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Scleroderma Renal Crisis

Scleroderma Renal Crisis. Mathini Jayaballa Renal Advanced Trainee. Scleroderma Overview. Uncommon F>M, peak onset 3 rd - 5 th decade Uncontrolled accumulation of collagen and other CT proteins which leads to fibrosis in the skin and other visceral organs Widespread vascular lesions

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Scleroderma Renal Crisis

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  1. Scleroderma Renal Crisis Mathini Jayaballa Renal Advanced Trainee

  2. Scleroderma Overview • Uncommon • F>M, peak onset 3rd - 5th decade • Uncontrolled accumulation of collagen and other CT proteins which leads to fibrosis in the skin and other visceral organs • Widespread vascular lesions • Classification • Localised Scleroderma • Systemic Scleroderma – Limited and Diffuse

  3. Scleroderma Renal Crisis • Renal involvement 50%, usually mild: • proteinuria • mild elevation in Cr • HT • Severe renal disease 10-20% • diffuse cutaneousSSc >> LcSSc

  4. Clinical Features of Scleroderma Renal Crisis (SRC): • Occurs early – within 5 yrs • Can be the initial presentation SRC: • Progressive ARF • Abrupt onset mod-severe HTN • Urine sediment – usually bland

  5. Normal Renal Biopsy (H&E stain)

  6. SRC Renal Biopsy

  7. Normal SRC Renal Angiogram

  8. Risk Factors for SRC • Diffuse or advancing skin involvement • Glucocorticoid >15mg/day • Large joint contractures • New cardiac conditions • New onset anemia • Anti-RNA polymerase or fine speckled ANA pattern • Decreased prevalence of anti-centromereAb

  9. Characteristic Findings • New onset BP > 150/85 • Decline in renal function • New proteinuria +/- hematuria • Retinal changes of malignant hypertension • Flash pulmonary edema • MAHA +/- thrombocytopenia • CNS involvement - seizures

  10. Differentials: • TTP/HUS • ANCA-associated crescentic GN • D-penicillamine-related GN

  11. SRC Management • Untreated  ESKD 1-2mths, death in 1 yr • Prompt & aggressive BP control is mainstay of Rx • reduce BP <72hrs • ACE-I is 1st choice • Better renal function recovery & improves survival • Resistant / Malignant HTN – Add IV agent • Long term, low-dose ACE-I even if BP controlled • 10% normotensive Steen Ann Intern Med 1990; 113:352. Helfrich DJ, Arthritis Rheum 1989; 32:1128.

  12. SRC Progress • 20-50% progress to ESKD despite ACE-I • Inferior 5YS DSSc on dialysis: • 40% with SRC • 90% w/out SRC • Survival SSc on dialysis is worse than others • Delayed renal recovery possible • can take up to 18m Penn H, QJM 2007; 100:485. Abbott KC, J Nephrol 2002; 15:236.

  13. Transplantation Incident patients ANZ 2006-2010 (ANZDATA) • Better survival cf dialysis • 3YS 80% vs 55% • Worse survival than other primary diseases • Risk factors for recurrence/damage to allograft: • progressive skin thickening • new onset anemia • cardiac complications • Strategies to reduce recurrent disease • Avoid CNI, high dose steroids • Continue ACE-I indefinitely Gibney Am J Transplant 2004; 4:2027.

  14. PDGF PDGF Impaired angiogenesis / cell division PDGF Imatinib in Systemic Sclerosis • A protein tyrosine kinase inhibitor • Interferes with the signaling PDGF, TGF-β • Limited data on use in SSc or its effect on renal function

  15. Imatinib in Systemic Sclerosis • Case Report - Rx of refractory DcSSc • Improvement in skin thickening, physical function, FVC • Within 3m, maintained at 9m • Spiera et al - open-label prospective study • 30 patients with DcSSc, no controls • Improvement in skin thickening, FVC after 12m • Pope et al – double-blind RCT, proof-of-concept pilot study • Single center, active DcSSc • Only 10 pts enrolled • Early termination: poor tolerability, AE++ • No benefit in skin thickening, CRP/ESR, global assessment at 6m Sfikakis PP,Rheumatology (2008) 47 (5): 735-737. Spiera RF, Ann Rheum Dis 2011; 70:1003.

  16. Take home messages about SRC: • Rare but high M&M • Mainstay of Rx is BP control with ACE-I • High rate of progression to ESKD • Delayed renal recovery possible • Renal transplant should be considered

  17. References • Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med 1990; 113:352. • Helfrich DJ, Banner B, Steen VD, Medsger TA Jr. Normotensive renal failure in systemic sclerosis. Arthritis Rheum 1989; 32:1128. • Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM 2007; 100:485. • Abbott KC, Trespalacios FC, Welch PG, Agodoa LY. Scleroderma at end stage renal disease in the United States: patient characteristics and survival. J Nephrol 2002; 15:236. • Gibney EM, Parikh CR, Jani A, et al. Kidney transplantation for systemic sclerosis improves survival and may modulate disease activity. Am J Transplant 2004; 4:2027. • Spiera RF, Gordon JK, Mersten JN, et al. Imatinib mesylate (Gleevec) in the treatment of diffuse cutaneous systemic sclerosis: results of a 1-year, phase IIa, single-arm, open-label clinical trial. Ann Rheum Dis 2011; 70:1003. • Kay J, High WA. Imatinib mesylate treatment of nephrogenic systemic fibrosis. Arthritis Rheum 2008; 58:2543. • Sfikakis PP, Gorgoulis VG, Katsiari CG, et al. Imatinib for the treatment of refractory, diffuse systemic sclerosis. Rheumatology (2008) 47 (5): 735-737

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