Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 Jan 6:2021:8862405.
doi: 10.1155/2021/8862405. eCollection 2021.

Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors

Jomana Madieh  1 , Iman Khamayseh  1 , Alaa Hrizat  1 , Abdurrahman Hamadah  2 , Kamel Gharaibeh  1
Affiliations
Case Reports

Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors

Jomana Madieh et al. Case Rep Nephrol. .

Abstract

Mixed connective tissue disease (MCTD) is a rheumatic disease syndrome with overlapping features of scleroderma, systemic lupus erythematosus, and polymyositis. An extremely rare but serious complication that can occur in MCTD is scleroderma renal crisis (SRC). There have been different approaches to the treatment of SRC associated with MCTD. We present a case of MCTD with chronic features of Raynaud's phenomenon, dermatomyositis, and thrombocytopenia complicated with acute SRC which showed a great response to ACE inhibitors. Here, we advise the early and aggressive use of ACE inhibitors as soon as SRC is suspected.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no conflicts of interest.

Figures

Figure 1
Figure 1
Serum creatinine trend (blue line) and urine output trend (red line) throughout patient's hospitalization.
Figure 2
Figure 2
Edematous mucoid intimal thickening of interlobular artery (H&E).
Figure 3
Figure 3
Intimal fibrinoid changes in interlobular artery (H&E).
Figure 4
Figure 4
Intimal fibrinoid changes in interlobular artery (trichrome stain). Same interlobular artery is shown in figure 3.
Figure 5
Figure 5
Normal glomeruli (PAS).
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Sharp G. C., Irvin W. S., Tan E. M., Gould R. G., Holman H. R. Mixed connective tissue disease-an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA) The American Journal of Medicine. 1972;52(2):148–159. doi: 10.1016/0002-9343(72)90064-2. - DOI - PubMed
    1. Venables P. J. Mixed connective tissue disease. Lupus. 2006;15(3):132–137. doi: 10.1191/0961203306lu2283rr. - DOI - PubMed
    1. Ortega-Hernandez O.-D., Shoenfeld Y. Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment. Best Practice & Research Clinical Rheumatology. 2012;26(1):61–72. doi: 10.1016/j.berh.2012.01.009. - DOI - PubMed
    1. DeMarco P. J., Weisman M. H., Seibold J. R., et al. Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis & Rheumatism. 2002;46(11):p. 2983. doi: 10.1002/art.10589. - DOI - PubMed
    1. Cheta J., Rijhwani S., Rust H. Scleroderma renal crisis in mixed connective tissue disease with full renal recovery within 3 months: a case report with expanding treatment modalities to treat each clinical sign as an independent entity. Journal of Investigative Medicine High Impact Case Report. 2017;5(4):1–5. doi: 10.1177/2324709617734012. - DOI - PMC - PubMed

Publication types

LinkOut - more resources