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

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Jomana Madieh ◽  
Iman Khamayseh ◽  
Alaa Hrizat ◽  
Abdurrahman Hamadah ◽  
Kamel Gharaibeh
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Ace Inhibitors ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Enzyme Inhibitors ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme Inhibitors ◽  
Renal Crisis

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.

scholarly journals Favourable Outcome of Scleroderma Renal Crisis

1996 ◽  
Vol 89 (1) ◽  
pp. 49P-50P ◽  
Author(s):  
D A Collins ◽  
S Patel ◽  
J B Eastwood ◽  
B E Bourke
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Enzyme Inhibitors ◽  
Severe Hypertension ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Favourable Outcome ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Renal Crisis

Severe hypertension and rapidly progressive acute renal failure is a well recognized complication of scleroderma, often referred to as the renal crisis, and widely thought to cause irreversible deterioration in renal function. With the advent of angiotensin-converting-enzyme inhibitors (ACE-I) the outlook for patients with this condition has dramatically improved. We report here one such patient.

scholarly journals Renal Involvement in Systemic Sclerosis: An Update

2020 ◽  
Vol 45 (4) ◽  
pp. 532-548
Author(s):  
Magdalena Chrabaszcz ◽  
Jolanta Małyszko ◽  
Mariusz Sikora ◽  
Rosanna Alda-Malicka ◽  
Anna Stochmal ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Angiotensin Converting Enzyme ◽  
Enzyme Inhibitors ◽  
Renal Involvement ◽  
Significant Proportion ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Renal Crisis

Background: Systemic sclerosis is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis and autoimmune phenomena. Summary: Renal disease occurring in patients with systemic sclerosis may have a variable clinicopathological picture. The most specific renal condition associated with systemic sclerosis is scleroderma renal crisis, characterized by acute onset of renal failure and severe hypertension. Although the management of scleroderma renal crisis was revolutionized by the introduction of angiotensin-converting enzyme inhibitors, there is still a significant proportion of patients with poor outcomes. Therefore, research on establishing disease markers (clinical, ultrasonographical and serological) and clear diagnostic criteria, which could limit the risk of developing scleroderma renal crisis and facilitate diagnosis of this complication, is ongoing. Other forms of renal involvement in systemic sclerosis include vasculitis, an isolated reduced glomerular filtration rate in systemic sclerosis, antiphospholipid-associated nephropathy, high intrarenal arterial stiffness and proteinuria. Key Messages: Scleroderma renal crisis is the most specific and life-threatening renal presentation of systemic sclerosis, albeit with declining prevalence. In patients with scleroderma renal crisis, it is mandatory to control blood pressure early with increasing doses of angiotensin-converting enzyme inhibitors, along with other antihypertensive drugs if necessary. There is a strong association between renal involvement and patients’ outcomes in systemic sclerosis; consequently, it becomes mandatory to find markers that may be used to identify patients with an especially high risk of scleroderma renal crisis.

Kidney involvement in systemic sclerosis: From pathogenesis to treatment

2018 ◽  
Vol 3 (1) ◽  
pp. 43-52 ◽  
Author(s):  
Cosimo Bruni ◽  
Giovanna Cuomo ◽  
Francesca W. Rossi ◽  
Emanuela Praino ◽  
Silvia Bellando-Randone
Keyword(s):  
Systemic Sclerosis ◽  
Angiotensin Converting Enzyme ◽  
Enzyme Inhibitors ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Prognostic Impact ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Kidney Involvement ◽  
Renal Crisis

Among all possible systemic sclerosis internal organ complications, kidney involvement is frequently neglected or underestimated, except for the life-threatening scleroderma renal crisis. Fortunately, this severe clinical presentation is nowadays better controlled with available treatments, in particular angiotensin-converting enzyme inhibitors, and this has led to a reduction in its short- and longer-term mortality. Pathogenetic determinants are not well understood and many different other kidney involvements are possible in systemic sclerosis, including proteinuria, albuminuria, reduction of renal filtration, autoantibodies-related glomerulonephritis, and drug-related side effects. Different serological and radiological methods of evaluations are nowadays available, some representing promising diagnostic tool and prognostic outcome measure. Except for angiotensin-converting enzyme inhibitors in scleroderma renal crisis, no other treatment is currently recommended for treatment of kidney involvement in systemic sclerosis. For this reason, further studies are necessary to investigate its prognostic impact, in particular in combination with other systemic sclerosis–related internal organ manifestations. This review summarizes current available literature on kidney involvement in systemic sclerosis.

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