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 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.

The potential role of the eGFR in differentiating between true and pseudohyperkalaemia

2020 ◽  
Vol 57 (6) ◽  
pp. 444-455
Author(s):  
Thomas G Morris ◽  
Sushmita Lamba ◽  
Thomas Fitzgerald ◽  
Gary Roulston ◽  
Helen Johnstone ◽  
...  
Keyword(s):  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Filtration Rate ◽  
Blood Count ◽  
Potential Role ◽  
Estimated Glomerular Filtration Rate ◽  
Full Blood Count ◽  
Cut Points

Background Differentiating between true and pseudohyperkalaemia is essential for patient management. The common causes of pseudohyperkalaemia include haemolysis, blood cell dyscrasias and EDTA contamination. One approach to differentiate between them is by checking the renal function, as it is believed that true hyperkalaemia is rare with normal function. This is logical, but there is limited published evidence to support it. The aim of this study was to investigate the potential role of the estimated glomerular filtration rate in differentiating true from pseudohyperkalaemia. Methods GP serum potassium results >6.0 mmol/L from 1 January 2017 to 31 December 2017, with a repeat within seven days, were included. Entries were retrospectively classified as true or pseudohyperkalaemia based on the potassium reference change value and reference interval. If the initial sample had a full blood count, it was classified as normal/abnormal to remove blood cell dyscrasias. Different estimated glomerular filtration rate cut-points were used to determine the potential in differentiating true from pseudohyperkalaemia. Results A total of 272 patients were included with potassium results >6.0 mmol/L, with 145 classified as pseudohyperkalaemia. At an estimated glomerular filtration rate of 90 ml/min/1.73 m2, the negative predictive value was 81% (95% CI: 67–90%); this increased to 86% (95% CI: 66–95%) by removing patients with abnormal full blood counts. When only patients with an initial potassium ≥6.5 mmol/L were included (regardless of full blood count), at an estimated glomerular filtration rate of 90 ml/min/1.73 m2, the negative predictive value was 100%. Lower negative predictive values were seen with decreasing estimated glomerular filtration rate cut-points. Conclusion Normal renal function was not associated with true hyperkalaemia, making the estimated glomerular filtration rate a useful tool in predicting true from pseudohyperkalaemia, especially for potassium results ≥6.5 mmol/L.

The patient with scleroderma

2015 ◽  
Author(s):  
Bernadette Lynch ◽  
Aine Burns
Keyword(s):  
Blood Pressure ◽  
Systemic Sclerosis ◽  
Raynaud’S Phenomenon ◽  
Systemic Disease ◽  
Raynaud's Phenomenon ◽  
Scleroderma Renal Crisis ◽  
High Morbidity ◽  
Skin Involvement ◽  
Early Management ◽  
Renal Crisis

Scleroderma is tightness, thickening, and non-pitting induration of skin. Two forms of the skin disease are described. Limited cutaneous systemic sclerosis (lcSSc) which occurs distal to the wrists (or ankles) and/or over the face and neck, often associated with longstanding Raynaud’s phenomenon, and diffuse cutaneous systemic sclerosis (dcSSc) where truncal as well as acral skin involvement occurs as well as tendon friction rubs. In this latter condition the onset of the skin changes occurs within 1 year of onset of Raynaud’s phenomenon; however, the skin involvement may precede onset of vascular symptoms.The skin manifestations are the outward manifestation of a systemic disease, systemic sclerosis. Lung, heart, and gut involvement are frequent. Scleroderma renal crisis, usually presenting as accelerated hypertension and acute kidney injury, is one of the most severe complications of this disease. Autoantibodies against RNA polymerase are associated with scleroderma renal crisis. It occurs in 12% of dcSSc and 2% of lcSSc patients (men and women) and carries a high morbidity and mortality although careful supportive care and blood pressure management using angiotensin converting enzyme inhibitors (ACEI) or angiotensin-II receptor blockers have improved short-term outcomes. In general, beta blockers should be avoided in the early management.Approximately two-thirds of patients require dialysis, of these many recover enough function to come off dialysis. Higher blood pressure and younger age at presentation have a better prognosis. ACEIs should be continued even after dialysis is established as the latter increases the chance of late recovery. Average time to coming off dialysis is 11 months but recovery is uncommon after 24 months. After a crisis renal function continues to improve for several years.

Effect of inhibition of MAO and COMT on intrarenal dopamine and serotonin and on renal function

2001 ◽  
Vol 280 (1) ◽  
pp. R248-R254 ◽  
Author(s):  
Yongqing Wang ◽  
Theresa J. Berndt ◽  
Jennifer M. Gross ◽  
Michael A. Peterson ◽  
Mathew J. So ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Monoamine Oxidase ◽  
Filtration Rate ◽  
Interstitial Fluid ◽  
Fractional Excretion ◽  
Urine Flow ◽  
Arterial Blood ◽  
Fractional Excretion Of Sodium

The purpose of the present investigation was to study the effects of inhibition of monoamine oxidase (MAO) and/or catechol- O-methyltransferase (COMT), enzymes involved in the degradation of dopamine (DA) and serotonin (5-HT), on intrarenal DA and 5-HT, as reflected in the renal interstitial fluid (RIF) microdialysate and urine, and on renal function. Inhibition of MAO selectively increased RIF 5-HT from 3.16 ± 0.38 to 8.03 ± 1.83 pg/min ( n = 7, P < 0.05), concomitant with decreases in mean arterial blood pressure and glomerular filtration rate (2.09 ± 0.18 to 1.57 ± 0.22 ml/min, n = 7, P < 0.05). Inhibition of COMT significantly increased RIF DA (3.47 ± 0.70 to 8.68 ± 1.96 pg/min, n = 9, P < 0.05), urinary DA (2.00 ± 0.16 to 2.76 ± 0.26 ng/min, n = 9, P < 0.05), and absolute excretion of sodium (6.42 ± 2.00 to 9.82 ± 1.62 μmol/min, n = 10, P < 0.05). Combined inhibition of MAO and COMT significantly increased RIF DA, urinary DA, and urinary 5-HT, which was accompanied with increases in urine flow rate, and absolute (3.03 ± 0.59 to 8.40 ± 1.61 μmol/min, n = 9, P < 0.01) and fractional excretion of sodium. We conclude that inhibition of MAO selectively increases RIF 5-HT. COMT appears to be more important than MAO in the metabolism of intrarenal DA. Physiological increases in intrarenal DA/5-HT induced by inhibition of their degrading enzymes are accompanied with significant alterations of renal function.

scholarly journals AB0053 THE ROLE OF ANGIOPOIETIN-LIKE PROTEINS TYPES 3 AND 4 IN THE DEVELOPMENT OF RENAL DYSFUNCTION IN PATIENTS WITH RHEUMATOID ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1059.1-1059
Author(s):  
V. Aleksandrov ◽  
L. Shilova ◽  
A. Aleksandrov
Keyword(s):  
Rheumatoid Arthritis ◽  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Kidney Disease ◽  
Renal Dysfunction ◽  
Filtration Rate ◽  
Serum Concentrations ◽  
Group Iii ◽  
Group I

Background:Rheumatoid arthritis (RA) often contributes to the development of kidney disease. Angiopoietin-like proteins can be target markers for studying cardiorenal complications of RA [1].Objectives:Assessment of the correlation of serum concentrations of angiopoietin-like proteins types 3 and 4 (ANGPTL 3 and 4) with the progression of renal dysfunction in RA patients.Methods:114 patients with reliable RA (90.4% of women, 9.6% of men) aged 21 to 80 years (mean age 55.4 ± 11.2 years old), disease duration - 11.18 ± 9.03 years, positive for rheumatoid factor (RF-IgM) - 63.2%, positive for anti-citrullinated protein antibody (ACPA) - 59.7%) were examined. The laboratory examination included the determination of serum concentrations of angiopoietin-like protein type 3 (Human Angiopoietin-like Protein 3 ELISA, Bio Vendor, Czech Republic) and type 4 (RayBio Human ANGPTL4 ELISA Kit; RayBiotech, USA). To assess renal function in RA patients we used the calculated glomerular filtration rate (GFR) according to the CKD-EPI formula [2], taking into account the height and weight of a particular patient without indexing by body surface area. GFR values <60 ml / min / 1.73 m2 were regarded as a certain decrease, and GFR values from 60 to 89 ml / min / 1.73 m2 - as a slight decrease in global kidney function.Results:The concentration of ANGPTL3 in the blood serum of RA patients (n = 158) was 641.9 ± 224.5 ng / ml, and that of ANGPTL4 (n = 158) - 3.15 [0.77; 12.1] ng / ml. 74.7% (n = 118) were considered positive for the presence of ANGPTL3; 49.4% (n = 78) of RA patients were recognized as positive for the presence of ANGPTL4. The average glomerular filtration rate in RA patients was 74.0 ± 18.6 ml / min. More than а half of the examined RA patients had GFR ranging from 89 to 60 ml / min / 1.73 m2 (C1 - 21.5%; C2 - 58.9%; C3 - 19.6%). No sharp reduce of renal function (GFR <30 ml / min / 1.73 m2) corresponding to CKD C4-5 stages was recorded. Negative correlations of average strength were found between GFR and the level of ANGPTL 3 (r = -0.32, p <0.001) and ANGPTL 4 (rS = -0.31, p <0.001), as well as with age (rS = -0.28, p <0.001), the duration of RA (rS = -0.22, p = 0.005) and blood pressure increase (rS = -0.25, p = 0.001). On the basis of GFR measurements, patients were divided into three groups: group I - optimal renal function (> 90 ml / min); group II - a slight decrease in renal function (89-60 ml / min); group III - reduced renal function (<59 ml / min).Table 1.Content of ANGPTL 3 and 4 in RA patients with different GFR, ng / mlGroup I (n=34)Group II (n=93)Group III (n=31)ANGPTL 3533,4±161,7 I-III650,0±223,9733,2±244,1ANGPTL 40,77 [0,28;3,6] I-II, I-III3,3 [0,93;12,1]6,48 [1,52;19,3]Note: upper case indicates intergroup differences at p <0.05.There was a significant difference in the content of ANGPTL3 in patients of the first group and the patients of the third group (H-test = 6.55, p = 0.032) and ANGPTL4 in the group of patients with normal renal function (group I) and groups of RA patients with decreased GFR (group I- II: H-test = 10.7, p = 0.001; groups I-III: H-test = 20.1, p <0.001). ANGPTL4 indices also had intergroup differences (groups II-III: H-test = 7.2, p = 0.007) with GFR less than 90 ml / min.Conclusion:Chronic rheumatoid inflammation potentiates the development of renal dysfunction according to our data in 78.5% of patients. It is also accompanied by an increase in the content of ANGPTL types 3 and 4 in the blood of RA patients. A better understanding of the actions and mechanisms of ANGPTL may be of paramount importance for the development of effective ways of treatment for cardiorenal complications in RA.References:[1]Aleksandrov A., Aleksandrov V., Shilova L. Study of the role of angiopoietin-like protein type 4 in metabolic disorders caused by inflammation in rheumatoid arthritis. Ann Rheum Dis. 2020;79(s1):1341. doi: 10.1136/annrheumdis-2020-eular.4558.[2]KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl. 2013;3:1–150.Disclosure of Interests:None declared

scholarly journals Prognostic role of comorbidities in patients with pulmonary arterial hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A De Lorenzis ◽  
F Dardi ◽  
M Palazzini ◽  
E Zuffa ◽  
F Pasca ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Multivariate Analysis ◽  
Glomerular Filtration Rate ◽  
Pulmonary Arterial Hypertension ◽  
Filtration Rate ◽  
Systemic Blood Pressure ◽  
Prognostic Role ◽  
Systemic Blood ◽  
Pulmonary Arterial

Abstract Background Epidemiology of pulmonary arterial hypertension (PAH) is changing and the age at diagnosis and the prevalence of comorbidities are increasing but their prognostic relevance is substantially undefined. Purpose To evaluate the prognostic value of comorbidities in patients with PAH and in the different clinical subgroups. Methods All patients with PAH referred to a single centre underwent baseline right heart catheterization, brain natriuretic peptide (BNP) plasma levels, 6-min walking distance (6MWD), WHO functional class and anamnestic comorbidities evaluation. Cox regression model was used for analysis (p-value &lt;0.1 was considered for inclusion in multivariate analysis). Results 1311 patients were included [age 51 years; aetiology: 522 idiopathic/heritable/drug-induced (I/H/D)-PAH, 258 connective tissue disease (CTD)-associated PAH, 242 congenital heart disease (CHD)-associated PAH, 196 portal hypertension/HIV (PoHIV)-associated PAH and 93 pulmonary veno-occlusive disease (PVOD)]. 5% of patients have no comorbidities. At multivariate analysis comorbidities independently associated with prognosis are: systemic hypertension in I/H/D [HR 0.616, p=0.030], mean systemic blood pressure in CTD [HR 0.980, p=0.002] and PVOD [HR 0.962, p=0.006], dyslipidemia in CTD [HR 0.447, p=0.001] and PoHIV [HR 0.201, p=0.026], estimated glomerular filtration rate in PoHIV [HR 1.000, p&lt;0.001] and body mass index (BMI) [HR 0.966, p=0.069] in CTD. In CHD comorbidities are not independent determinants of prognosis. Other variables independently predictive of a worse prognosis are: advanced age in all PAH subgroups except PVOD; male gender in I/H/D; reduced 6MWD in I/H/D, CTD and PVOD; high BNP in I/H/D, CHD and PVOD; low cardiac index in CTD, high right atrial pressure in I/H/D and low mixed venous oxygen saturation in CHD. Conclusion The age at PAH diagnosis and the prevalence of comorbidities are increasing but their prognostic role seems of poor relevance as we found a protective role of these variables: high systemic blood pressure (maybe indicative of a better haemodynamic stability) in I/H/D, CTD and PVOD; dyslipidemia and high BMI (maybe indicative of a better nutritional status and a less severe autoimmune disease) in CTD; dyslipidemia and a high glomerular filtration rate (both indicative of a less severe liver disease) in PoHIV. Funding Acknowledgement Type of funding source: None

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.

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