scholarly journals Overview of Scleroderma Renal Crisis - A Review

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.

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Adam Hafeez ◽  
Dillon Karmo ◽  
Adrian Mercado-Alamo ◽  
Alexandra Halalau

Aortic dissection is a life-threatening condition in which the inner layer of the aorta tears. Blood surges through the tear, causing the inner and middle layers of the aorta to separate (dissect). It is considered a medical emergency. We report a case of a healthy 56-year-old male who presented to the emergency room with sudden onset of epigastric pain radiating to his back. His blood pressure was 167/91 mmHg, equal in both arms. His lipase was elevated at 1258 U/L, and he was clinically diagnosed with acute pancreatitis (AP). He denied any alcohol consumption, had no evidence for gallstones, and had normal triglyceride level. Two days later, he endorsed new suprapubic tenderness radiating to his scrotum, along with worsening epigastric pain. A MRCP demonstrated evidence of an aortic dissection (AD). CT angiography demonstrated a Stanford type B AD extending into the proximal common iliac arteries. His aortic dissection was managed medically with rapid blood pressure control. The patient had excellent recovery and was discharged home without any surgical intervention.


2020 ◽  
Vol 2020 (7) ◽  
Author(s):  
Aakriti Yadav ◽  
Uttam Krishna Shrestha ◽  
Kajan Raj Shrestha ◽  
Dinesh Gurung

Abstract Aorto-esophageal fistula is a life-threatening condition, accounting for a small number of cases of upper gastrointestinal bleeding where patients present with one or more features of Chiari’s triad. We present the case of a 43-year-old woman, referred to us with symptoms of central chest pain, sudden onset dysphagia followed by massive hemoptysis. She was diagnosed with an aorto-esophageal fistula due to a ruptured thoracic aortic aneurysm and rushed for an emergency endovascular thoracic aortic stent and feeding jejunostomy with intravenous antibiotics and supportive care. After 6 weeks of surgery, the patient was re-evaluated to plan for an esophageal stent if required. The purpose of this presentation is to make the surgical fraternity aware of the gravity of this disease and novel techniques to manage it.


2014 ◽  
Vol 41 (6) ◽  
pp. 1040-1048 ◽  
Author(s):  
Luc Mouthon ◽  
Guillaume Bussone ◽  
Alice Berezné ◽  
Laure-Hélène Noël ◽  
Loïc Guillevin

Scleroderma renal crisis (SRC) is characterized by malignant hypertension and oligo-anuric acute renal failure. It occurs in 5% of patients with systemic sclerosis (SSc), particularly in patients with diffuse disease during the first years. SRC is more common in patients receiving corticosteroids, the risk increasing with increasing dose. The disease is sometimes triggered by use of nephrotoxic drugs and/or intravascular volume depletion. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of cases, and anti-RNA-polymerase III antibodies are present in one-third of patients. Renal biopsy is not necessary if SRC presents classical features. However, biopsy may help to define the prognosis and guide treatment in atypical forms. The prognosis of SRC has greatly improved with the introduction of angiotensin-converting enzyme (ACE) inhibitors. However, the 5-year survival for SSc patients with full SRC remains low (65%). The treatment of SRC relies on aggressive blood pressure control with an ACE inhibitor, combined with other antihypertensive drugs if needed. Dialysis is frequently indicated but can be stopped in about half of patients, mainly those with good blood pressure control. Patients who need dialysis for more than 2 years qualify for renal transplantation.


2009 ◽  
Vol 2009 ◽  
pp. 1-3 ◽  
Author(s):  
Karen L. Koscica ◽  
Uzoma Nwaubani ◽  
Munir Nazir ◽  
Martin Gimovsky

Background. We report a case of familial hyperlipidemia in pregnancy that resulted in hemorrhagic pancreatitis.Case. A patient at 27-week gestation was admitted for recurrent pancreatitis secondary to severe hyperlipidemia. With conservative care, the patient improved but on the fourth day of admission she experienced a sudden onset of hypotension and was diagnosed with hemorrhagic pancreatitis.Conclusion. Pancreatitis caused by hyperlipidemia is an uncommon event during pregnancy. A familiarity with the severe complications associated with this potentially life-threatening condition is important.


Renal Failure ◽  
1996 ◽  
Vol 18 (4) ◽  
pp. 567-574 ◽  
Author(s):  
Piero Stratta ◽  
Luca Besso ◽  
Stephania Ferrero ◽  
Caterina Canavese ◽  
Susanna Hollo ◽  
...  

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1440.1-1440
Author(s):  
D. Xu ◽  
J. He ◽  
T. LI ◽  
R. Mu

Background:Scleroderma renal crisis (SRC) is a rare but life-threatening complication in SSc, which defined as malignant hypertension and progressive renal failure [1]. Previous reports of the prevalence for scleroderma renal crisis (SRC) show variable outcomes.Objectives:We aim to find out the prevalence for SRC in Chinese, and conduct a meta-analysis to assess the prevalence of SRC in worldwide and investigate possible factors of variability.Methods:We evaluated the prevalence for SRC and compared the clinical characteristics in SSc patients with or without SRC. A systematic review on the prevalence of SRC was carried out in PubMed, Embase and Cochrane. Meta-analysis was performed using available data on the prevalence for SRC.Results:302 patients were enrolled in PKUPH-SSc cohort and the prevalence of SRC was 4.97%. In our cohort, SRC was found in 3.60% limited cutaneous SSc (lcSSc) and 7.75% diffused cutaneous SSc (dcSSc) patients. Twenty-nine studies including a total population of 22686 SSc patients were analyzed in the meta-analysis. The overall pooled prevalence of SRC was 4.0%, but heterogeneity among the studies was substantial (I2=86.1%, P <0.00001). Meta-regression revealed that subtype of SSc and geographic factors partially explained this heterogeneity. Sub-group analyses demonstrated that the prevalence of SRC in dcSSc and lcSSc were 9.0% and 2.0%. The SRC prevalence in SSc was 3.0% in Asia, 5.0% in Europe, and 3.0% in North America, respectively.Conclusion:Data on our new cohort showed the prevalence of SRC was around 5% in China, and meta-analysis confirmed that the prevalence of SRC varies among centers. The marked heterogeneity of SRC prevalence was partially explained by subtype of SSc and geographic origin of patients.References:[1]Woodworth TG, Suliman YA, Li W, Furst DE, Clements P (2016) Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 12 (11):678-91.Disclosure of Interests:None declared


2007 ◽  
Vol 1108 (1) ◽  
pp. 249-258 ◽  
Author(s):  
L. TEIXEIRA ◽  
A. MAHR ◽  
A. BEREZNE ◽  
L.-H. NOEL ◽  
L. GUILLEVIN ◽  
...  

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Divyesh Reddy Nemakayala ◽  
Manoj P Rai ◽  
Shilpa Kavuturu ◽  
Supratik Rayamajhi

Lemierre’s disease is a rare but life-threatening condition characterized by an oropharyngeal infection complicating with thrombophlebitis of the internal jugular vein and disseminated abscesses. We are presenting a case of a young female who initially presented with fevers, chills, sore throat, and swollen neck later developed progressively worsening shortness of breath along with sudden onset pleuritic chest pain. She then developed progressively worsening acute hypoxic respiratory failure requiring intubation and mechanical ventilation. Interval chest X-ray showed worsening bilateral effusions. She also developed septic shock requiring pressors. Blood culture showed Fusobacterium, and antibiotics were changed accordingly following which there was a clinical improvement. The diagnosis of Lemierre’s syndrome was then established based on her presenting age and bilateral pulmonary empyema in the setting of septicemia with Fusobacterium.


2018 ◽  
Vol 27 (4) ◽  
pp. 236-240
Author(s):  
Abdul Hafiz Shaharudin ◽  
Muhamad Hafiq Ab Hamid ◽  
Rosliza Yahaya ◽  
Nik Ahmad Shaiffudin Nik Him ◽  
Nik Arif Nik Mohamed ◽  
...  

Introduction: Aortic dissection is a clinical chameleon that can have variable presenting features that require a careful history and physical examination. A non-specific presentation of this life-threatening condition causes a diagnostic dilemma among clinicians especially in the emergency department leading to grave consequences. Case Presentation: We present a case of aortic dissection that presented as an acute bilateral blindness that was associated with a sudden onset of loss of consciousness and central chest pain. Bedside carotid ultrasound showed a double lumen carotid artery suggesting an intraluminal flap. Computed tomography angiography revealed extensive dissection of the entire length of the aorta. Discussion: This case illustrated the need for a high index of suspicion to diagnose patients with aortic dissection especially as the patient presented with an acute binocular visual loss and chest pain. Conclusion: A bedside carotid artery ultrasound in the emergency department was found useful in screening and diagnosing a carotid artery–related pathology.


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