ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: TH-PO561

Scleroderma-Associated Kidney Disease: A Case Series

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • O'Sullivan, Tara, University College Cork, Cork, Cork, Ireland
  • O'Donoghue, Darragh, University College Cork, Cork, Cork, Ireland
  • Redmond, Andrew Johns, University College Cork, Cork, Cork, Ireland
  • Ryan, John G., University College Cork, Cork, Cork, Ireland
  • Moran, Sarah Margaret, University College Cork, Cork, Cork, Ireland
  • Gleeson, James, University College Cork, Cork, Cork, Ireland
  • Ahmed, Adeel Rafi, University College Cork, Cork, Cork, Ireland
Introduction

Scleroderma is a rare multisystem disorder categorised into limited and diffuse subtypes. Recognised renal manifestations of scleroderma include renal crisis (SRC) and ANCA associated vasculitis. We present three differing cases of scleroderma associated renal disease.

Case Description

Case 1: 64 year old normotensive female with systemic sclerosis developed AKI and microangiopathic haemolytic anaemia. ANCA and ANA were negative. IgG Kappa paraprotein detected (9.7g/L). Bone marrow biopsy showed 7.2% plasma cells. Renal biopsy demonstrated intraglomerular thrombotic microangiopathy, without onion-skinning or involvement of interlobular or arcuate arteries. Treatment was targeted at the plasma cell clone.
Case 2: 53 year old female with CREST presented with hypertension (BP 231/124mmHg) and developed AKIN3. She was anti-centromere positive. Renal biopsy demonstrated thrombotic microangiopathy, with onion-skin appearance of arterioles. She was treated as SRC with captopril, leading to improvement in renal function.
Case 3: 75 year old female with limited systemic sclerosis admitted with nephrotic syndrome. She was hypertensive but did not tolerate RAAS inhibition. Urinary PCR was 873.6mg/mmol. Renal biopsy demonstrated a cellular form of focal segmental glomerulosclerosis, without tip lesions. Corticosteroids were avoided due to risk of precipitating SRC. She was treated with tacrolimus, which reduced proteinuria to the sub-nephrotic range.

Discussion

These cases demonstrate three different presentations of scleroderma associated renal disease. The intraglomerular distribution of TMA in Case 1 is more consistent with MGRS rather than SRC, in the context of a paraprotein. While there is one published case of SRC in a patient with concurrent scleroderma and monoclonal gammopathy, SRC is less frequent in seronegative scleroderma. SRC presenting in CREST, as in case 2, is unusual as it is more commonly reported with diffuse scleroderma, and anti-centromere antibodies have a negative association. In the final case, primary FSGS is a highly atypical manifestation of limited systemic sclerosis. The contrasting pathologies highlight the diagnostic value of kidney biopsy in scleroderma patients presenting with renal impairment, and its importance in guiding treatment.