A CASE OF CATASTROPHIC CRYOGLOBULINAEMIC VASCULITIS: MICROANGIOPATHIC HAEMOLYTIC ANAEMIA, RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS, PARAPARESIS AND STROKE

E BONGETTI1, D THARMARAJ1, E RICE1

1Monash Medical Centre, Clayton, Australia

Background: Cryoglobulinaemia is characterised by abnormal immunoglobulin causing injury via precipitation at lower temperatures. Type II and III cryoglobulinemia involve the rheumatoid factor. We present a case of cryoglobulinaemia presenting with neurological deficits, renal impairment, microangiopathic haemolytic anaemia and rapid clinical deterioration.
Case Report: A 69-year-old woman with a background of renal cell carcinoma with partial nephrectomy presented to hospital with sudden onset lower limb paraplegia and paraesthesia. Magnetic resonance imaging of the spine demonstrated an intramural lesion at the level of T10 and subarachnoid haemorrhage.
Initial investigations showed a creatinine of 224 µmol/L, thrombocytopenia (platelets 66 x109/L [150-450]), haemolytic anaemia with schistocytes on blood film, low haptoglobin (<0.5 g/L [0.36-1.95]), high LDH (532 U/L [120-250]), and ADAMTS13 of 76%. Her autoimmune panel revealed ANA >1280 (units) speckled, elevated rheumatoid factor (853 kIU/L [0-20]), IgM kappa monoclonal gammopathy 2g/L, suppressed complements (C3 0.61 g/L [0.8-1.5], C4 <0.01 g/L [0.16-0.38]), anti-Ro52 positive, and negative hepatitis serology. Preliminary renal biopsy results showed membranoproliferative glomerulonephritis and high dose steroids were commenced. She developed new delirium and progressive lower limb weakness and underwent urgent laminectomy. Her cryoglobulins returned positive (6% [0-0.5%]) for monoclonal IgG Kappa cryoglobulin, monoclonal IgM Kappa cryoglobulin and polyclonal IgG cryoglobulin. A formal renal biopsy report returned with cryoglobulinaemic vasculitis and features of thrombotic microangiopathy (TMA). She received renal replacement therapy and daily plasma exchange for five sessions. Despite resolution of kidney injury, she had poor neurological recovery and repeat neuroimaging showed multiple cerebral infarcts. Active treatment was subsequently ceased.
Conclusion: Cryoglobulinaemia should be considered early in the clinical course in patients with rapidly progressive glomerulonephritis on the renal biopsy, TMA and high positive rheumatoid factor.


Biography:
Elisa Bongetti is a second year advanced trainee working at Monash Medical Centre in Melbourne.

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