Skip to main content

Main menu

  • Home
  • Content
    • Published Ahead of Print
    • Current Issue
    • Podcasts
    • Subject Collections
    • Archives
    • Kidney Week Abstracts
    • Saved Searches
  • Authors
    • Submit a Manuscript
    • Author Resources
  • Trainees
    • Peer Review Program
    • Prize Competition
  • About CJASN
    • About CJASN
    • Editorial Team
    • CJASN Impact
    • CJASN Recognitions
  • More
    • Alerts
    • Advertising
    • Feedback
    • Reprint Information
    • Subscriptions
  • ASN Kidney News
  • Other
    • ASN Publications
    • JASN
    • Kidney360
    • Kidney News Online
    • American Society of Nephrology

User menu

  • Subscribe
  • My alerts
  • Log in
  • My Cart

Search

  • Advanced search
American Society of Nephrology
  • Other
    • ASN Publications
    • JASN
    • Kidney360
    • Kidney News Online
    • American Society of Nephrology
  • Subscribe
  • My alerts
  • Log in
  • My Cart
Advertisement
American Society of Nephrology

Advanced Search

  • Home
  • Content
    • Published Ahead of Print
    • Current Issue
    • Podcasts
    • Subject Collections
    • Archives
    • Kidney Week Abstracts
    • Saved Searches
  • Authors
    • Submit a Manuscript
    • Author Resources
  • Trainees
    • Peer Review Program
    • Prize Competition
  • About CJASN
    • About CJASN
    • Editorial Team
    • CJASN Impact
    • CJASN Recognitions
  • More
    • Alerts
    • Advertising
    • Feedback
    • Reprint Information
    • Subscriptions
  • ASN Kidney News
  • Visit ASN on Facebook
  • Follow CJASN on Twitter
  • CJASN RSS
  • Community Forum

About the Cover

February 08, 2021; Volume 16,Issue 2

Looking for COVID information? All ASN Journal COVID articles are available in the COVID collection. All articles are free.​

Cover image

Cover image expansion

On the Cover

What is the diagnosis?

A 43-year-old woman, with diffuse cutaneous systemic sclerosis on oral steroids for past 6 months, presented with acute onset breathlessness and newly detected accelerated hypertension. On examination, there was skin tightening involving her neck, chest, and upper and lower limbs with hypopigmented lesions over her limbs and oral mucosa. Her BP was 170/100 mmHg with bilateral papilledema. Laboratory evaluation revealed microcytic hypochromic anemia (hemoglobin, 7.7 g/dl). Urinalysis showed 2+ proteinuria with occasional white blood cells, and her spot urine protein-creatinine ratio was 0.9. Serum creatinine was 9.7 mg/dl, sodium and potassium were 141 and 2.8 mmol/L, respectively, and lactate dehydrogenase was 489 IU/L. Her antinuclear antibodies, anti–U1 ribonucleoprotein autoantibodies, anti–Scl-70 (also called anti–topoisomerase I) antibodies, and anti-Ro52 antibodies were positive. Radiologic evaluation showed normal-sized kidneys with preserved corticomedullary differentiation, and the renal blood flow (as detected by renal Doppler study) was normal. She was initiated on hemodialysis, and a kidney biopsy was performed. The patient was treated with angiotensin-converting enzyme inhibitors (20 mg of enalapril per day, which was slowly tapered to 5 mg/day) and thrice weekly hemodialysis. A month later, she developed flash pulmonary edema and passed away.

Image Description:

Left: The biopsy specimen showed severe concentric, myxoid intimal proliferation, giving an “onion-skin” appearance, with almost total obliteration of the vascular lumen. Hematoxylin and eosin stain. Original magnification, ×200.

Right: Shown is the prominent juxtaglomerular apparatus and ischemic wrinkling of the glomerular capillaries. Jones methenamine silver stain. Original magnification, ×400. These biopsy specimen findings are consistent with scleroderma renal crisis.

Teaching Points:

Scleroderma renal crisis occurs in about 5%–10% of patients with diffuse cutaneous systemic sclerosis. It has an abrupt onset with moderate to severe hypertension, AKI, and relatively bland urine sediment with minimal proteinuria. Risk factors include rapidly progressive diffuse cutaneous disease within 5 years of diagnosis, palpable tendon friction rubs, pericardial effusion, and thrombocytopenia. Anti–Scl-70 and anti–RNA polymerase 3 positivity increases the risk. High-dose corticosteroid treatment is also known to increase the risk of scleroderma renal crisis. The histologic picture of scleroderma renal crisis is that of a thrombotic microangiopathy process with myxoid intimal changes, thrombi, onion-skin lesions, and fibrointimal and adventitial sclerosis involving mainly the small vessels. Kidney biopsy helps in confirming the clinical diagnosis and also in excluding superimposed diseases that might lead to AKI. Nearly half of the patients progress to kidney failure. Renin-angiotensin-aldosterone system activation plays a critical role in the pathogenesis of scleroderma renal crisis, and use of angiotensin-converting enzyme inhibitors, along with kidney replacement therapy, helps reduce the mortality rate.

(Images and text provided by Anila Abraham Kurien, Department of Pathology, Renopath, Center for Renal and Urological Pathology, Chennai, India; and Badri Kannan and Gopalakrishnan Natarajan, Department of Nephrology, Madras Medical College, Chennai, India.)

Back to top
PreviousNext

In this issue

Clinical Journal of the American Society of Nephrology: 16 (2)
Clinical Journal of the American Society of Nephrology
Vol. 16, Issue 2
February 08, 2021
  • Table of Contents
  • Table of Contents (PDF)
  • About the Cover
  • Index by author
Sign up for alerts
View Selected Citations (0)

Podcast

Subscribe to podcast
Download MP3

Jump to

  • Patient Voice
  • Editorials
  • Original Articles
    • Clinical Nephrology
    • Cystic Kidney Disease
    • Genetics
    • Glomerular and Tubulointerstitial Diseases
    • Maintenance Dialysis
    • Transplantation
  • Research Letters
  • Erratum
  • Genomics of Kidney Disease
  • Kidney Case Conferences: How I Treat
  • Perspectives
  • Features
  • Most Read
Loading
  • Drug-Induced Acute Kidney Injury
  • The Intensivist's Perspective of Shock, Volume Management, and Hemodynamic Monitoring
  • Low-Flow Acute Kidney Injury
  • Classification of Uremic Toxins and Their Role in Kidney Failure
  • Sepsis Management for the Nephrologist
More...

Articles

  • Current Issue
  • Early Access
  • Subject Collections
  • Article Archive
  • ASN Meeting Abstracts

Information for Authors

  • Submit a Manuscript
  • Trainee of the Year
  • Author Resources
  • ASN Journal Policies
  • Reuse/Reprint Policy

About

  • CJASN
  • ASN
  • ASN Journals
  • ASN Kidney News

Journal Information

  • About CJASN
  • CJASN Email Alerts
  • CJASN Key Impact Information
  • CJASN Podcasts
  • CJASN RSS Feeds
  • Editorial Board

More Information

  • Advertise
  • ASN Podcasts
  • ASN Publications
  • Become an ASN Member
  • Feedback
  • Follow on Twitter
  • Password/Email Address Changes
  • Subscribe to ASN Journals
  • Wolters Kluwer Partnership

© 2022 American Society of Nephrology

Print ISSN - 1555-9041 Online ISSN - 1555-905X

Powered by HighWire