Explain a new diagnosis of granulomatosis with polyangiitis and the long-term plan — OSCE

Candidate brief

You have eight minutes to explain a new diagnosis of granulomatosis with polyangiitis to a 14-year-old boy and his parents. He presented with three weeks of bloody nasal discharge, a septal ulcer, a cough and cola-coloured urine. The PR3 (c-ANCA) is positive and the renal biopsy shows a crescentic pauci-immune glomerulonephritis. The induction therapy with rituximab and glucocorticoids is about to start. The parents have read online that the disease causes kidney failure and is lifelong, and the boy is worried about the steroid side effects. Use a structured, honest, empathic approach that names the diagnosis, explains the mechanism in plain language, addresses the online fears and the steroid worry, and builds a clear treatment and surveillance plan. [7][6]

Key teaching and communication objectives

Acknowledge and validate the fear before delivering the information, and allow the silence. Explain in plain language that granulomatosis with polyangiitis is a rare condition in which the immune system, the body's defence, has made a mistake and produced an antibody that attacks the small blood vessels in the nose, the lungs and the kidneys. The nosebleeds and the septal ulcer are the vessels in the nose, the cough is the vessels in the lungs, and the dark urine is the vessels in the kidney filtering unit, the glomerulus. The biopsy has shown this clearly, and the positive antibody test has confirmed it. [7]

Address the kidney-failure fear honestly without minimising it. Explain that the kidneys are inflamed, which is why the urine was dark and the blood test was rising, and that the aim of the treatment is to switch off the inflammation quickly and protect the kidneys. Reassure the family that with the prompt treatment the great majority of children keep good kidney function, but be honest that the disease is serious and that the kidney tests will be watched closely over the coming weeks and months. The reason the team is starting the treatment today, before the kidney function can worsen further, is that the untreated inflammation can damage the kidneys within days. [10][6]

Address the steroid and the immunosuppression worry directly. Explain that the treatment is in two parts. The first part, the induction, uses a medicine called rituximab that removes the cells making the faulty antibody, together with the steroids that calm the inflammation fast. The steroids are given for a limited time and then reduced, and the team will watch for and manage the side effects such as the weight change, the mood, the bone health and the infection risk. The second part, the maintenance, keeps the disease quiet over the longer term to prevent it coming back, which the disease can do, and the boy will be involved in the decisions about his care. Address the infection safety-net and the vaccination, and explain the plan for the bone and the infection protection. [6]

Close with the long-term outlook and the plan. Reassure the family that the disease is treatable, that the kidney function will be watched with the blood tests and the urine tests at the scheduled intervals, and that the boy will be cared for by the paediatric rheumatology and nephrology teams together. Explain that the disease can relapse, which is why the maintenance and the surveillance run for at least a year or two, and that the plan includes the transition to the adult service as he grows older. Leave the family with a named contact, the next appointment, and the clear instructions to return for the fever, the reduced urine, the coughing of blood or the worsening nose. [6][10]