renal crisis
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2021 ◽  
pp. 239936932110611
Author(s):  
Marcella M Frediani ◽  
Francisco Z Mattedi ◽  
Livia B Cavalcante ◽  
Verônica T Costa e Silva ◽  
Renato A Caires ◽  
...  

The incidence of malignancy is increased in systemic sclerosis (SS). Nevertheless, only a few cases of paraneoplastic SS (pSS) have been described. Scleroderma renal crisis is an uncommon but severe complication of SS, with acute kidney failure, abrupt onset of hypertension and microangiopathy. We present the case of a previously healthy patient who was diagnosed with ovarian carcinoma and underwent chemotherapy with carboplatin and paclitaxel. In association with the cancer, she developed SS and scleroderma renal crisis. She received initial supportive treatment, but her renal function worsened, and she started on hemodialysis. Furthermore, she received adjuvant surgical treatment for the cancer. Eighty-four days after cytoreductive surgery, her renal function recovered, and her SS manifestations improved.


2021 ◽  
Vol 22 (21) ◽  
pp. 11793
Author(s):  
Michèle Simon ◽  
Christian Lücht ◽  
Isa Hosp ◽  
Hongfan Zhao ◽  
Dashan Wu ◽  
...  

Background. Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc). Autoantibodies (Abs) against endothelial cell antigens have been implicated in SSc and SRC. However, their detailed roles remain poorly defined. Pro-inflammatory cytokine interleukin-6 (IL-6) has been found to be increased in SSc, but its role in SRC is unclear. Here, we aimed to determine how the autoantibodies from patients with SSc and SRC affect IL-6 secretion by micro-vascular endothelial cells (HMECs). Methods. Serum IgG fractions were isolated from either SSc patients with SRC (n = 4) or healthy individuals (n = 4) and then each experiment with HMECs was performed with SSc-IgG from a separate patient or separate healthy control. IL-6 expression and release by HMECs was assessed by quantitative reverse transcription and quantitative PCR (RT-qPCR) and immunoassays, respectively. The mechanisms underlying the production of IL-6 were analyzed by transient HMEC transfections with IL-6 promoter constructs, electrophoretic mobility shift assays, Western blots and flow cytometry. Results. Exposure of HMECs to IgG from SSc patients, but not from healthy controls, resulted in a time- and dose-dependent increase in IL-6 secretion, which was associated with increased AKT, p70S6K, and ERK1/2 signalling, as well as increased c-FOS/AP-1 transcriptional activity. All these effects could be reduced by the blockade of the endothelial PAR-1 receptor and/or c-FOS/AP-1silencing. Conclusions. Autoantibodies against PAR-1 found in patients with SSc and SRC induce IL-6 production by endothelial cells through signalling pathways controlled by the AP-1 transcription factor. These observations offer a greater understanding of adverse endothelial cell responses to autoantibodies present in patients with SRC.


Author(s):  
Mohammed Salah Hussein ◽  
Fozah sultan F. Alshammari ◽  
Rayan Jahz N. Almutihi ◽  
H. Alrougi Abdullah Fahad ◽  
Hussain Ali Busaleh ◽  
...  

Scleroderma renal crisis is a life-threatening condition. It usually starts with a sudden onset of severe hypertension, followed by renal failure, hypertensive encephalopathy, congestive heart failure, and/or microangiopathic hemolytic anemia. Renal ischemia, hyperplasia of the juxtaglomerular apparatus, activation of the renin-angiotensin-aldosterone system (RAAS), and an increase in blood pressure are caused by decreased blood flow caused by structural changes in the blood vessels as well as renal vasospasm ("Raynaud's phenomenon"). This overview discusses the evaluation, diagnosis, and treatment of scleroderma renal crisis, emphasizing the importance of early detection of disease, strong correlation of corticosteroids intake and the disease incidence, and best approach of such cases.


2021 ◽  
Vol 8 (3) ◽  
pp. 4
Author(s):  
Gülşah Boz ◽  
Samet Karahan ◽  
Nurdan Yüksek

Background: Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs, marked changes in microvascular structure, cellular and humoral immune disorders. Renal involvement is more frequent and mainly characterized by moderate proteinuria, elevated serum creatinine levels, and hypertension. The most common kidney involvement in SSc is scleroderma renal crisis (SRC) that is fatal without prompt intervention.Case report: A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia. On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone, decreased urine output, arterial hypertension, decreased renal function and pulmonary edema developed. The patient was diagnosed with a scleroderma renal crisis. Emergency hemodialysis was applied to the patient, and captopril 6 × 25 mg/day and nifedipine 2*60 mg/day treatment were given. He received a routine hemodialysis program for about three months. The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions: SRC, characterized by malignant hypertension, azotemia, microangiopathic hemolytic anemia, and kidney failure, is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention. Steroid use is one of the important risk factors that precipitate SRC development. With angiotensin-converting enzyme inhibitors, survival increased after SRC, the need for dialysis decreased, and usually allowed the discontinuation of dialysis treatment within about 6-18 months. Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step.


Author(s):  
Tomoya Nishida ◽  
Kazuhisa Nakano ◽  
Minoru Satoh ◽  
Shunsuke Fukuyo ◽  
Koichi Akashi ◽  
...  

Abstract Anti-PM/Scl antibodies are associated with the overlap syndrome of systemic sclerosis and dermatomyositis/polymyositis (SSc-DM/PM), and are found in 50% of SSc-DM/PM cases in Europe and the United States, whereas they are rare in Japan. We report a case of an 80-year-old Japanese female with SSc-amyopathic dermatomyositis (ADM) overlap syndrome, who developed scleroderma renal crisis (SRC), a complication of SSc. She had positive antinuclear antibodies in a discrete-speckled and nucleolar pattern and anticentromere antibodies and anti-PM/Scl antibodies were confirmed by ELISA and immunoprecipitation, respectively. The incidence rate of SRC in SSc patients varies significantly depending on the specificity of autoantibodies, with the highest incidence of ~50% in anti-RNA polymerase III antibody positive patients, followed by ~10% in anti-PM/Scl and lower incidence of 0.45% in anticentromere antibody-positive cases. Anti-PM/Scl antibodies are uncommon in Japanese patients presumably due to its strong association with certain HLA haplotype that is rare in Japanese. Clinical significance of anti-PM/Scl antibodies in Japanese patients will need to be clarified with accumulation of cases in future studies.


Hypertension ◽  
2021 ◽  
Vol 78 (Suppl_1) ◽  
Author(s):  
Mohammed Uddin ◽  
Tanveer Mir ◽  
Obaid Shafi ◽  
Hamza Tahir ◽  
Sarvani Surapaneni ◽  
...  

Background: Literature regarding trends for incidence and mortality of Scleroderma renal crisis (SRC) in systemic sclerosis (SSc) within the United States (US) emergency departments (ED) is limited. Objective: To study the trends for incidence and mortality of SRC among SSc patient encounters within the US EDs. Methods: Data from the national emergency department sample (NEDS) constitutes 20% sample of hospital-owned EDs and in-patient sample in the United States. All ED encounters were included in the analysis. Major complications related to each ED encounter were obtained by using ICD codes. A Linear p-trend was used to assess the trends. Results: Of the total 180,435 ED encounters with a diagnosis of SSc in NEDS for the years 2009-2014, 771 (4.27/1,000) (mean age of 59.6 ± 15.5 years, 75.4% females) patients were recorded with SRC. A total of 32 (4.1%) patients had died during these ED hospital encounters. The SSc with SRC subgroup was associated with higher rates of life-threatening complications when compared to SSc without the SRC subgroup. Cerebrovascular complications include acute ischemic stroke 44 (5.6%). Cardiac complications include new-onset atrial fibrillation 62 (8%), conduction block 13 (1.6%) and acute onset heart failure 186 (24.1%). Other complications include pulmonary arterial hypertension 122 (15.8%), respiratory failure 212 (27.5%), and deep vein thrombosis 36 (4.7%). The yearly trend for the incidence of SRC every 1,000 ED SSc encounters in the United States increased over the study years from 2.11/1,000 in 2009 to 5.79/1,000 in 2014 (linear p-trend 0.002). The yearly trend for the mortality related to SRC in the United States remained steady over the study years 2009-2014 (linear p-trend 0.3187). Conclusion: SRC is a relatively rare medical emergency among patients with SSc. Although there has been a significant rise in the incidence of SRC among SSc patients over the study years, mortality rates had remained steady. Proper management of the underlying risk factors can prevent the development of SRC and associated complications. Keywords: Systemic sclerosis, Scleroderma renal crisis, pulmonary hypertension, deep vein thrombosis.


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