Paeds Cases · rheumatology-musculoskeletal-and-sports
Counsel a family on a new diagnosis of IgA vasculitis and the renal monitoring — OSCE
OSCE communication and safety-netting station: explaining a new diagnosis of IgA vasculitis (Henoch-Schonlein purpura) to a five-year-old boy and his parents, addressing the diagnosis, the typical course, the prognosis, the role of the corticosteroids, and the critical importance of the blood pressure and urinalysis monitoring for at least six months.
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Candidate brief
You have eight minutes to counsel the parents of a five-year-old boy who has just been diagnosed with IgA vasculitis (Henoch-Schonlein purpura), following the overnight appearance of a palpable purpuric rash on his lower legs and ankles, some knee pain, and a tummy ache. The platelet count and coagulation are normal, and the urinalysis shows microscopic haematuria with trace protein. The parents have read online that the disease causes kidney failure and that steroids are needed, and they are frightened. Use a structured, honest, empathic approach that names the diagnosis, explains the typical course, addresses the online fears, and builds a clear monitoring and safety-netting plan. [2][4]
Key teaching and communication objectives
Acknowledge and validate the parents' fear before delivering the information, and allow the silence. Explain in the plain language that IgA vasculitis, also called Henoch-Schonlein purpura, is the commonest condition of its kind in children, that it follows an infection like the sore throat he had last week, and that it causes the small blood vessels in the skin, the joints, the tummy and the kidneys to become a little inflamed and leaky. The rash they see is the leak in the skin, the knee pain is the leak in the joints, and the tummy ache is the leak in the gut. [2]
Address the kidney-failure fear honestly without minimising it. Explain that the kidneys are affected in about a third to a half of children with this condition, usually as a small amount of blood or protein in the urine, which is what the test has already shown. Reassure them that the great majority of children recover fully within a few weeks, and that the serious kidney problems are uncommon, occurring in only a small percentage. The reason the team is taking it seriously, and the reason for the monitoring plan, is that the kidney involvement can appear or change over the weeks and months after the rash, even when the child looks completely well, so the monitoring is how the team catches it early. [2][3]
Address the steroid question directly. Explain that the steroids are not needed for most children, because the body clears the inflammation on its own, and that the research has shown that the steroids do not reliably prevent the kidney involvement. The steroids are reserved for the children with the severe tummy pain, the severe joint pain that does not settle with the simple painkillers, the severe swelling of the scrotum, or the severe kidney involvement. This boy's tummy ache and knee pain will be managed with the simple painkillers and the rest, and the steroids will be considered only if the symptoms become severe. [2][4]
Lay out the monitoring plan clearly, because it is the heart of the safety-netting. The blood pressure and the urine test will be checked every week for the first four to six weeks, and then every month for six months. The parents are given the clear instructions to bring him back straight away if he develops the reduced urine, the swelling of the face or the legs, the severe tummy pain, the vomiting, or the headache. Close with the reassurance that the outlook is excellent, that the team will watch the kidneys carefully, and that a named contact and the next appointment are arranged before they leave. [2][4]
References
- [1]Ozen S, Pistorio A, Iusan SM, et al EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria Ann Rheum Dis, 2010.PMID 20413568
- [2]McCarthy HJ, Tizard EJ Clinical practice: Diagnosis and management of Henoch-Schönlein purpura Eur J Pediatr, 2010.PMID 20012647
- [3]Davin JC, Coppo R Henoch-Schönlein purpura nephritis in children Nat Rev Nephrol, 2014.PMID 25072122
- [4]Ozen S, Marks SD, Brogan P, et al European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative Rheumatology (Oxford), 2019.PMID 30879080