ArticleCase reports in nephrology2018
Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab.
Article in Case reports in nephrology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 28 citations in OpenAlex.
- [S2k guidelines on diagnostics and treatment of systemic sclerosis : Full version].Zeitschrift fur Rheumatologie · 2025Review
- Takayasu arteritis-associated refractory hypertension induces nephrotic syndrome through glomerular microangiopathy.CEN case reports · 2025Article
- Article
- Targeting complement dysregulation: eculizumab in scleroderma renal crisis management-a case-based review.Rheumatology international · 2024Review
- Treatable Traits in Systemic Sclerosis.Clinical reviews in allergy & immunology · 2023Review
- Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis.Clinical reviews in allergy & immunology · 2023Review
- Alterations of the Primary Cilia Gene SPAG17 and SOX9 Locus Noncoding RNAs Identified by RNA-Sequencing Analysis in Patients With Systemic Sclerosis.Arthritis & rheumatology (Hoboken, N.J.) · 2023Article
- Article
- Systemic Scleroderma-Definition, Clinical Picture and Laboratory Diagnostics.Journal of clinical medicine · 2022Review
- Scleroderma renal crisis: Case reports and update on critical issues.European journal of rheumatology · 2021Article
- Role of C5 inhibition in Idiopathic Inflammatory Myopathies and Scleroderma Renal Crisis-Induced Thrombotic Microangiopathies.Kidney international reports · 2021Article
- Complement in Secondary Thrombotic Microangiopathy.Kidney international reports · 2021Review
- Review
- Refractory scleroderma renal crisis precipitated after high-dose oral corticosteroids and concurrent intravitreal injection of bevacizumab.SAGE open medical case reports · 2020Article
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We had the challenged to treat a 40-year-old female with Systemic Scleroderma who was showing unspecific symptoms. During her time at the hospital she rapidly develops renal dysfunction, associated with hypertension. She required renal replacement therapy initiation and we observed a decline in hemoglobin and platelets numbers. We confirm a microangiopathic hemolytic anemia and rule out other immune diseases or thrombotic thrombocytopenic purpura. Systemic Sclerosis is a chronic immune disorder of unknown origin that it is not completely understood. It is believed that environmental factors may contribute and also altered genes may be implicated in the immune system's function. Microangiopathic hemolytic anemia occurs in 43% of patients who develop scleroderma renal crisis and an activation of the complement system through the classical pathway may be involved. Given that context we decided to treat the patients with C5 blocker Eculizumab and obtain an extraordinary positive response.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.