Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease resulting in dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition

Khalil, Muhammad Abdul Mabood; Iftikhar, Nadia; Hussain, Syed Ather; Tan, Jackson

doi:10.1007/s13730-012-0036-z

CEN Case Rep

2012

DOI: 10.1007/s13730-012-0036-z

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Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease resulting in dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition

Muhammad Abdul Mabood Khalil

Nadia Iftikhar

Syed Ather Hussain

et al.

Abstract: Mixed connective tissue disease

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Cited by 3 publications

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“…Summary of Previous Case Reports of MCTD and SRC and Their Outcomes4,13,[14][15][16][17][18] . mild ischemic changes, no deposits, segmental lesions or crescents.…”

mentioning

confidence: 99%

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

Cheta

Rijhwani

Rust

2017

Journal of Investigative Medicine High Impact Case Reports

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Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity. In this article, we report a rare case of scleroderma renal crisis in a patient with MCTD who we treated with plasma exchange for clinical suspicion for an underlying thrombotic thrombocytopenia and mycophenolate mofetil for MCTD. The patient had multiple poor prognostic indicators yet made a full renal recovery in less than 3 months.

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“…Summary of Previous Case Reports of MCTD and SRC and Their Outcomes4,13,[14][15][16][17][18] . mild ischemic changes, no deposits, segmental lesions or crescents.…”

mentioning

confidence: 99%

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

Cheta

Rijhwani

Rust

2017

Journal of Investigative Medicine High Impact Case Reports

View full text Add to dashboard Cite

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

Madieh

Khamayseh

Hrizat

et al. 2021

Case Reports in Nephrology

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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.

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A 63-year-old Chinese female with mixed connective tissue disease who suffered from renal crisis

Shao,

Liu,

Guo

et al. 2024

Eur J Inflamm

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Mixed connective tissue disease (MCTD) is a rare autoimmune disorder. We present a case of a Chinese female who has been admitted to our hospital on eight separate occasions. Her initial symptoms involved internal organs including the lung and heart. Due to the presence of anti-U1RNP antibodies and other clinical features, a diagnosis of MCTD was considered. Throughout her first to seventh admissions, her serum creatinine levels remained normal. During her eighth hospitalization, her renal function deteriorated rapidly, culminating in renal crisis. Chest computed tomography (CT) confirmed pulmonary edema, necessitating hemodialysis. Her interleukin (IL)-6 level increased significantly during throughout hospitalizations. The patient responded well to treatment with an IL-6 receptor antagonist (IL-6RA). This case raises the question of whether IL-6 could serve as a potential biomarker for predicting renal crisis incidence in MCTD patients, a topic warranting further investigation in the future.

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Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease resulting in dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition

Abstract: Mixed connective tissue disease

Cited by 3 publications

References 21 publications

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

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

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

A 63-year-old Chinese female with mixed connective tissue disease who suffered from renal crisis

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