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Diagnosis: Scleroderma Renal Crisis
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a mortality of 20% at 6 months. Once the leading cause of mortality in Scleroderma (SSc), it remains a serious complication, often necessitating level three care for patients affected. Whilst renal outcomes have significantly improved following the advent of angiotensin-converting enzyme inhibitor (ACEi) therapy, SRC remains a precarious challenge for clinicians, due to lack of preventative measures and the fact that patients can rapidly decline despite best medical management. SRC arises from reduced blood supply to the kidney which is proposed to occur in susceptible individuals for a variety of reasons. Susceptible individuals with SSc have the unifying abnormal intra-renal features of vasculopathy, fibrosis, and autoimmunity which allow injury to the vessel wall to initiate an amplification loop of local damage and activation of the renin-aldosterone-angiotensin (RAA) axis and hence SRC. The overall pathological picture can be characterized by endothelial damage and thrombus formation. SRC demonstrates predominant small vessel involvement with early changes such as mucoid intimal oedema, thrombosis, and fibrinoid necrosis with later intimal thickening leading to obliteration of the lumen which gives an ‘onion skin’ appearance under microscopy. Adventitial and peri-adventitial fibrosis is also observed which indicates a chronic vasculopathy process. In extensive histological studies of SRC, it has been found that the extent of acute vascular injury, glomerular ischaemic collapse, and C4d deposits are linked to delayed recovery or failure to recover renal function (Cole A, et al. Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis. Clin Rev Allergy Immunol. 2023;64(3):378-391. [PubMed link]).
Los hallazgos son similares a los que se encuentran en el compromiso renal por emergencia hipertensiva ("hipertensión maligna") de otras causas.
See the chapter: Vascular Diseases of our Tutorial.
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References
- Cole A, Ong VH, Denton CP. Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis. Clin Rev Allergy Immunol. 2023;64(3):378-391. [PubMed link]
- Azoulay LD, Mathian A, Hekimian G, Schmidt M, Luyt CE, Amoura Z, Combes A, Pineton de Chambrun M. Scleroderma cardiac crisis and scleroderma renal crisis: Two sides of the same coin? Autoimmun Rev. 2022;21(11):103179. [PubMed link]
- Gigante A, Leodori G, Pellicano C, Villa A, Rosato E. Assessment of kidney involvement in systemic sclerosis: From scleroderma renal crisis to subclinical renal vasculopathy. Am J Med Sci. 2022;364(5):529-537. [PubMed link]
- Foocharoen C, Tonsawan P, Pongkulkiat P, Anutrakulchai S, Mahakkanukrauh A, Suwannaroj S. Management review of scleroderma renal crisis: An update with practical pointers. Mod Rheumatol. 2023;33(1):12-20. [PubMed link]
- Gigante A, Rosato E. Reduced glomerular filtrate rate, end-stage renal disease, scleroderma renal crisis, and risk of mortality in patients with systemic sclerosis. Scand J Rheumatol. 2022;51(3):246-247. [PubMed link]
- Hudson M, Ghossein C, Steen V. Scleroderma renal crisis. Presse Med. 2021;50(1):104063. [PubMed link]