occasions or if any concerns regarding oedema or oliguria or any of the investigations abnormal. 1. Immunoglobulins 2. Autoimmune Profile 3. Complement 4. Renal USS 5. early morning urinary protein:creatinine ratios Discuss with the nephrology team in RBHSC if: 1. Hypertension 2. Abnormal Renal function 3. Macroscopic haematuria 4. Nephrotic/nephritic Syndrome

6 Date / / Re: name/address of patient or insert addressograph Dear Short stay assessment Unit STH, DHH, CAH / community paediatric nurse. As per our telephone call on I am enclosing a proposed renal surveillance plan. This child/young person s Consultant Paediatrician is.. The above child has been diagnosed with Henoch Schonlein Purpura when attending on the / /. Renal involvement could occur within 3 months in 97% of cases. Given the potential for complications to develop we would appreciate your help following these children up. Weeks 1-4 Weekly review (to include B/P and urinary dipstick). Weeks 5-12 Fortnightly review (to include B/P and urinary dipstick). Week 12 onwards 6 monthly for 1 year review (to include B/P and urinary dipstick). Please refer to general paediatrician if hypertension/macroscopic haematuria/2+ or more proteinuria on 2 separate urine samples or if any concerns regarding oedema or oliguria. Recurrence of episodes of macroscopic haematuria may occur following upper respiratory infections. The prognosis generally remains good unless there is significant proteinuria. Microscopic haematuria can persist for many months or years. Please seek paediatric advice in these situations Your help is much appreciated in the long term surveillance of this condition. Yours sincerely Name/grade/ also add name of consultant in charge of patient (Please attach appendix 1 with this letter on discharge

7 His/her ACCEPTABLE BP CENTILES ARE (WITHIN 90 TH CENTILE FOR AGE ARE) SYSTOLIC DIASTOLIC DATE AND WEEK BP URINE DISPTICK ACTION/COMMENT WEEK1 WEEK 2 WEEK 3 WEEK 4 WEEK 6 WEEK 8 WEEK 10 WEEK 12 AT 6 MONTHS AT 1 YEAR Appendix 1: Blood Pressure Centile Charts

8 Please remember blood pressure centiles are calculated by use of age and height and therefore it is mandatory that a height is taken prior to the use of these charts. It would also be helpful to our GP colleagues if we could send a print out of these charts along with the advice letter.

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12 Appendix 2: Steroid Therapy Although most studies show that glucocorticoid therapy shortens the duration of abdominal pain in patients with HSP, there is little evidence to suggest that glucocorticoids otherwise impact the clinical course. Glucocorticoids may be considered in patients with severe disease affecting their ability to ambulate and perform activities of daily living and/or those who require hospitalisation. Initiation of glucocorticoid therapy should be a consultant led decision. Prednisolone may be used for oral therapy (1 to 2 mg/kg per day, maximum dose of 60 to 80 mg per day). In patients who cannot tolerate oral medications intravenous Methylprednisolone (0.8 to 1.6 mg/kg per day, maximum dose of 64 mg per day) may be used. Steroids should be weaned slowly, typically over four to eight weeks.

13 Appendix 3: Parent Information Leaflet What is Henoch-Schonlein Purpura(HSP)? Henoch Schönlein purpura (HSP) is a disease where small blood vessels called capillaries become inflamed and damaged, producing a rash on the skin called purpura. It develops because of an abnormal reaction of the body defence (immune) system. It is not clear exactly what causes this reaction but it is thought that something acts as a trigger for HSP. For example, the trigger may be a particular infection or certain medicines, such as certain antibiotics. How does Henoch-Schonlein Purpura(HSP) present? 1.Rash 95%. These are usually small, round, red spots and areas of reddishpurple skin discolouration. The rash usually takes about 10 days to fade but could take longer. 2. Joint pains 75% of people with HSP develop inflammation of their joints. The inflammation will gradually clear over time and there is not any lasting damage to the joints. 3. Tummy (abdominal) pain- Most but not all people with HSP develop pain in their abdomen. Abdominal pain tends to come on about a week after the rash has developed in most cases. Are there any complications? 1. Kidney involvement - 60% of children with HSP have kidney involvement: most within the first few months. In the majority of children, it will get better on its own, however 33% will re occur. Less than 5% have any long term complications 3. Intussusecption of the bowel uncommon. Part of the intestine folds into another section of intestine, similar to the way the parts of a collapsible telescope slide into one another, if your child develops severe abdominal pain please let the paediatric team know. 4. Orchitis - inflammation of the testis, causing pain, redness and swelling of the scrotum. However should your child develop any testicular pain he should be assessed immediately by a doctor. 5. Very rarely: other organs like the brain, heart and lung can be affected

14 What tests do we do? Henoch-Schönlein purpura (HSP) is usually suspected because of the typical symptoms, it tends to be a clinical diagnosis, however we may do some tests to help us monitor the condition, or exclude others which may present similarly. 1. Blood tests 2. Urine dipstick 3. Blood pressure What is the treatment for Henoch-Schönlein purpura (HSP)? For most people, HSP will get better of its own and so no specific treatment is needed, however we do recommend the following for symptoms. 1. Pain relief - these may help with joint pains. Paracetamol and Ibuprofen tend to be very helpful. However ibuprofen may be avoided if there are any kidney complications or bleeding in the gut. 2. Rest - resting with the legs raised may help reduce the degree of rash that develops. 3.Steroid medication these are only for very specific circumstances and will be discussed with a senior clinician. What is the outcome of Henoch-Schönlein purpura (HSP)? HSP generally recovers by itself with NO major medical intervention. It is followed up for at least a period of six months to a year to ensure there are no relapses or kidney involvement in this period. Around one third of children have symptoms for <14 days, one third for 2-4 weeks, and one third >4 weeks. However, 33% of children have relapses within 6 months, and this tends to be more common in those with kidney involvement. Where can I get more information? REFERENCES

15 Tizard EJ. Hamilton-Ayres MJJ. Henoch-Schönlein purpura. Arch Dis Child Educ Pract Ed. 2008;93:1 8. Weiss PF et al. Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review. Pediatrics. 2007;120(5):1079 Leung SP Use of intravenous hydrocortisone in Henoch-Schonlein purpura J Paediatric Child Health. 2001;37(3):309. Szer IS Gastrointestinal and renal involvement in vasculitis: management strategies in Henoch-Schönlein purpura. Cleve Clin J Med. 1999;66(5):312 Dudley J, Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Arch Dis Child Oct;98(10): Epub 2013 Jul 11