scholarly journals Isolated end-stage renal disease in Sjögren’s syndrome due to immune complex mediated glomerulonephritis

2018 ◽  
Vol 2018 (12) ◽  
Author(s):  
Shashank Agarwal ◽  
Jaishvi Eapen ◽  
Ming Wu ◽  
Bruce Garner ◽  
Adib Alhaddad
Keyword(s):  
Renal Disease ◽  
Sjögren’S Syndrome ◽  
Immune Complex ◽  
End Stage Renal Disease ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Stage Renal Disease ◽  
End Stage ◽  
Sjögren‘S Syndrome

scholarly journals Pregnancy outcomes in a patient with Sjögren's disease with renal involvement

2013 ◽  
Vol 6 (4) ◽  
pp. 175-178
Author(s):  
Abhishek Joshi ◽  
Keelin O'Donoghue ◽  
Uzma Mahmood ◽  
Debasish Banerjee ◽  
Anita Banerjee
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Renal Disease ◽  
Sjögren’S Syndrome ◽  
Pregnancy Outcomes ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
End Stage ◽  
Sjögren‘S Syndrome

Background: Maternal and fetal outcome in pregnancy with renal failure is unpredictable, where each condition can adversely affect the other. We present a case of steroid sensitive Sjögren's nephritis worsened by pregnancy, demonstrated over the course of multiple pregnancies and investigated the aetiology. Case: A 28-year-old nullipara with a diagnosis of primary Sjögren's syndrome presented with a deterioration of renal function. A diagnosis of secondary tubulo-interstitial nephritis was made on renal biopsy. Her first pregnancy ended in the second trimester with a decision to deliver a female infant at 27 weeks due to worsening maternal renal function. Renal function improved immediately. A second pregnancy ended in a first trimester miscarriage. The third and fourth pregnancies delivered male infants at 35 and 34 weeks, with worsening renal function in each pregnancy, reaching end stage. Repeat biopsy showed extensive glomerulosclerosis and male cells were identified. Conclusions: This case of Sjögren's syndrome with renal disease demonstrated the increased risk of fetal and maternal adverse pregnancy outcomes. Renal function worsened in each pregnancy and progressed to end-stage renal disease. Fetal microchimerism offers an interesting mechanism for our patient's renal failure and its apparent relationship to her pregnancies.

scholarly journals FRI0182 RISK FACTORS ASSOCIATED WITH RENAL INVOLVEMENT IN PRIMARY SJÖGREN’S SYNDROME: DATA FROM THE SPANISH SJÖGRENSER COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 674.3-675
Author(s):  
J. Narváez ◽  
C. Sánchez-Piedra ◽  
M. Fernandez Castro ◽  
V. Martinez Taboada ◽  
A. Olive ◽  
...  
Keyword(s):  
Risk Factors ◽  
Renal Disease ◽  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Renal Involvement ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
Sjögren‘S Syndrome

Objectives:To investigate the prevalence, risk factors, and effects of primary renal disease on morbidity and mortality in patients with primary Sjögren’s syndrome (pSS).Methods:All patients in the SJÖGRENSER (registry of adult SSp patients of the Spanish Society of Rheumatology, cross-sectional phase) cohort were retrospectively investigated for the presence of clinically significant renal involvement directly related to pSS activity.Results:Of the 437 patients investigated, 39 (9%) presented overt renal involvement during follow-up. Severe renal disease necessitating kidney biopsy was relatively rare (23%).Renal involvement may complicate pSS at any time during the disease course and is associated with severe disease (indicated by higher scores of involvement, activity, and damage), systemic multiorgan involvement, and a higher frequency of lymphoma. Multivariate analysis showed that older age (odds ratio [OR] 1.03, 95% confidence interval [CI] 1.00–1.07), higher European League Against Rheumatism Sjogren’s Syndrome Disease Activity Index scores (OR 1.1, CI 1.03–1.18), serum anti-La/SSB positivity (OR 6.44, CI 1.36–30.37), and non-vasculitic cutaneous involvement (OR 8.64, 1.33–55.90) were independently associated with this complication.Chronic renal failure developed in 23 of 39 patients (59%); only 1 of them progressed to end-stage renal disease necessitating renal replacement therapy. Patients with renal disease showed higher Sjögren’s syndrome disease damage index scores (SSDDI), higher rates of hospitalization due to disease activity and higher rates of clinically relevant comorbidities.Conclusion:Renal involvement is an uncommon complication in pSS that was observed in 9% of patients. Although categorized as a non-negligible comorbidity, this condition shows a favorable prognosis.Disclosure of Interests:None declared

scholarly journals Clinical studies of renal disease in Sjogren's syndrome.

1987 ◽  
Vol 46 (10) ◽  
pp. 768-772 ◽  
Author(s):  
S Shiozawa ◽  
K Shiozawa ◽  
S Shimizu ◽  
M Nakada ◽  
T Isobe ◽  
...  
Keyword(s):  
Renal Disease ◽  
Sjögren’S Syndrome ◽  
Clinical Studies ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

scholarly journals Renal Involvement In Primary Sjögren’s Syndrome: Data From The Spanish Sjögrenser Cohort.

2020 ◽  
Author(s):  
Javier Narvaez ◽  
Carlos Sánchez-Piedra ◽  
Mónica Fernández-Castro ◽  
Víctor Martínez-Taboada ◽  
Alejando Olivé ◽  
...  
Keyword(s):  
Renal Disease ◽  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Renal Involvement ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
Primary Sjogren’S Syndrome ◽  
Sjögren‘S Syndrome

Abstract Objectives To investigate the prevalence, associated factors, and effects of primary renal disease on morbidity in patients with primary Sjögren’s syndrome (pSS).Methods All patients in the SJÖGRENSER (registry of adult SSp patients of the Spanish Society of Rheumatology) cohort were retrospectively investigated for the presence of clinically significant renal involvement directly related to pSS activity.Results Of the 437 patients investigated, 39 (9%) presented overt renal involvement during follow-up. Severe renal disease necessitating kidney biopsy was relatively rare (23%). Renal involvement may complicate pSS at any time during the disease course and is associated with severe disease (indicated by higher scores of involvement, activity, and damage), systemic multiorgan involvement, and a higher frequency of lymphoma. Multivariate analysis showed that older age (odds ratio [OR] 1.03, 95% confidence interval [CI] 1.00–1.07), higher European League Against Rheumatism Sjogren’s Syndrome Disease Activity Index scores (OR 1.1, CI 1.03–1.18), serum anti-La/SSB positivity (OR 6.65, CI 1.41–31.372), and non-vasculitic cutaneous involvement (OR 5.47, 1.03–29.02) were independently associated with this complication. Chronic renal failure developed in 23 of 39 patients (59%); only 1 of them progressed to end-stage renal disease necessitating renal replacement therapy. Patients with renal disease showed higher Sjögren’s syndrome disease damage index scores, higher rates of hospitalization due to disease activity and higher rates of clinically relevant comorbidities.Conclusion Renal involvement is an uncommon complication in pSS that was observed in 9% of patients. Although categorized as a non-negligible comorbidity, this condition shows a favorable prognosis.

End-stage renal failure in adolescence with Sjögren’s syndrome autoantibodies SSA and SSB

2007 ◽  
Vol 22 (10) ◽  
pp. 1793-1797 ◽  
Author(s):  
Sally Johnson ◽  
Sally-Anne Hulton ◽  
Marie-Anne Brundler ◽  
Celia Moss ◽  
Aarnoud Huissoon ◽  
...  
Keyword(s):  
Renal Failure ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
End Stage Renal Failure ◽  
End Stage ◽  
Sjögren‘S Syndrome

scholarly journals O12 Pulseless electrical activity arrest in a young woman: could renal tubular acidosis due to Sjögren’s syndrome be the underlying cause?

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ali El Rashid ◽  
Myrto Cheila ◽  
Karen Douglas
Keyword(s):  
Case Report ◽  
Renal Disease ◽  
Renal Biopsy ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Renal Involvement ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Renal Tubular ◽  
Sjögren‘S Syndrome

Abstract Case report - Introduction Distal renal tubular acidosis (RTA) type 1 is a rare condition in adults, which is characterised by impaired distal urine acidification. It is caused by failure of alpha intercalated cells and presents with alkaline urine (pH.5.5), metabolic acidosis, hypokalaemia, osteomalacia, nephrocalcinosis and hypercalciuria. Autoimmune diseases, such as Sjögren’s Syndrome (SS) and Rheumatoid Arthritis are among the most common causes. RTA can be the presenting feature of the rheumatic disease. SS is typically characterised by impaired lacrimal and salivary gland function but has a broad spectrum of symptoms and disease severity, which includes renal involvement in up to 40%. Case report - Case description We present the case of a 29-year-old female, referred to rheumatology by endocrinology following a recent emergency admission. The patient, who had woken with severe muscle paralysis of sudden onset, presented at A&E and imminently suffered a Pulseless Electrical Activity arrest. Urgent investigations identified profound hypokalaemia (serum potassium <1.5mmol/l), creatinine 120umol/l, a metabolic acidosis (bicarbonate 5.8 mmol/l), hypercalcemia (2.96 mmol/l) and hypermagnesemia (1.87mmol/l). She was successfully resuscitated and stabilised.  During her admission, under the care of endocrine and renal teams, a diagnosis of Distal RTA was reached. A CT scan showed medullary nephrocalcinosis. Immunology results: ANA 1:2560 (speckled pattern) and strongly positive Ro antibodies; polyclonal IgG of 19g/l, normal complement. Renal function returned to normal and she had negligible albuminuria. The causation of RTA was not certain. She was discharged well and on magnesium, potassium, and bicarbonate supplements. No renal biopsy was performed. Past medical history included a pregnancy resulting in c-section (intrauterine growth retardation), six months previously. The baby was normal but post-partum the patient remained unwell with fatigue, nausea, and vomiting. She was diagnosed with postnatal depression. She had no other significant medical history but since the admission had suffered from a pneumonia and hearing loss due to recurrent otitis media.  As SS was considered as a possible cause of RTA, she was referred for a rheumatological opinion. She was fatigued but denied sicca symptomatology (Schirmer’s test was negative, and normal saliva production). She had a patch of alopecia, but no other classical features of SS. Salivary glands ultrasound were unremarkable. Her biochemistry was now normal except a low normal bicarbonate; inflammatory markers were within normal range.  Case report - Discussion This patient presents several challenges. She represents a common scenario of the balance between the high clinical suspicion and the failure to meet the ACR/EULAR diagnostic criteria for SS and the resultant management dilemma. She has no sicca symptoms, normal salivary gland US and hence biopsy was not sought. Anti-Ro/SSA and anti-La/SSB antibodies are detected on average 5 years before the appearance of an overt clinical phenotype of SS and thus serve also as predictive markers of the disease. In this patient with a strongly positive speckled ANA with anti RO along with RTA1(and renal microcalcification indicating long duration) without other explanation is almost certainly SS. The absence of renal biopsy in this patient also added to the challenge of clarification of her renal pathology. Renal involvement  in SS is divided into: Overt glomerular disease which presents with haematuria, proteinuria or frank nephrotic syndrome; and Covert  tubular disease which manifests  with the metabolic abnormalities due to: proximal tubular injury; distal RTA or urinary concentrating defects.  The prevalence of overt (glomerular) renal disease in SS is 4.3-5%. The prevalence of covert disease is 10-42% (Proximal tubular injury 10-42%; Distal RTA 5-24%; urinary concentrating defect 17-28%). Type1 RTA is confirmed by inappropriately alkaline urine (PH > 5.3) during systemic acidosis. The acidosis may occur spontaneously or be induced by an acid load test (NH4CL). Also, by stimulating intercalated cell acid secretion using a combination of fludrocortisone and furosemide. In tubular disease, as in this patient, treatment to correct the metabolic abnormalities (with e.g. Bicarbonate) is normally required. However, as tubular interstitial nephritis (TIN) is the usual cause for renal tubular defects there is evidence that these patients respond to immunosuppression. Monitoring of long-term effects, such as on bone metabolism, is required and thus patients warrant input from a multidisciplinary team for management.  Case report - Key learning points This case is a reminder of the considerable prevalence of renal involvement in Sjögren’s Syndrome. TIN is a typical manifestation of SS, and we should keep in mind the possible evolution to Distal RTA. Our routine clinical work up for SS includes a urine dip and we may focus on proteinuria and haematuria to monitor for glomerular disease and ignore the pH. We perhaps ought to routinely check calcium, magnesium bicarbonate in addition to renal function in patients with SS. A plain abdominal X Ray would detect nephrocalcinosis in chronic RTA. Beware nausea and vomiting may be a warning sign of tubular renal disease.  A renal biopsy is critical in the evaluation of renal disease in SS. Our renal team is reluctant to biopsy this patient. Thus, with the lack of tissue diagnosis, the patient not fulfilling ACR/EULAR criteria and the history of significant recent infections has made the introduction of immunosuppression difficult in this young woman.

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