Anirban Sen, Atanu Pal* and Ankit Ray

Department of Nephrology, IPGMER and SSKM, Kolkata, India

*Corresponding Author: Atanu Pal, Department of Nephrology, IPGMER and SSKM, Kolkata, India.

Received: September 13, 2021; Published: September 24, 2021

Abstract

  Systemic sclerosis is an autoimmune disorder involving skin, gastro-intestinal tract, lungs, kidneys and heart and can present with a variety of manifestations like esophageal dysmotility, pulmonary fibrosis, hypertension and renal dysfunction. Scleroderma renal crisis is a dreaded complication of systemic sclerosis presenting as rapidly progressive renal dysfunction and accelerated hypertension. However around 10% of the patients with this condition have normal blood pressure which makes it difficult to diagnose early and have poor outcome. Here we present a case of normotensive scleroderma renal crisis in a 48 year old female, who presented with clinical signs of systemic sclerosis and unexplained renal dysfunction. The diagnosis was made with the help of immunological profile and renal biopsy, but the renal function of the patient did not improve and she remains dialysis dependent.

Keywords: Systemic Sclerosis; Scleroderma Renal Crisis; Normotensive Scleroderma Renal Crisis; Rapidly Progressive Renal Failure; Onion Skinning of Arterioles; Autoantibodies

References

1. Asamoah-Odei Efua. “Scleroderma Renal Crisis in a Normotensive Patient”. Kidney International Reports 4 (2016): 311-315.
2. Akoglu Hadim., et al. “A "silent" course of normotensive scleroderma renal crisis: case report and review of the literature”. Rheumatology International 10 (2009): 1223-1229.
3. Aburizik A., et al. “Mistaken Identity: Normotensive Scleroderma Renal Crisis”. Case Reports BMJ (2014): bcr2013202566-bcr2013202566.
4. Al-Dhaher Firas F., et al. “Determinants of morbidity and mortality of systemic sclerosis in Canada”. Seminars in Arthritis and Rheumatism4 (2010): 269-277.
5. Traub Y M., et al. “Hypertension and renal failure (scleroderma renal crisis) in progressive systemic sclerosis. Review of a 25-year experience with 68 cases”. Medicine6 (1983): 335-352.
6. Ubukata Masamitsu., et al. “Anticentromere Antibody-positive Scleroderma Renal Crisis Requiring Dialysis”. Internal Medicine23 (2018): 3479-3483.
7. Van Den Hoogen Frank., et al. “2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative”. Annals of the Rheumatic Diseases11 (2013): 1747-1755.
8. Sakkas Lazaros I. “New developments in the pathogenesis of systemic sclerosis”. Autoimmunity2 (2005): 113-116.
9. Worda Miriam., et al. “In Vivo Analysis of the Apoptosis-Inducing Effect of Anti-Endothelial Cell Antibodies in Systemic Sclerosis by the Chorionallantoic Membrane Assay”. Arthritis and Rheumatism 9 (2003): 2605-2614.
10. Sakkas Lazaros I., et al. “Mechanisms of Disease: The Role of Immune Cells in the Pathogenesis of Systemic Sclerosis”. Nature Reviews Rheumatology 2 (2006): 679-685.
11. Kobayashi H., et al. “Immunohistological study endothelin-1 and endothelin-A and B receptors in two patients with scleroderma renal crisis”. Clinical Rheumatology5 (1999): 425-427.
12. Penn H., et al. “Scleroderma renal crisis: patient characteristics and long-term outcomes”. QJM: Monthly Journal of the Association of Physicians8 (2007): 485-494.
13. Batal Ibrahim., et al. “Scleroderma renal crisis: a pathology perspective”. International Journal of Rheumatology (2010): 543704.
14. Benz Kerstin and Kerstin Amann. “Thrombotic microangiopathy: new insights”. Current Opinion in Nephrology and Hypertension3 (2010): 242-247.
15. Batal Ibrahim., et al. “Renal biopsy findings predicting outcome in scleroderma renal crisis”. Human Pathology 3 (2009): 332-340.
16. Denton CP. “Renal manifestations of systemic sclerosis--clinical features and outcome assessment”. Rheumatology5 (2008): v54-56.
17. Lopez-Ovejero J A., et al. “Reversal of vascular and renal crises of scleroderma by oral angiotensin-converting-enzyme blockade”. The New England Journal of Medicine 25 (1979): 1417-1419.
18. Steen V D and T A Medsger Jr. “Long-term outcomes of scleroderma renal crisis”. Annals of Internal Medicine8 (2000): 600-603.

Citation

Citation: Anirban Sen., et al. “Scleroderma Renal Crisis with Normal Blood Pressure: A Diagnostic Dilemma Solved by Renal Biopsy". Acta Scientific Clinical Case Reports 2.10 (2021): 52-57.

Copyright

Copyright: © 2021 Anirban Sen., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.