Development of lupus nephritis in a patient with previous scleroderma renal crisis

Lupus ◽  
2018 ◽  
Vol 27 (10) ◽  
pp. 1732-1734 ◽  
Author(s):  
E McConville ◽  
D Smith ◽  
E Bélanger ◽  
C Ivory
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Overlap Syndrome ◽  
Scleroderma Renal Crisis ◽  
Undifferentiated Connective Tissue Disease ◽  
Laboratory Findings ◽  
Repeat Renal Biopsy ◽  
Nephrotic Range Proteinuria ◽  
Renal Crisis ◽  
Made In

We present a case of scleroderma overlap syndrome with systemic lupus erythematosus (SLE) including complications of both scleroderma renal crisis and lupus nephritis. Our patient was initially diagnosed with undifferentiated connective tissue disease in 1996. A diagnosis of scleroderma was made in 2010 after she developed scleroderma renal crisis. She remained stable until 2016, when she presented with Salmonella bacteremia, renal failure, nephrotic range proteinuria and microscopic hematuria. Laboratory findings were consistent lupus with positive ds-DNA, hypocomplementemia and repeat renal biopsy showed lupus nephritis.

Mesangial lupus nephritis with associated nephrotic syndrome.

1997 ◽  
Vol 8 (7) ◽  
pp. 1199-1204
Author(s):  
N Stankeviciute ◽  
W Jao ◽  
A Bakir ◽  
J P Lash
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
World Health ◽  
Clinical Tool ◽  
Laboratory Findings ◽  
Glomerular Lesion ◽  
Health Organization ◽  
Lupus Glomerulonephritis ◽  
Nephrotic Range Proteinuria

Patients with mesangial proliferative lupus glomerulonephritis (World Health Organization class II) are generally believed to have only mild to moderate proteinuria and normal renal function. However, there have been several reports of patients with mesangial lupus with nephrotic-range proteinuria. In this report, we present two additional cases and review the literature. Of seven reported cases, persistent nephrotic syndrome was observed in four, morphologic transformation occurred in three, and all but one presented with varying degrees of azotemia. These cases reinforce the concept that in systemic lupus erythematosus, laboratory findings may not correlate well with the underlying glomerular lesion, and therefore, the renal biopsy is an essential clinical tool in the approach to lupus nephritis.

scholarly journals A clinico-pathological study of lupus nephritis based on the International Society of Nephrology-Renal Pathology Society 2003 classification system

2017 ◽  
Vol 9 (03) ◽  
pp. 149-155
Author(s):  
Suchitha Satish ◽  
Pallavi Deka ◽  
Manjunath Sanjeev Shetty
Keyword(s):  
Lupus Nephritis ◽  
Renal Biopsy ◽  
International Society ◽  
Lupus Erythematosus ◽  
Classification System ◽  
Renal Involvement ◽  
Response To Therapy ◽  
Renal Pathology ◽  
Laboratory Findings ◽  
Presenting Symptoms

Abstract INTRODUCTION: Lupus nephritis (LN) is a major complication of systemic lupus erythematosus (SLE). Renal involvement is a major determinant of the prognosis of SLE. The histological classification of LN is a key factor in determining the renal survival of patients with LN. Prompt recognition and treatment of renal disease are important, as early response to therapy is correlated with better outcome and renal biopsy plays an important role in achieving this. OBJECTIVES: The objective of this study was to correlate the clinical and laboratory findings with histopathological classes of LN as per the 2003 International Society of Nephrology-Renal Pathology Society (ISN/RPS) classification system. PATIENTS AND METHODS: Fifty-six patients with SLE, undergoing a renal biopsy for renal dysfunction were studied. The comparison of data from multiple groups was made by Pearson’s Chi-square test and between two groups by independent samples t-test. The values of P < 0.05 were considered statistically significant. RESULTS: Of the 56 cases studied, 51 (91.1%) were females. The most common presenting symptoms were edema, arthralgia, and hypertension. Class IV (55.4%) was the most common class. Thirty-nine (69.6%) cases showed full house immunostaining. Hypertension, hematuria, proteinuria, and tu bulo-interstitial disease showed a significant correlation (P < 0.05) with ISN/RPS classification, 2003. CONCLUSION: Assessment and management of patients with suspected LN are greatly facilitated through information obtained by renal biopsy. Since renal morphology may predict long-term prognosis, and no clinical or laboratory feature uniformly predicts prognosis, it is important to study the constellation of features in LN for better patient management.

scholarly journals Systemic lupus erythematosus-myositis overlap syndrome with lupus nephritis

2020 ◽  
Vol 9 (4) ◽  
pp. 2104
Author(s):  
Biswajit Dey ◽  
Vandana Rapahel ◽  
Yookarin Khonglah ◽  
Md Jamil
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Overlap Syndrome ◽  
Systemic Lupus

scholarly journals Predominant Mesangial IgM, C3 and λ Light Chain Depositions and Interstitial Nephritis in a Patient with Overlap Syndrome and Positivity for Anti-Mitochondrial M2 Antibody: A Case Report

2021 ◽  
Author(s):  
Saeko Yamada ◽  
Hiroko Kanda ◽  
Hiroyuki Abe ◽  
Yukako Shintani Domoto ◽  
Ryochi Yoshida ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Renal Dysfunction ◽  
Interstitial Nephritis ◽  
Light Chain ◽  
Lupus Erythematosus ◽  
Plasma Cells ◽  
Overlap Syndrome ◽  
Pathological Findings ◽  
Glucocorticoid Treatment ◽  
Λ Light Chain

Abstract Overlap syndrome refers to a group of conditions that have clinical features of more than one well-characterized rheumatic disease and meet the respective classification criteria. There are no typical renal histological findings in overlap syndrome. When patients with overlap syndrome develop renal dysfunction, various potential causes, including lupus nephritis (LN), renal crisis by systemic sclerosis, interstitial nephritis and so on, need to be distinguished. Here we report a 44-year-old woman with overlap syndrome involving systemic lupus erythematosus (SLE), diffuse cutaneous systemic scleroderma and Sjogren’s syndrome, who was also positive for anti-mitochondrial M2 antibody. She developed glomerular hematuria, proteinuria and increase in creatinine appeared gradually. Suspecting lupus nephritis, renal biopsy was performed. However, in the interstitium, mild infiltration of lymphocytes and plasma cells and very partial fibrosis were observed. Immunofluorescence (IF) microscopy revealed predominant mesangial IgM, C3 and λ light chain staining. Overall, LN was not diagnosed based on these findings. Renal dysfunction was normalized by glucocorticoid treatment for three months. This case suggests the importance of a renal diagnosis based on renal pathological findings, especially in a case of overlap syndrome including SLE.

scholarly journals Systemic sclerosis complicated with renal thrombotic microangiopathy: a case report and literature review

2022 ◽  
Vol 23 (1) ◽  
Author(s):  
Weiwei Kong ◽  
Yaomin Wang ◽  
Huiping Wang ◽  
Qin Zhou ◽  
Jianghua Chen ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Thrombotic Microangiopathy ◽  
Connective Tissue Diseases ◽  
Kidney Injury ◽  
Renin Angiotensin System ◽  
Overlap Syndrome ◽  
Scleroderma Renal Crisis ◽  
Related Factors ◽  
Complex Deposition

Abstract Background Systemic sclerosis (SSc) may overlap with other connective tissue diseases, which is named overlap syndrome. Scleroderma renal crisis (SRC) is a rare but severe complication of SSc. SSc related thrombotic microangiopathy (SSc-TMA) is an infrequent pathology type of SRC, while SSc-TMA accompanied by overlap syndrome is very rare. Case presentation This study reported a case of acute kidney injury (AKI) accompanied with overlap syndrome of SSc, systemic lupus erythematosus (SLE) and polymyositis (PM). The renal pathology supported the diagnosis of SSc-TMA but not SLE or PM-related renal injury, characterized by renal arteriolar thrombosis, endothelial cells edema, little cast in tubules and mild immune complex deposition. The primary TMA related factors (ADAMTS13 and complement H factor) were normal. Thus, this case was diagnosed as secondary TMA associated with SSc. The patient was treated with renin angiotensin system inhibitors, sildenafil, supportive plasma exchange/dialysis, and rituximab combined with glucocorticoids. After 2 months of peritoneal dialysis treatment, her renal function recovered and dialysis was stopped. Conclusion This study presented a case of SSc-TMA with overlap syndrome. Rituximab can be used as a treatment option in patients with high SRC risk or already manifesting SRC.

scholarly journals Patterns of renal pathology in Chinese patients with systemic sclerosis undergoing renal biopsy at a tertiary medical center

2019 ◽  
Vol 48 (4) ◽  
pp. 030006051989445
Author(s):  
Xiao-yu Cao ◽  
He Liu ◽  
Dong Xu ◽  
Meng-tao Li ◽  
Qian Wang ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lupus Nephritis ◽  
Renal Biopsy ◽  
Renal Injury ◽  
Aristolochic Acid ◽  
Minimal Change ◽  
Chinese Patients ◽  
Scleroderma Renal Crisis ◽  
Minimal Change Nephropathy ◽  
Renal Crisis

Objective We investigated renal injury characteristics in Chinese patients with systemic sclerosis (SSc) who had undergone renal biopsy. Methods We searched the medical records of patients with SSc who were hospitalized at Peking Union Medical College Hospital between January 1990 and August 2019. We analyzed the clinical characteristics and pathological results of these patients. Results We identified 25 patients who had undergone renal biopsy. Of these patients, 10 had scleroderma renal crisis (SRC); one underwent renal biopsy twice (for diffuse mesangial proliferative glomerulonephritis and for SRC); two had antineutrophil cytoplasmic antibody-associated glomerulonephritis; one had immunoglobulin M nephropathy; one had minimal change nephropathy; seven had lupus nephritis; one had scleroderma renal crisis with comorbid lupus nephritis; and two had drug-related kidney injury (caused by aristolochic acid in one and D-penicillamine in the other). Acute tubular necrosis was observed in the patient taking oral aristolochic acid, while minimal change nephropathy was observed in the patient with D-penicillamine-induced renal injury. Conclusions SRC was the most commonly encountered renal damage in patients with SSc. We recommend biopsy for patients undergoing treatment for SRC who have persistent renal injury with proteinuria, regardless of hematuria. Rheumatologists in Eastern countries should be aware of aristolochic acid nephropathy.

scholarly journals OP0163 2019 UPDATE OF THE JOINT EUROPEAN LEAGUE AGAINST RHEUMATISM AND EUROPEAN RENAL ASSOCIATION–EUROPEAN DIALYSIS AND TRANSPLANT ASSOCIATION (EULAR/ERA-EDTA) RECOMMENDATIONS FOR THE MANAGEMENT OF LUPUS NEPHRITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 102.1-103
Author(s):  
A. Fanouriakis ◽  
M. Kostopoulou ◽  
K. Cheema ◽  
H. J. Anders ◽  
M. Aringer ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
Oral Prednisone ◽  
Calcineurin Inhibitors ◽  
Induction Treatment ◽  
High Dose ◽  
Research Support ◽  
Nephrotic Range Proteinuria

Background:Up to 40% of systemic lupus erythematosus (SLE) patients develop kidney disease, which represents a major cause of morbidity.Objectives:To update the 2012 EULAR/ERA-EDTA recommendations for the management of lupus nephritis (LN).Methods:We followed the EULAR standardised operating procedures for the publication of treatment recommendations. Delphi-based methodology led to 15 questions for systematic literature review (SLR), which was undertaken by three fellows.Results:The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNI), and management of end-stage-kidney-disease (ESKD). The target of therapy is complete response (proteinuria <0.5-0.7gr/24h with [near-]normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2-3g/day, or mycophenolic acid at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500mg x6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3-0.5mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1g/24h despite renin-angiotensin-aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations.Conclusion:The updated recommendations intend to inform rheumatologists, nephrologists, patients, national professional societies, hospital officials, social security agencies and regulators about the treatment of LN based on most recent evidence.Disclosure of Interests:Antonis Fanouriakis Paid instructor for: Paid instructor for Enorasis, Amgen, Speakers bureau: Paid speaker for Roche, Genesis Pharma, Mylan, Myrto Kostopoulou: None declared, Kim Cheema: None declared, Hans-Joachim Anders: None declared, Martin Aringer Consultant of: Boehringer Ingelheim, Roche, Speakers bureau: Boehringer Ingelheim, Roche, Ingeborg Bajema Consultant of: GSK, John N. Boletis Grant/research support from: GSK, Pfizer, Paid instructor for: GSK, Abbvie, UCB, Enorasis, Eleni Frangou: None declared, Frederic Houssiau Grant/research support from: UCB, Consultant of: GSK, Jane Hollis: None declared, Alexandre Karras: None declared, Francesca Marchiori: None declared, Stephen Marks: None declared, Gabriela Moroni: None declared, Marta Mosca: None declared, Ioannis Parodis: None declared, Manuel Praga: None declared, Matthias Schneider Grant/research support from: GSK, UCB, Abbvie, Consultant of: Abbvie, Alexion, Astra Zeneca, BMS, Boehringer Ingelheim, Gilead, Lilly, Sanofi, UCB, Speakers bureau: Abbvie, Astra Zeneca, BMS, Chugai, GSK, Lilly, Pfizer, Sanofi, Josef S. Smolen Grant/research support from: AbbVie, AstraZeneca, Celgene, Celltrion, Chugai, Eli Lilly, Gilead, ILTOO, Janssen, Novartis-Sandoz, Pfizer Inc, Samsung, Sanofi, Consultant of: AbbVie, AstraZeneca, Celgene, Celltrion, Chugai, Eli Lilly, Gilead, ILTOO, Janssen, Novartis-Sandoz, Pfizer Inc, Samsung, Sanofi, Vladimir Tesar: None declared, Maria Trachana: None declared, Ronald van Vollenhoven Grant/research support from: AbbVie, Amgen, Arthrogen, Bristol-Myers Squibb, GlaxoSmithKline (GSK), Janssen Research & Development, LLC, Lilly, Pfizer, Roche, and UCB, Consultant of: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo Bioscience, GSK, Janssen, Lilly, Medac, Merck, Novartis, Pfizer, Roche, UCB and Vertex, Speakers bureau: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo Bioscience, GlaxoSmithKline, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, UCB, Vertex, Alexandre Voskuyl: None declared, Y.K. Onno Teng Grant/research support from: GSK, Consultant of: GSK, Aurinia Pharmaceuticals, Novartis, Bernadette van Leeuw: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, Dimitrios Boumpas: None declared

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