Scleroderma renal crisis in a patient with paraneoplastic systemic sclerosis

2021 ◽  
pp. 239936932110611
Author(s):  
Marcella M Frediani ◽  
Francisco Z Mattedi ◽  
Livia B Cavalcante ◽  
Verônica T Costa e Silva ◽  
Renato A Caires ◽  
...  
Keyword(s):  
Renal Function ◽  
Systemic Sclerosis ◽  
Surgical Treatment ◽  
Cytoreductive Surgery ◽  
Kidney Failure ◽  
Abrupt Onset ◽  
Scleroderma Renal Crisis ◽  
Supportive Treatment ◽  
Healthy Patient ◽  
Renal Crisis

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.

scholarly journals THU0600 A CASE OF SYSTEMIC SCLEROSIS COMPLICATED BY RENAL CRISIS: POTENTIAL ETIOPATHOGENETIC ROLE OF CYTOMEGALOVIRUS AND TREATMENT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 542.1-542
Author(s):  
A. Palermo ◽  
E. Galli ◽  
A. Spinella ◽  
E. Cocchiara ◽  
F. Lumetti ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Systemic Sclerosis ◽  
Filtration Rate ◽  
Ace Inhibitor ◽  
Viral Infections ◽  
Scleroderma Renal Crisis ◽  
Renal Crisis

Background:Scleroderma renal crisis (SRC) is a rare complication of systemic sclerosis (SSc), which can be triggered by viruses, such as Cytomegalovirus (CMV). SRC presents as a new-onset accelerated-phase hypertension with/without rapidly progressive renal failure.Objectives:Here we describe the case of a patient developing SSc complicated by the appearance of SRC after a recent episode of acute Cytomegalovirus infection.Methods:A 66-year-old male was referred to our Scleroderma Unit in March 2019. He presented with widespread skin rash, exertional dyspnoea and peripheral oedemas. He reported a myocarditis due to CMV occurred in October 2018. Antibodies anti-CMV IgM were detected in his serum. The patient developed a progressive cutaneous involvement characterized by diffuse oedema, sclerosis and melanoderma. Subsequently, Raynaud’s phenomenon, puffy hands and pitting scars occurred. Laboratory tests showed positive ANA in a titer of 1:640 in a nucleolar staining pattern. Additionally, persistence of anti-CMV IgM was found. Skin biopsy showed scleroderma-like finding. Nailfold capillaroscopy revealed a SSc pattern. Chest high resolution computed tomography displayed basal interstitial thickening and subpleuric ground-glass opacities. Therefore, the patient was diagnosed with SSc. Three weeks later he developed severe hypertension and a rapid, progressive renal impairment. Serum creatinine increased (up to 4.15 mg/dl), glomerular filtration rate impaired (25 ml/min). Renal biopsy (picture A, B) revealed acute thrombotic microangiopathy. A diagnosis of thrombotic thrombocytopenic purpura was excluded. The patient was diagnosed with SRC and we started therapy with ACE-inhibitor and loop diuretic. Even if the dosage of ACE-inhibitor was increased up to the maximum tolerate dose, his renal function did not improve and the blood pressure control was inadequate. Consequently, the patient underwent plasma exchange (PEx) sessions. Two weeks later there was an improvement of renal function and blood pressure normalized. Six months later the disease was controlled: glomerular filtration rate was 41 ml/min and blood pressure was within the normal range. The patient was treated with ACE-inhibitor and underwent fortnightly apheretic sessions. Treatment for scleroderma vasculopathy is ongoing.Results:Viral infections may be responsible for SSc. A brief interval between an acute viral infection and the onset of SSc may suggest CMV as a possible trigger for the disease. Similarly, other infectious agents could be involved in the multistep and multifactorial mechanism of SSc. This case sheds light on the potential and intriguing role of CMV in SSc. Moreover, it leads us to hypothesize a CMV possible direct role in sclerodermal kidney damage. Use of ACE-inhibitor significantly reduced the mortality rate due to this complication. Exact therapeutic mechanism of PEx in the treatment of SSc is unclear.Conclusion:In our case the integrated ACE-inhibitor-PEx approach has showed effectiveness and safety in the management of SRC.References:[1]Ferri C, et al. Viral infections and systemic sclerosis. Clin Exp Rheumatol. 2014 32 (6Suppl86), S-229.[2]Zanatta E, et al. Therapy of scleroderma renal crisis: State of the art. Autoimmunity Rev. 2018 Sep;17(9):882-889.Disclosure of Interests:None declared

scholarly journals POS1439 A MULTI-PREDICTOR MODEL TO PREDICT RISK OF SCLERODERMA RENAL CRISIS IN SYSTEMIC SCLEROSIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1004.1-1004
Author(s):  
D. Xu ◽  
R. Mu
Keyword(s):  
Systemic Sclerosis ◽  
Prediction Model ◽  
Renal Involvement ◽  
Area Under The Curve ◽  
Individual Risk ◽  
Multivariable Logistic Regression Analysis ◽  
Scleroderma Renal Crisis ◽  
Lasso Regression ◽  
Patients At Risk ◽  
Renal Crisis

Background:Scleroderma renal crisis (SRC) is a life-threatening syndrome. The early identification of patients at risk is essential for timely treatment to improve the outcome[1].Objectives:We aimed to provide a personalized tool to predict risk of SRC in systemic sclerosis (SSc).Methods:We tried to set up a SRC prediction model based on the PKUPH-SSc cohort of 302 SSc patients. The least absolute shrinkage and selection operator (Lasso) regression was used to optimize disease features. Multivariable logistic regression analysis was applied to build a SRC prediction model incorporating the features of SSc selected in the Lasso regression. Then, a multi-predictor nomogram combining clinical characteristics was constructed and evaluated by discrimination and calibration.Results:A multi-predictor nomogram for evaluating the risk of SRC was successfully developed. In the nomogram, four easily available predictors were contained including disease duration <2 years, cardiac involvement, anemia and corticosteroid >15mg/d exposure. The nomogram displayed good discrimination with an area under the curve (AUC) of 0.843 (95% CI: 0.797-0.882) and good calibration.Conclusion:The multi-predictor nomogram for SRC could be reliably and conveniently used to predict the individual risk of SRC in SSc patients, and be a step towards more personalized medicine.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

scholarly journals French recommendations for the management of systemic sclerosis

2021 ◽  
Vol 16 (S2) ◽  
Author(s):  
Eric Hachulla ◽  
Christian Agard ◽  
Yannick Allanore ◽  
Jerome Avouac ◽  
Brigitte Bader-Meunier ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Proximal Region ◽  
Scleroderma Renal Crisis ◽  
Cardiac Damage ◽  
Favorable Prognosis ◽  
Causes Of Mortality ◽  
Cutaneous Form ◽  
Pulmonary Arterial ◽  
Renal Crisis ◽  
Severe Pulmonary Arterial Hypertension

AbstractSystemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.

scholarly journals Vascular Complications of Systemic Sclerosis during Pregnancy

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Eliza F. Chakravarty
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Pregnancy Outcomes ◽  
Vascular Complications ◽  
Autoimmune Disorder ◽  
Morbidity And Mortality ◽  
Scleroderma Renal Crisis ◽  
Hemodynamic Changes ◽  
Pulmonary Arterial ◽  
Renal Crisis

Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by progressive fibrosis of the skin and visceral tissues as well as a noninflammatory vasculopathy. Vascular disease in systemic sclerosis is a major cause of morbidity and mortality among nonpregnant patients with SSc and is even a bigger concern in the pregnant SSc patient, as the underlying vasculopathy may prevent the required hemodynamic changes necessary to support a growing pregnancy. Vascular manifestations including scleroderma renal crisis and pulmonary arterial hypertension should be considered relative contraindications against pregnancy due to the high associations of both maternal and fetal morbidity and mortality. In contrast, Raynaud's phenomenon may actually improve somewhat during pregnancy. Women with SSc who are considering a pregnancy or discover they are pregnant require evaluation for the presence and extent of underlying vasculopathy. In the absence of significant visceral vasculopathy, most women with SSc can expect to have reasonable pregnancy outcomes.

Alveolar Hemorrhage and Pulmonary Hypertension in Systemic Sclerosis: A Continuum of Scleroderma Renal Crisis?

2001 ◽  
Vol 7 (2) ◽  
pp. 115-119 ◽  
Author(s):  
Thomas M. Herndon ◽  
Theodore T. Kim ◽  
Bruce E. Goeckeritz ◽  
Lisa K. Moores ◽  
Robert J. Oglesby ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Alveolar Hemorrhage ◽  
Scleroderma Renal Crisis ◽  
Renal Crisis

Analysis of Anti-RNA Polymerase III Antibody-positive Systemic Sclerosis and Altered GPATCH2L and CTNND2 Expression in Scleroderma Renal Crisis

2020 ◽  
Vol 47 (11) ◽  
pp. 1668-1677
Author(s):  
Edward P. Stern ◽  
Sandra G. Guerra ◽  
Harry Chinque ◽  
Vanessa Acquaah ◽  
David González-Serna ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Renal Biopsy ◽  
Genetic Susceptibility ◽  
Rna Polymerase Iii ◽  
Human Kidney ◽  
Scleroderma Renal Crisis ◽  
Nucleotide Polymorphisms ◽  
Polymerase Iii ◽  
Renal Crisis

ObjectiveScleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) strongly associated with anti-RNA polymerase III antibody (ARA) autoantibodies. We investigated genetic susceptibility and altered protein expression in renal biopsy specimens in ARA-positive patients with SRC.MethodsARA-positive patients (n = 99) with at least 5 years’ follow-up (49% with a history of SRC) were selected from a well characterized SSc cohort (n = 2254). Cases were genotyped using the Illumina Human Omni-express chip. Based on initial regression analysis, 9 single-nucleotide polymorphisms (SNP) were chosen for validation in a separate cohort of 256 ARA-positive patients (40 with SRC). Immunostaining of tissue sections from SRC or control kidney was used to quantify expression of candidate proteins based upon genetic analysis of the discovery cohort.ResultsAnalysis of 641,489 SNP suggested association of POU2F1 (rs2093658; P = 1.98 × 10−5), CTNND2 (rs1859082; P = 5.58 × 10−5), HECW2 (rs16849716; P = 1.2 × 10−4), and GPATCH2L (rs935332; P = 4.92 × 10−5) with SRC. Further, the validation cohort showed an association between rs935332 within the GPATCH2L region, with SRC (P = 0.025). Immunostaining of renal biopsy sections showed increased tubular expression of GPATCH2L (P = 0.026) and glomerular expression of CTNND2 (P = 0.026) in SRC samples (n = 8) compared with normal human kidney controls (n = 8), despite absence of any genetic replication for the associated SNP.ConclusionIncreased expression of 2 candidate proteins, GPATCH2L and CTNND2, in SRC compared with control kidney suggests a potential role in pathogenesis of SRC. For GPATCH2L, this may reflect genetic susceptibility in ARA-positive patients with SSc based upon 2 independent cohorts.

scholarly journals Classifications of scleroderma renal crisis and reconsideration of its pathophysiology

Rheumatology ◽  
2019 ◽  
Vol 58 (12) ◽  
pp. 2099-2106 ◽  
Author(s):  
Hiroyuki Yamashita ◽  
Ryosuke Kamei ◽  
Hiroshi Kaneko
Keyword(s):  
Blood Pressure ◽  
Renal Function ◽  
Pathological Condition ◽  
Therapeutic Interventions ◽  
Elevated Blood Pressure ◽  
Scleroderma Renal Crisis ◽  
Severe Thrombocytopenia ◽  
Elevated Blood ◽  
Prognostic Prediction ◽  
Renal Crisis

Abstract Categorization of scleroderma renal crisis (SRC) as hypertensive or normotensive can potentially overlook the underlying pathophysiology that might be unique in each patient, as they often exhibit a mixture of distinct pathological characteristics of narrowly defined SRC (nd-SRC) and systemic sclerosis associated thrombocytic micro-angiopathy (SSc-TMA). In this review, we provide evidence suggesting that better categorization of patients presenting with certain clinical features of both nd-SRC and TMA will improve treatment approaches. Based on our clinical experience and literature review, distinguishing between nd-SRC and SSc-TMA is important because the association of SSc-TMA with prior steroid administration and poor prognosis was stronger than that of nd-SRC. Although the two pathological entities cannot be easily distinguished based on blood pressure, we suggest that the detailed clinical course is helpful. Typically, nd-SRC exhibits prominently elevated blood pressure and worsening of renal function initially, followed by mild thrombocytopenia. Conversely, SSc-TMA presents first with severe thrombocytopenia, followed by elevated blood pressure and renal function deterioration. The degree of involvement in each pathological condition should be considered for determination of appropriate therapeutic interventions and prognostic prediction.

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