Paediatric Vasculitis Activity Assessment Training: Pvasadvanced Training Cases 6-10Homework
Paediatric Vasculitis Activity Assessment Training: PVASAdvanced Training Cases 6-10Homework II
Case 06
On assessment in the clinic today a 10-year-old boy presents for the first time to you with a six month history of sudden onset of deafness in the left ear. He has never been seen in hospital before. In the last four months he has lost about 2 kg from his previous weight of 40 kg and has experienced sweating attacks associated with a temperature of 40C, with no evidence of infection. One month ago he developed a chronic cough and chest x-ray revealed widespread pulmonary infiltrates. There was no haemoptysis. At the same time his mother noticed a purpuric rash on both of his legs. Two days before admission he suddenly lost control of his legs and has been incontinent of urine and faeces. He says he cannot feel his legs and is unable to walk. Examination showed sensorineural hearing loss, skin purpura and a sensory level at Th10 with poor anal tone and weakness of upper motor neurone distribution affecting both legs, with bilateral up going plantars. A lung biopsy confirmed the presence of granulomatous inflammation. MRI of the thoracic cord shows oedema in the cord compatible with transverse myelitis. CSF examination shows elevated protein but no infection. A diagnosis of non-renal GPA (Wegener’s) was made.
Please score his PVAS and BVAS/WG for this visit.
Case 07
On assessment in the clinic today a 14-year-old girl presents for the first time to your hospital with a six month history of nasal crusting, a four month history of polyarthritis affecting her feet followed a month later by development of a purpuric rash on both legs leading to an ulcer over the left medial malleolus, both of which are still present. Two weeks ago she developed headaches, grand mal seizures and a right hemiparesis. At the same time she experienced visual blurring and was found to have retinal vasculitis with haemorrhages in both eyes. A nasal biopsy confirmed the presence of granulomatous inflammation. The patient was c-ANCA positive and was thought to have non-renal GPA (Wegener’s) with CNS disease.
Please score her PVAS and BVAS/WG for the most recent visit.
Case 08
On assessment in the clinic today a 13-year-old girl presents to you for the first time with a six-month history of purpuric skin lesions on both legs, associated with necrotic ulceration around the right ankle. One month ago she developed a right foot drop and has started dragging her shoe when she walks. There are no obvious precipitating causes. Cutaneous biopsy shows a leukocytoclastic vasculitis. For the past three months she has noticed a constant blood stained nasal discharge and frequent golden/red crusts from both nostrils. She also complains of increasing pains in her ankles, knees and hands. She is not aware of any fevers. Examination shows a right foot drop, shallow ulcers on the right leg, purpuric lesions on both legs, no arthritis but there is joint line tenderness at ankles, knees and wrists. Urine dipstick is normal, as is renal function. She has received no therapy. A nasal biopsy shows granulomatous infiltrates and she is a c-ANCA positive.
Please score her PVAS and BVAS/WG for this presentation.
Case 09
On assessment at the clinic today, a 16-year-old lady presents for the first time with a two week history of sudden painless loss of vision in both eyes. She also reports chest pain, breathlessness on exertion, and cough with haemoptysis, and finds it difficult to take deep inspirations. She has recently developed mouth ulcers over the previous week and three weeks ago felt tingling and numbness in her feet. Three months ago she started getting recurrent nasal ulcers with crustingaccompanied by epistaxis. The sinuses were tender with x-ray evidence of chronic sinusitis.
Examination reveals a temperature of 39C, inspiratory stridor, retinal haemorrhages and a left sided pleural effusion. There is a pericardial rub. She has a sensory peripheral neuropathy with stocking distribution in both feet. Chest radiographs confirm the presence of a pleural effusion with large bilateral infiltrates, and an arterial blood gas reveals a pO2 of 6kPa, which is hypoxic on room air. The pCO2 is 4 kPa (normocapnic). An ENT examination performed urgently reveals 90% subglottic narrowing with granulation tissue, a sample of which is diagnostic of GPA. She requires a tracheostomy, ventilation, and immunosuppressive therapy. Nasal examination confirms the presence of nasal ulceration and a biopsy from an edge shows granulomatous inflammation. After extubation, she complains of a fullness in her vulval region. Biopsy of a palpable nodule reveals necrotizing small vessel arteritis, with granulomatous features.
Please score her PVAS and BVAS/WG for the current presentation.
Case 10 a, b
A 14-year-old girl (weight at presentation 50 kg) on assessment in the clinic today presents with a four week history of fever of 39C and weight loss of 3kg. She has arthritis with some small joint swelling affecting the MCP and PIP joints of both hands. Within the last ten days she has developed acute abdominal pain with bloody diarrhoea and three of her fingers have become blackened and cold. Examination reveals a tender acute abdomen, pyrexia of 39°C, generalised arthritis and multiple digit gangrene affecting the tips of her left index and right ring and little fingers. She is seen by the surgeons, who suspect a large bowel perforation and this is confirmed at laparotomy. She has peritoneal fluid and this is culture positive to E coli. Her appendix is acutely inflamed as is the caecum and she is given a defunctioning colostomy at operation. The resected section of bowel shows changes of a medium vessel vasculitis affecting the caecum and the appendix. She is negative for hepatitis B and C and a diagnosis of classical polyarteritis nodosa is made. After the colostomy she is given treatment.
Please score her PVAS and BVAS/WG for this presentation (a).
Eighteen months later she attends the clinic for a follow-up visit. She has been managed with cyclophosphamide and prednisolone and then switched to azathioprine and prednisolone. Tiredness has become a constant feature of her condition in the last year. She has lost a lot of time off her schooling (she has had to give it up temporarily). She has intermittent episodes of diarrhoea. She has had her colostomy reversed but never really regained her bowel habit properly since then. She feels very depressed about this since she spends a lot of time going to the toilet at short notice with feelings of urgency and very loose stool. There is no blood; it is all liquid and sometimes undigested matter. Three-weeks prior to the clinic visit she had to have her azathioprine temporarily stopped because her platelet count dipped to 26 and she developed purpuric haemorrhage over her feet. Her azathioprine had been stopped at the time and the platelet count has recovered. At the same time she developed some dysuria and her GP had tested her urine and found it to be positive with blood (++)and protein (+++). An E coli urinary tract infection was discovered and treated with antibiotics for the previous ten days. Unfortunately, this exacerbated her diarrhoea and made her feel even more depressed.
Please assess her PVAS and BVAS/WG for the current visit (b).