New-onset systemic sclerosis and scleroderma renal crisis under docetaxel

2021 ◽  
pp. 239719832110076
Author(s):  
Véronique Debien ◽  
Arthur Petitdemange ◽  
Dorothée Bazin ◽  
Carole Ederle ◽  
Benoit Nespola ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase Iii ◽  
Systemic Autoimmune Disease ◽  
Scleroderma Renal Crisis ◽  
Prostate Cancers ◽  
Poor Outcomes ◽  
Induced Changes ◽  
First Time ◽  
Lung And Kidney ◽  
New Onset

Systemic sclerosis is a rare systemic autoimmune disease characterized by microvascular impairment and fibrosis of the skin and other organs with poor outcomes. Toxic causes may be involved. We reported the case of a 59-year-old woman who developed an acute systemic sclerosis after two doses of adjuvant chemotherapy by docetaxel and cyclophosphamide for a localized hormone receptor + human epithelial receptor 2—breast cancer. Docetaxel is a major chemotherapy drug used in the treatment of breast, lung, and prostate cancers, among others. Scleroderma-like skin-induced changes (morphea) have been already described for taxanes. Here, we report for the first time a case of severe lung and kidney flare with thrombotic microangiopathy after steroids for acute interstitial lung disease probably induced by anti-RNA polymerase III + systemic sclerosis after docetaxel.

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 Prevalence, Correlates and Outcomes of Gastric Antral Vascular Ectasia in Systemic Sclerosis: A EUSTAR Case-control Study

2013 ◽  
Vol 41 (1) ◽  
pp. 99-105 ◽  
Author(s):  
Etienne Ghrénassia ◽  
Jérome Avouac ◽  
Dinesh Khanna ◽  
Chris T. Derk ◽  
Oliver Distler ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Scleroderma Renal Crisis ◽  
Gastric Antral Vascular Ectasia ◽  
Vascular Ectasia ◽  
Polymerase Iii ◽  
European League Against Rheumatism ◽  
Vascular Phenotype ◽  
Renal Crisis

Objective.To estimate the prevalence, determine the subgroups at risk, and the outcomes of patients with systemic sclerosis (SSc) and gastric antral vascular ectasia (GAVE).Methods.We queried the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) network for the recruitment of patients with SSc-GAVE. Each case was matched for cutaneous subset and disease duration with 2 controls with SSc recruited from the same center, evaluated at the time the index case made the diagnosis of GAVE. SSc characteristics were recorded at the time GAVE occurred and the last observation was collected to define the outcomes.Results.Forty-nine patients with SSc and GAVE were included (24 with diffuse cutaneous SSc) and compared to 93 controls with SSc. The prevalence of GAVE was estimated at about 1% of patients with SSc. By multivariate analysis, patients with SSc-GAVE more frequently exhibited a diminished (< 75%) DLCO value (OR 12.8; 95% CI 1.9–82.8) despite less frequent pulmonary fibrosis (OR 0.2; 95% CI 0.1–0.6). GAVE was also associated with the presence of anti-RNA-polymerase III antibodies (OR 4.6; 95% CI 1.2–21.1). SSc-GAVE was associated with anemia (82%) requiring blood transfusion (45%). Therapeutic endoscopic procedures were performed in 45% of patients with GAVE. After a median followup of 30 months (range 1–113 months), survival was similar in patients with SSc-GAVE compared to controls, but a higher number of scleroderma renal crisis cases occurred (12% vs 2%; p = 0.01).Conclusion.GAVE is rare and associated with a vascular phenotype, including anti-RNA-polymerase III antibodies, and a high risk of renal crisis. Anemia, usually requiring blood transfusions, is a common complication.

scholarly journals Clinical and Immunologic Predictors of Scleroderma Renal Crisis in Japanese Systemic Sclerosis Patients With Anti-RNA Polymerase III Autoantibodies

2015 ◽  
Vol 67 (4) ◽  
pp. 1045-1052 ◽  
Author(s):  
Yasuhito Hamaguchi ◽  
Masanari Kodera ◽  
Takashi Matsushita ◽  
Minoru Hasegawa ◽  
Yuki Inaba ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Scleroderma Renal Crisis ◽  
Polymerase Iii ◽  
Renal Crisis

scholarly journals Demyelinating Syndrome in Systemic Sclerosis and Neuromyelitis Optica

2019 ◽  
Author(s):  
khaled Deeb ◽  
Jessika Eby ◽  
Jose Labault-Santiago
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Neuromyelitis Optica ◽  
Rna Polymerase Iii ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Clinical Findings ◽  
Therapeutic Interventions ◽  
High Dose ◽  
Scleroderma Renal Crisis

Abstract INTRODUCTION: The objective of this study is to report a case of a 44-year-old female who presented with intractable hiccups, vomiting, and later complicated with paraplegia. Imaging and sero/immunological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity and transverse myelitis on MRI. Further investigation revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes, namely systemic sclerosis and neuromyelitis optica, and their underlying clinical manifestations and therapeutic interventions, are seldom reported in literature and are worth reporting. CASE REPORT: The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified as steroid induced scleroderma renal crisis on renal biopsy. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received consent forms from the participant in our study, and we have them on file in case they are requested. We have also received patient’s consent for the data presented in this article and Figure 1. CONCLUSION: This report expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.

scholarly journals Disparities in Outcomes Among Patients Diagnosed With Cancer in Proximity to an Emergency Department Visit

2022 ◽  
Author(s):  
Nicholas Pettit ◽  
Elisa Sarmiento ◽  
Jeffrey Kline
Keyword(s):  
Emergency Department ◽  
Low Income ◽  
Control Analysis ◽  
Adjusted Risk ◽  
Risk Of Death ◽  
Department Of Health ◽  
Prostate Cancers ◽  
Poor Outcomes ◽  
Case Control Analysis ◽  
First Time

Abstract A suspected diagnosis of cancer in the emergency department (ED) may be associated with poor outcomes, related to health disparities, however data are limited. This study is a case-control analysis of the Indiana State Department of Health Cancer Registry, and the Indiana Network for Patient Care. First time cancer diagnoses appearing in the registry between January 2013 and December 2017 were included. Cases were patients who had an ED visit in the 6 months before their cancer diagnosis; controls had no recent ED visits. The primary outcome was mortality, comparing ED-associated mortality to non-ED-associated. 134,761 first-time cancer patients were identified, including 15,432 (11.5%) cases. The mean age was same at 65, more of the cases were Black than the controls (12.4% vs 7.4%, P<.0001) and more were low income (36.4%. vs 29.3%). The top 3 ED-associated cancer diagnoses were lung (18.4%), breast (8.9%), and colorectal cancers (8.9%), whereas the controls were breast (17%), lung (14.9%), and prostate cancers (10.1%). Cases observed an over three-fold higher mortality, with cumulative death rate of 32.9% for cases vs 9.0% for controls (P<.0001). Regression analysis predicting mortality, controlling for many confounders produced an odds ratio of 4.12 (95% CI 3.72-4.56 for cases). This study found that an ED visit within 6 months prior to the first time of ICD-coded cancer is associated with Black race, low income and an overall three-fold increased adjusted risk of death. The mortality rates for ED-associated cancers are uniformly worse for all cancer types. These data suggest that additional work is needed to reduce disparities among ED-associated cancer diagnoses.

scholarly journals Demyelinating syndrome in systemic sclerosis and neuromyelitis optica

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Khaled Deeb ◽  
Jessika Eby ◽  
Jose Labault-Santiago
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Neuromyelitis Optica ◽  
Rna Polymerase Iii ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Clinical Findings ◽  
Therapeutic Interventions ◽  
High Dose ◽  
Scleroderma Renal Crisis

Abstract Background This article reports a case diagnosis of a 44-year-old female who presented with intractable hiccups and vomit complicated with an acute onset of paraplegia. Transverse myelitis was evident on MRI and serological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity. Further studies revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes and their underlying clinical manifestations and therapeutic interventions are seldom reported in literature and are worth reporting. Case presentation The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified on biopsy as steroids-induced scleroderma renal crisis. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where the patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received a written consent forms from the participant in our study, and we have them on file in case they are requested. We have also received the patient’s written consent for the data and images presented in this article. Conclusion This article expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.

scholarly journals A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Cody M. Lee ◽  
Diana Girnita ◽  
Arundhati Sharma ◽  
Surabhi Khanna ◽  
Jean M. Elwing
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Wide Spectrum ◽  
Rna Polymerase Iii ◽  
Clinical Manifestations ◽  
Autoimmune Disorder ◽  
Scleroderma Renal Crisis ◽  
Polymerase Iii ◽  
Digital Ischemia ◽  
Pulmonary Arterial

Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia. However, anti-RNAP III has not been associated with systemic sclerosis sine scleroderma. We describe a patient with an atypical presentation of anti-RNAP III positive systemic sclerosis sine scleroderma who presented without the typical features of anti-RNAP III disease. Instead, she presented with critical digital ischemia, pulmonary arterial hypertension, gastroesophageal reflux disease, interstitial lung disease, and no clinically detectable sclerodactyly.

scholarly journals Demyelinating Syndrome in Systemic Sclerosis and Neuromyelitis Optica

2019 ◽  
Author(s):  
khaled Deeb ◽  
Jessika Eby ◽  
Jose Labault-Santiago
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Neuromyelitis Optica ◽  
Rna Polymerase Iii ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Clinical Findings ◽  
Therapeutic Interventions ◽  
High Dose ◽  
Scleroderma Renal Crisis

Abstract INTRODUCTION: The objective of this study is to report a case of a 44-year-old female who presented with intractable hiccups, vomiting, and later complicated with paraplegia. Imaging and sero/immunological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity and transverse myelitis on MRI. Further investigation revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes, namely systemic sclerosis and neuromyelitis optica, and their underlying clinical manifestations and therapeutic interventions, are seldom reported in literature and are worth reporting. CASE REPORT: The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified as steroid induced scleroderma renal crisis on renal biopsy. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received consent forms from the participant in our study, and we have them on file in case they are requested. We have also received patient’s consent for the data presented in this article and Figure 1. CONCLUSION: This report expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.

scholarly journals Demyelinating Syndrome in Systemic Sclerosis and Neuromyelitis Optica

2019 ◽  
Author(s):  
khaled Deeb ◽  
Jessika Dold ◽  
Jose Labault-Santiago
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Neuromyelitis Optica ◽  
Rna Polymerase Iii ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Clinical Findings ◽  
Therapeutic Interventions ◽  
High Dose ◽  
Scleroderma Renal Crisis

Abstract INTRODUCTION: The objective of this study is to report a case of a 44-year-old female who presented with intractable hiccups, vomiting, and later complicated with paraplegia. Imaging and sero/immunological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity and transverse myelitis on MRI. Further investigation revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes, namely systemic sclerosis and neuromyelitis optica, and their underlying clinical manifestations and therapeutic interventions, are seldom reported in literature and are worth reporting. CASE REPORT: The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified as steroid induced scleroderma renal crisis on renal biopsy. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received consent forms from the participant in our study, and we have them on file in case they are requested. We have also received patient’s consent for the data presented in this article and Figure 1. CONCLUSION: This report expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.

scholarly journals Scleroderma nephropathy: unsolved problems

2021 ◽  
Vol 7 (5) ◽  
pp. 5-18
Author(s):  
I. E. Bulavko ◽  
E. V. Timofeev ◽  
K. J. U. Alkak ◽  
V. A. Isakov
Keyword(s):  
Risk Factors ◽  
Blood Pressure ◽  
Systemic Sclerosis ◽  
Rna Polymerase Iii ◽  
Kidney Injury ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme Inhibitors ◽  
Treatment Approaches ◽  
Renal Crisis

Kidney injury is a common pathology in the group of patients with systemic sclerosis. At least half of the patients show histological signs of it. Acute condition is known as scleroderma renal crisis. Although discussions regarding the risk factors for scleroderma renal crisis are open, most researchers consider the following factors: female sex, previous proteinuria and hypertension, the presence of anti-RNA polymerase III antibodies, and a decrease in lung diffusion capacity ≤75%. Diagnostic criteria for scleroderma renal crisis include an acute increase in blood pressure, accompanied by acute renal failure and abnormalities in the urinary sediment, anemia, and thrombocytopenia. Treatment of scleroderma renal crisis entails decreasing blood pressure, mainly with short-acting angiotensin-converting enzyme inhibitors, followed by selecting effective antihypertensive therapy. Further research of new treatment approaches is being carried on: the use of endothelin receptor antagonists (bosentan), monoclonal antibodies against the complement component 5 (eculizumab). Despite the approved strategies for identifying risk factors for scleroderma renal crisis development and treatment approaches, this group of patients is still characterized by high rates of mortality, the need for renal replacement therapy, and kidney transplantation. Thus, the problem of kidney injury in systemic sclerosis remains relevant.

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