SUCCESSFUL TREATMENT OF LIFE-THREATENING MULTIORGAN INVOLVEMENT OF IgA VASCULITIS ASSOCIATED WITH LIPOSARCOMA WITH PLASMAPHERESIS, IVIg AND CORTICOSTEROIDS

 

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SUCCESSFUL TREATMENT OF LIFE-THREATENING MULTIORGAN INVOLVEMENT OF IgA VASCULITIS ASSOCIATED WITH LIPOSARCOMA WITH PLASMAPHERESIS, IVIg AND CORTICOSTEROIDS

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Ken
Chau
Liam Qi liamyqi@gmail.com The Royal Brisbane and Women’s Hospital Metro North Kidney Health Service Brisbane Australia -
Sharad Ratanjee sharad.ratanjee@health.qld.gov.au The Royal Brisbane and Women’s Hospital Metro North Kidney Health Service Brisbane Australia -
Jayna Gill jayna.gill@health.qld.gov.au The Royal Brisbane and Women’s Hospital Metro North Kidney Health Service Brisbane Australia -
Dharmenaan Palamuthusingam dharmenaan.palamuthusingam@health.qld.gov.au The Royal Brisbane and Women’s Hospital Metro North Kidney Health Service Brisbane Australia -
Eoin O'Sullivan eoin.osullivan@health.qld.gov.au The Royal Brisbane and Women’s Hospital Metro North Kidney Health Service Brisbane Australia - QIMR Berghofer Medical Research Institute QIMR Brisbane Australia University of Queensland Institute for Molecular Bioscience St Lucia Australia
Taehyun Kim taehyun.kim@health.qld.gov.au The Royal Brisbane and Women’s Hospital Department of Anatomical Pathology Brisbane Australia -
Ken Chau kennethchau1990@gmail.com The Royal Brisbane and Women’s Hospital Metro North Kidney Health Service Brisbane Australia *
Shaun Chandler shaun.chandler@health.qld.gov.au The Royal Brisbane and Women’s Hospital Metro North Kidney Health Service Brisbane Australia -
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Immunoglobulin A Vasculitis (IgAV) is a systemic vasculitis that is typically self-limiting during childhood but can present with life-threatening organ involvement in adults. Although skin manifestations respond promptly to systemic corticosteroids, treatment of other organ involvement has limited evidence. We present a case of successful treatment of life-threatening IgAV and multiorgan involvement with plasmapheresis, intravenous immunoglobulin (IVIg) and systemic corticosteroids.

A 74-year-old male developed multiorgan biopsy-proven IgAV in the weeks following a total pharyngolaryngectomy with jejunal reconstruction for T4a pharyngeal liposarcoma. Significant background included prior coronary artery bypass graft and pacemaker 1 year ago, dyslipidaemia, type 2 diabetes mellitus, hypertension, and prior limbic encephalitis 11 years ago treated with cyclophosphamide.

he patient developed an acute kidney injury (AKI) 1 month after his surgery with a creatinine of 318μmol/L from a baseline creatinine of 80μmol/L (eGFR of 84mL/min/1.73m2). Urinalysis showed haematuria and proteinuria (urine protein creatinine ratio 100g/mol). Blood pressure was 130/80mmHg. This was in the setting of a proximal jejunum leak and polymicrobial infections requiring multiple surgical washouts. Furthermore, histology revealed an involved surgical margin.

A purpuric rash developed over his hands and feet 6 weeks post-operatively, and a skin biopsy revealed a leukocytoclastic vasculitis pattern with IgA deposition on direct immunofluorescence testing characteristic of IgAV. Corticosteroids were commenced (0.5mg/kg) with a good dermatological response over 7 days; however, creatinine did not improve over 17 days of treatment. C3 was low at 0.79g/L, but other serological glomerulonephritis screen was negative. A kidney biopsy was performed in the context of ongoing active urine sediment, which showed IgAV with significant endocapillary hypercellularity, a focal segmental glomerulosclerosis lesion and a cellular crescent.  The patient subsequently developed significant gastrointestinal (GI) bleeding, manifesting as hematochezia and melena, requiring multiple blood transfusions. The patient deteriorated further with decompensated cardiac failure with acute pulmonary oedema requiring intensive care unit support and haemodialysis. Upper and lower GI endoscopy was discussed but deemed too high-risk to perform given recent jejunal reconstruction and leak. GI involvement of IgA vasculitis was presumed. Cyclophosphamide was considered inappropriate due to prior exposure, malignancy and infection risk. Plasmapheresis with IVIg was initiated. Kidney function improved and dialysis was ceased with a discharge creatinine of 153μmol/L and eGFR of 38mL/min/1.73m2.

This case demonstrates the challenge of managing IgAV in the setting of malignancy and multiple infections, and the successful treatment with plasmapheresis, IVIg and systemic corticosteroids. It is theorised that the mobilisation of the jejunum to reconstruct the pharynx, or the malignancy itself, resulted in galactose-deficient IgA formation and a severe phenotype of IgAV with life-threatening multiorgan involvement of the skin, kidney and possible GI tract.

Kewords