An Interesting Case of Nonlupus Full-House Nephropathy

Full-house immunofluorescence and endothelial tubuloreticular inclusions are known as characteristic features of lupus nephritis. However, both features are not pathognomonic for lupus nephritis. A kidney biopsy specimen showing full-house immunofluorescence pattern in the absence of autoantibodies and classical clinical features of Systemic Lupus Erythematosus (SLE) is now considered as nonlupus full-house nephropathy (FHN). Nonlupus FHN may be idiopathic or due to other disease processes known as secondary nonlupus FHN. Here, we report the case of a 36-year-old female who presented with nephrotic proteinuria with bland urine sediment. Additional analyses revealed normal serum antinuclear antibody (ANA), normal anti-double-stranded DNA (anti-dsDNA) antibodies, and normal serum C3 and C4 levels. A renal biopsy showed a normal-appearing glomerulus without any proliferation or capillary wall thickening and widespread glomerular immune deposits (full-house effect; IgA, IgG, IgM, C3, and C1Q) on direct immunofluorescence. Renal electron microscopy showed diffuse effacement of visceral epithelial cell foot processes and mesangial electron dense deposits. The patient was diagnosed as nonlupus FHN. There is a controversial role of steroids and other immunosuppressive drugs in the treatment of nonlupus FHN patients, but our case patient responded favourably to steroid therapy. The term nonlupus FHN can be used as an umbrella term for patients who do not satisfy the clinical and serological criteria of SLE.

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A patient with systemic lupus erythematosus and lupus nephritis: A 12-year follow-up

Vojnosanitetski pregled ◽

10.2298/vsp1108705j ◽

2011 ◽

Vol 68 (8) ◽

pp. 705-708

Author(s):

Natasa Jovanovic ◽

Jasmina Markovic-Lipkovski ◽

Stevan Pavlovic ◽

Biljana Stojimirovic

Keyword(s):

Systemic Lupus Erythematosus ◽

Nephrotic Syndrome ◽

Mycophenolate Mofetil ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Kidney Biopsy ◽

Immunosuppressive Drugs ◽

Systemic Lupus ◽

Younger Women ◽

The Right

Introduction. Systemic lupus erythematosus (SLE) is a chronic immunological disease causing a significant morbidity and mortality in younger women and involving several organs and systems, most often the kidneys, being consequently the incidence of lupus nephritis (LN) about 60%. Case report. We reported a 57 year-old patient with the diagnosed SLE in 1995. Pathohistological analysis of kidney biopsy revealed LN type V. The patient was treated with corticosteroid pulses and azathioprine during one year. A remission was achieved and maintained with prednisone, 15 mg daily. Nephrotic relapse was diagnosed in 2006 and the second kidney biopsy revealed recent kidney infarction due to extensive vasculitis. Soon, a cerebrovascul insult developed and CT-scan revealed endocranial infarctus. The patient was treated with corticosteroids and cyclophosphamide pulses (totally VI monthly pulses), and also with low-molecular heparine, anticoagulants and salicylates because of the right leg phlebothrombosis. After the pulses, the patient was adviced to take prednisone 20 mg daily and azothioprine 100 mg daily, and 6 months later mycophenolate mofetil because of persistent active serological immunological findings (ANA 1 : 320) and nephrotic syndrome. Mycophenolate mofetil was efficient in inducing and maintaining remission of nephrotic syndrome. Conclusion. The aim of LN treatment is to achieve and maintain remission, improve patients? outcome, reduce the toxicity of immunosuppressive drugs and the incidence of relapses. Mycophenolate mofetil was shown to be efficient in inducing and maintaining remission of nephrotic syndrome in the frame of LN.

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Major lupus organ involvement: severe lupus nephritis

Lupus ◽

10.1177/0961203310376522 ◽

2010 ◽

Vol 19 (12) ◽

pp. 1391-1398 ◽

Cited By ~ 16

Author(s):

Y. Avihingsanon ◽

N. Hirankarn

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Opportunistic Infections ◽

Early Recognition ◽

Asian Population ◽

Immunosuppressive Drugs ◽

First Episode ◽

Asian Patients ◽

Renal Flare ◽

Number Of Patients

Lupus nephritis is a common and severe complication of systemic lupus erythematosus. A number of patients have nephritis as a presenting feature that, in its severe form, can shortly lead to end-stage renal disease and/or death. Renal flare usually occurs a few years after the first episode and is remarkably predominant in the Asian population. Frequent monitoring for renal flare enhances early recognition and timely treatment. The mainstay therapy continues to be the prolonged use of cytotoxic/immunosuppressive drugs that have a number of undesirable effects, particularly ovarian failure and development of opportunistic infections. This review will focus on the pathogenesis and the unique genetic factors found in Asian patients with lupus nephritis. Here, we propose an appropriate management scheme for the treatment of lupus nephritis in Asian patients.

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Childhood-onset bullous systemic lupus erythematosus

Lupus ◽

10.1177/0961203314544187 ◽

2014 ◽

Vol 23 (13) ◽

pp. 1422-1425 ◽

Cited By ~ 16

Author(s):

D M R Lourenço ◽

R Cunha Gomes ◽

N E Aikawa ◽

L M A Campos ◽

R Romiti ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Severe Disease ◽

Immunosuppressive Drugs ◽

University Hospital ◽

Direct Immunofluorescence ◽

Childhood Onset ◽

Systemic Lupus

Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30–60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14–24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations.

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Class IV Lupus Nephritis in the Setting of Serologically Quiescent Disease and Normal Urine Sediment in a Patient with Late-Onset Systemic Lupus Erythematosus

Case Reports in Rheumatology ◽

10.1155/2019/1219529 ◽

2019 ◽

Vol 2019 ◽

pp. 1-6

Author(s):

Benedicta Nnodum ◽

Lauren Dudley

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Late Onset ◽

The Body ◽

Systemic Lupus ◽

Urine Sediment ◽

Reactive Protein ◽

Normal Urine ◽

Class Iv

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. Lupus nephritis (LN) is a frequent and serious complication of SLE. We report a case of an 80-year-old woman who was initially diagnosed with late-onset SLE and eventually developed LN in the setting of normal complements, double-stranded DNA, C-reactive protein, erythrocyte sedimentation rate, and urine sediment. She developed abnormal renal function (creatinine of 1.7 mg/dl) and mild proteinuria (1-2+) without hematuria. Renal biopsy showed class IV lupus glomerulonephritis, active and chronic. The patient was started on mycophenolate mofetil which led to improvement of proteinuria and stabilization of creatinine. The suspicion for LN in a patient with late-onset SLE should remain high when there is development of suspicious renal or urinary abnormalities even if laboratory values do not suggest high disease activity and urinary sediment is normal. To our knowledge, this is one of the oldest patients with biopsy-proven LN and late-onset SLE.

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Secondary angle-closure glaucoma as an ocular presentation of systemic lupus erythematosus: a case report

Journal of International Medical Research ◽

10.1177/0300060520959492 ◽

2020 ◽

Vol 48 (9) ◽

pp. 030006052095949

Author(s):

Xincen Hou ◽

Wenping Pan ◽

Anli Wang ◽

Tao Yu ◽

Aiping Song

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Immunosuppressive Drugs ◽

Acute Angle ◽

Angle Closure Glaucoma ◽

Angle Closure ◽

High Dose ◽

Systemic Lupus

Systemic lupus erythematosus (SLE) is a chronic idiopathic autoimmune disease. SLE can involve almost any part of the eyes. However, bilateral angle-closure glaucoma due to lupus choroidopathy that is accompanied by polyserositis and nephropathy is rare. We report a 21-year-old woman whose clinical manifestations were diagnosed as bilateral angle-closure glaucoma caused by ciliochoroidal effusion. Subsequently, SLE and lupus nephritis were diagnosed on the basis of malar rash, photosensitivity, proteinuria, positive anti-Smith and anti-DNA antibodies, and a renal histopathological biopsy. After 1 month of treatment with steroids and immunosuppressive drugs, the patient’s intraocular pressure returned to normal, visual acuity improved, and lupus nephritis was effectively controlled. Bilateral secondary acute angle closure caused by SLE choroidal disease can be an ocular manifestation of SLE, and is usually accompanied by polyserositis and nephropathy. High-dose steroids and immunosuppressive therapy should be immediately and actively provided for this condition.

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Successful Treatment of Hemophagocytic Lymphohistiocytosis Associated with Lupus Nephritis by Using Mycophenolate Mofetil

Case Reports in Rheumatology ◽

10.1155/2017/4159727 ◽

2017 ◽

Vol 2017 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Takashi Nawata ◽

Makoto Kubo ◽

Kosaku Shiragami ◽

Yukinori Nakamura ◽

Masafumi Yano

Keyword(s):

Systemic Lupus Erythematosus ◽

Mycophenolate Mofetil ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Hemophagocytic Lymphohistiocytosis ◽

Corticosteroid Treatment ◽

Immunosuppressive Drugs ◽

Japanese Woman ◽

Systemic Lupus ◽

Old Japanese

An estimated 0.9% to 2.4% of patients with systemic lupus erythematosus (SLE) also have hemophagocytic lymphohistiocytosis (HLH). HLH associated with autoimmune diseases is often refractory to corticosteroid treatment; thus, additional immunosuppressive drugs, such as cyclosporine, cyclophosphamide, or tacrolimus, are required. Here, we describe the case of a 44-year-old Japanese woman who developed HLH associated with lupus nephritis. Initially, her HLH was refractory to treatment with a corticosteroid, tacrolimus, and mizoribine. However, alternative treatment with a corticosteroid, mycophenolate mofetil, and tacrolimus improved both her HLH and lupus nephritis. This case suggests the possibility of mycophenolate mofetil as a key drug for treating HLH associated with SLE.

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Treatments for Lupus Nephritis: A Systematic Review and Network Metaanalysis

The Journal of Rheumatology ◽

10.3899/jrheum.160041 ◽

2016 ◽

Vol 43 (10) ◽

pp. 1801-1815 ◽

Cited By ~ 13

Author(s):

Jasvinder A. Singh ◽

Alomgir Hossain ◽

Ahmed Kotb ◽

Ana Oliveira ◽

Amy S. Mudano ◽

...

Keyword(s):

Systematic Review ◽

Herpes Zoster ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Standard Dose ◽

Immunosuppressive Drugs ◽

High Dose ◽

Endstage Renal Disease ◽

Credible Intervals ◽

Risk Of Malignancy

Objective.To compare benefits and harms of lupus nephritis (LN) induction and maintenance treatments.Methods.We performed a systematic review and Bayesian network metaanalyses of randomized controlled trials (RCT) of immunosuppressive drugs or corticosteroids (CS) in LN. OR and 95% credible intervals (CrI) were calculated.Results.There were 65 RCT that met inclusion and exclusion criteria. Significantly lower risk of endstage renal disease (ESRD; 17 studies) was seen with cyclophosphamide (CYC; OR 0.49, 95% CrI 0.25–0.92) or CYC + azathioprine (AZA; OR 0.18, 95% CrI 0.05–0.57) compared with standard-dose CS, and with high-dose (HD) CYC (OR 0.16, 95% CrI 0.03–0.61) or CYC + AZA (OR 0.10, 95% CrI 0.03–0.34) compared with HD CS. HD CS was associated with higher risk of ESRD compared with CYC (OR 3.59, 95% CrI 1.30–9.86), AZA (OR 2.93, 95% CrI 1.08–8.10), or mycophenolate mofetil (MMF; OR 7.05, 95% CrI 1.66–31.91). Compared with CS, a significantly higher proportion of patients had renal response (14 studies) when treated with CYC (OR 1.98, 95% CrI 1.13–3.52), MMF (OR 2.42, 95% CrI 1.27–4.74), or tacrolimus (TAC; OR 4.20, 95% CrI 1.29–13.68). No differences were noted for the risk of malignancy (15 studies). The risk of herpes zoster (17 studies) was as follows: OR (95% CrI) MMF versus CS 4.38 (1.02–23.87), CYC versus CS 6.64 (1.97–25.71), TAC versus CS 9.11 (1.13–70.99), and CYC + AZA versus CS 8.46 (1.99–43.61).Conclusion.Renal benefits and the risk of herpes zoster were higher for immunosuppressive drugs versus CS. Data on relative and absolute differences are now available, which can be incorporated into patient-physician discussions related to systemic lupus erythematosus medication use.

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Evaluation of urinary FOXP3 mRNA as a biomarker of lupus nephritis in Egyptian patients with systemic lupus erythematosus

Lupus ◽

10.1177/09612033211030559 ◽

2021 ◽

pp. 096120332110305

Author(s):

Ahmed Fayed ◽

AbdelAal Mohamed ◽

Reham Abdelghany Ahmed ◽

Sameh Abouzeid ◽

Ahmed Soliman ◽

...

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Messenger Rna ◽

Activity Index ◽

Mrna Level ◽

Mrna Levels ◽

Urine Sediment ◽

Foxp3 Mrna ◽

Regulatory T Lymphocytes ◽

Severity Of The Disease

Aim Lupus nephritis (LN) is one of the most serious complications of SLE. Tregs (Regulatory T lymphocytes) are thought to play a part in the pathogenesis of SLE. According to recent research, Foxp3, a Treg identification marker, plays a significant role in the pathogenesis of SLE. This study aimed to compare the urinary Foxp3 mRNA levels of patients with active and inactive forms of LN and healthy control subjects to see whether it played a role in disease activity. Methods We measured FOXP3 messenger RNA (mRNA) expression in the urine of 50 people with active LN, 50 people with inactive lupus, and 50 healthy people. Results We found that the expression level of FOXP3 was significantly higher in urine from patients with active LN than from subjects with inactive lupus and healthy controls (22.93 ± 4.13 vs 5.66 ± 0.47 vs 0.57 ± 0.15copy; P < 0.001). Urinary FOXP3 mRNA level significantly correlated with SLEDAI (0.000057) In the active group, urinary FOXP3 mRNA level also significantly correlated with histological activity index (< 0.00001). Conclusion We concluded that urinary FOXP3 mRNA is elevated in patients with active LN and that it is linked to the SLEDAI and the severity of the disease. FOXP3 mRNA in urine sediment may be used as a non-invasive biomarker for evaluating the severity of LN and risk stratification.

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Bullous Systemic Lupus Erythematosus and Membranous Lupus Nephritis in a Filipino Child

Acta Medica Philippina ◽

10.47895/amp.v53i1.226 ◽

2019 ◽

Vol 53 (1) ◽

Author(s):

Marc Andrew O. Perez ◽

Candice B. Brillante ◽

Lourdes Paula R. Resontoc ◽

Dolores D. Bonzon ◽

Francisco E. Anacleto ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Basement Membrane ◽

Lupus Erythematosus ◽

Plasma Cells ◽

Kidney Biopsy ◽

Direct Immunofluorescence ◽

Systemic Lupus ◽

Cutaneous Manifestations ◽

Membranous Lupus Nephritis

Bullous eruptions are rare cutaneous manifestations of systemic lupus erythematosus. We report a case of an 8-year old Filipino girl with vesiculobullous systemic lupus erythematosus (SLE) and membranous lupus nephritis on kidney biopsy who presented with clinical nephrotic features of generalized edema, proteinuria, hypoalbuminemia and hyperlipidemia. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE were met. Immunohistopathologic examination of the skin lesion revealed a sub-epidermal split with neutrophilic infiltrates along the dermo-epidermal junction, moderate perivascular, periadnexal and interstitial infiltrates composed of predominantly neutrophils with neutrophilic dusts, lymphocytes, plasma cells, rare eosinophils and increased dermal mucin. Direct immunofluorescence showed strong continuous linear IgG deposits along the basement membrane and weak linear IgM and IgA deposition along the basement membrane zone (BMZ). To our knowledge, this is the first report of vesiculobullous SLE in a Filipino child. This case is a rare form of cutaneous lupus in children. Bullous SLE (BSLE) should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.

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Characteristics of systemic lupus erythematosus and lupus nephritis in children in the Republic of Belarus and the Republic of Tatarstan

Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) ◽

10.21508/1027-4065-2019-64-5-199-203 ◽

2019 ◽

Vol 64 (5) ◽

pp. 199-203

Author(s):

T. P. Makarova ◽

A. V. Sukalo ◽

I. A. Kazyro ◽

Yu. S. Melnikova ◽

N. N. Firsova ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Immunosuppressive Drugs ◽

Systemic Lupus ◽

American College Of Rheumatology ◽

Severe Manifestation ◽

Extrarenal Manifestations ◽

The Republic

Systemic lupus erythematosus is an autoimmune disease characterized by a pronounced polymorphism of clinical manifestations. Lupus nephritis is the most severe manifestation of the disease. The article presents a retrospective analysis of the cases of systemic lupus erythematosus and assessment of the clinical manifestations of the disease and variants of lupus nephritis in children in the Republics of Belarus and Tatarstan. The authors analyzed 60 cases of systemic lupus erythematosus, lupus nephritis. All patients had at least 4 of the 11 diagnostic criteria proposed by the American College of rheumatology (ACR, 1997), and 35 patients had a morphologically verified nephritis. It was found that the disease in children developed very actively with fast multi-organ involvement and it required aggressive therapy with several immunosuppressive drugs. During follow-up, the percentage of patients with renal damage increased, so renal function should be controlled in all patients with systemic lupus erythematosus, especially with early onset. Lupus nephritis is combined with extrarenal manifestations and it is difficult to diagnose when it begins with kidney damage. The overall survival rate of children with systemic lupus erythematosus is closely related to the severity of renal manifestations. Lupus nephritis is a serious problem that requires early aggressive intervention and continuous maintenance therapy.

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