E029 Intracranial haemorrhage in scleroderma renal crisis; an unusual presentation
Authors: Parker Lucy , Buchanan Neil , Williams Emma L 
Published: April 2024

Background/Aims

Scleroderma renal crisis is a life-threatening complication of systemic sclerosis (SSc), which typically occurs in the first 5 years after disease onset. Several risk factors for development of renal crisis are well recognised, including diffuse skin involvement, presence of RNA polymerase 3 antibodies and preceding use of corticosteroids.

Methods

A 66 year old female 6 years post diagnosis of stable diffuse systemic sclerosis/ polymyositis overlap (PM Scl 100 positive) presented to her GP with a 1 week history of fatigue. She had been seen 2 months earlier by her rheumatologist with stable, mild skin involvement. She had no preceding history of hypertension or other significant co-morbidity.

A month earlier she received antibiotics for a chest infection from which she made a slow but complete recovery. She was found to have shingles and started a course of oral acyclovir. Blood tests revealed acute kidney injury stage 3 (eGFR 10 from baseline 70). However, she deteriorated overnight, waking with headache and disturbed colour vision. This quickly deteriorated to bilateral blindness precipitating admission to the emergency department (ED).

On arrival she was conscious but severely hypertensive with a BP of 240/105. She received ramipril, amlodipine and a GTN infusion with limited efficacy. CT head (CTH) showed a left occipitoparietal bleed. She then experienced a seizure in ED, which was terminated with lorazepam. She was intubated in the post ictal phase. Repeat CTH following the seizure showed a right frontal bleed extending into the subarachnoid space.

Repeat CTH at 48 hours showed progression of the right frontal and left parieto-occipital intracerebral haemorrhages, with evidence of posterior reversible encephalopathy syndrome. Lumbar puncture was normal (VZV negative). Treatment was commenced with intravenous hydralazine. On day 2 of admission she required haemofiltration and subsequently regular renal dialysis.

Results

SSc was diagnosed in 2016, since when her main issue had been severe Raynaud’s phenomenon for which she took sildenafil. Her muscle disease had only ever manifested as a modestly raised CPK, which settled with a brief course of low dose prednisolone at diagnosis. She has never required DMARDs. She had mild reflux symptoms. Skin tightening was noted distally over her fingers, forearms, lower legs and feet. Annual echocardiography showed nothing to suggest developing pulmonary arterial hypertension. With supportive management she has made a remarkable neurological recovery and has returned to her pre-morbid level of function. She remains dialysis dependent at 4 months.

Conclusion

This lady lacked a single recognised risk factor for developing a renal crisis, and this occurred quite unexpectedly following a reassuring clinic visit just 2 months prior to presentation. As rheumatologists we must remain vigilant for features of a renal crisis and avoid being falsely reassured by a low risk profile.

Disclosure

L. Parker: None. N. Buchanan: None. E.L. Williams: None.