Henoch-Schönlein purpura with intracerebral haemorrhage in an adult patient: a case report
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Henoch-Schönlein purpura is a small vessel vasculitis that affects mainly the skin, joints, gastrointestinal tract and kidneys. The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes. Intracerebral haemorrhage is a rare complication of Henoch-Schönlein purpura that so far has mainly been described in children and young adolescence.
We describe a 42-year-old man with Henoch-Schönlein purpura who developed an acute intracerebral haemorrhage that coincided with a reactivation of his vasculitis and the development of renal failure following discontinuation of steroids. In this patient, both the Henoch-Schönlein purpura and his neurological symptoms were successfully treated with intravenous cyclophosphamide and methylprednisolone, followed by a short course of oral cyclophosphamide and long-term oral prednisolone. His renal function also recovered sufficiently not to require renal replacement therapy.
The management of Henoch-Schönlein nephritis remains unclear, especially in the presence of severe complications such as intracerebral haemorrhage. We describe a successful outcome in such a patient.
KeywordsRenal Replacement Therapy Purpura Intracerebral Haemorrhage Oral Prednisolone Proliferative Glomerulonephritis
protein to creatinine ratio.
Henoch-Schönlein purpura (HSP) is a small vessel vasculitis characterized by IgA1 deposition in the renal mesangium and in the blood vessels. It is seen most frequently in early childhood, although it can occur at any age [1, 2]. It is usually preceded by upper respiratory tract infections, having a peak incidence in the autumn and winter [1, 2]. In most cases it is a self-limiting disorder and tends to resolve within 1 month of presentation, although it can re-occur in a third of cases [1, 2].
HSP affects mainly the skin, joints, gastrointestinal tract and kidneys [1, 2, 3]. The severity of symptoms is usually worse in older patients, who tend to have more frequent skin, joint and renal involvement . The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes . More serious neurological complications are rare and include seizures, cranial or peripheral neuropathies, intracerebral haemorrhage and encephalopathy [1, 3]. We describe a man with HSP who developed an acute intracerebral haemorrhage that coincided with a reactivation of his vasculitis.
Upon review 2 weeks later the patient's rash had returned and he remained hypertensive with a BP of 145/95 mmHg. He was therefore prescribed azathioprine 100 mg and ramipril 1.25 mg daily and the prednisolone was increased again to 40 mg once daily. Unfortunately he was again unable to tolerate the increased dose of steroids due to gastrointestinal side effects and the prednisolone was therefore discontinued.
The cyclophosphamide was discontinued 2 weeks later and the patient was discharged home on oral prednisolone 25 mg once daily. Four weeks after the acute admission he had made an almost complete recovery from the intracerebral haemorrhage apart from some mild dysphasia and his renal function improved enough not to need renal replacement therapy. One year later he remains well and dialysis-independent with a serum creatinine of 295 μmol/l, but has continued taking oral prednisolone 10 mg once daily, a dose which is gradually being reduced. His PCR has improved to 87 and urine dipstick has remained positive for blood 1+ and protein 1+.
Intracerebral haemorrhage is a rare complication of HSP that so far has mainly been described in children and young adolescents [3, 4, 5, 6, 7, 8, 9]. Apart from this case, there is only one other published report in an older patient, to the best of our knowledge . Such patients may develop acute elevations in BP, and this is considered to be the primary mechanism of intracerebral haemorrhage . However, other possible causes include the presence of cerebral vasculitis and the increased risk of haemorrhagic complications seen in patients with HSP. The increased risk of bleeding in HSP has been attributed to reduced levels of factor XIII  and prothrombin . Other reported sites of bleeding in patients with HSP include the gastrointestinal tract, lungs, testicles and bladder . Intracerebral haemorrhage in patients with HSP has been successfully treated in the past with surgical evacuation of the haematoma , steroids  or plasmapheresis if cerebral vasculitis is confirmed by magnetic resonance imaging . The underlying coagulopathy should also be corrected [4, 5].
HSP nephritis may affect as many as 80% of adult patients with HSP and approximately 30% of them will develop chronic kidney disease . Adverse prognostic indicators for progression of HSP nephritis are the presence of crescents on biopsy, more than 1 g of proteinuria per 24 hours and renal impairment on presentation . The optimal treatment of HSP nephritis remains unclear, because of the lack of prospective randomized trials. Intravenous pulse methylprednisolone followed by oral steroids has been shown to be effective in the management of severe HSP nephritis . Other possible treatment regimens for severe HSP nephritis include a combination of corticosteroids with cyclophosphamide, azathioprine or cyclosporin [1, 2].
Intracerebral haemorrhage is a rare complication of HSP that may be caused by acute hypertension, cerebral vasculitis, and the increased risk of bleeding observed in this disorder. Although our patient was severely hypertensive at the time of presentation, both the HSP nephritis and his neurological symptoms were successfully treated with intravenous cyclophosphamide and methylprednisolone, followed by a short course of oral cyclophosphamide and long-term oral prednisolone. His renal function recovered enough not to require renal replacement therapy.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- 10.Lévaif F, Szücs LH, Jászai Z: Cerebral hemorrhage and acute glomerulonephritis in Schonlein-Henoch syndrome in old age. Z Gesamte Inn Med. 1971, 26: 309-311.Google Scholar
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