Systemic sclerosis complicated with nephrotic syndrome: relevance with antiribosomal P antibody

Hirohata, Shunsei; Ohse, Toru; Takeuchi, Akiteru; Hashimoto, Takashi

doi:10.1007/s002960100131

Cited by 8 publications

(4 citation statements)

References 10 publications

(21 reference statements)

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“…Recent studies have suggested a direct participation of antiribosomal P antibody in the pathogenesis of lupus nephritis. 15,16 Hirohata et al 17 reported a case of SSc presenting with NS who tested positive for antiribosomal P antibody, which might have been involved in the development of NS. In this case, no such antibody was detected.…”

Section: Discussionmentioning

confidence: 97%

A case of mixed connective tissue disease presenting with nephrotic syndrome

et al. 2004

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A 70-year-old woman was admitted to our hospital for the treatment of diffuse scleroderma and marked edema in the lower extremities. Renal biopsy revealed membranous change, interstitial nephritis, and intimal hyperplasia of the small arteries. The patient was diagnosed as having mixed connective tissue disease (MCTD) presenting with nephrotic syndrome (NS). She responded well to a combination treatment consisting of methylprednisolone (m-PSL) pulse therapy, oral PSL, and cyclosporine A (CsA). We speculated on the actual pathogenesis of NS in this case of MCTD.

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Section: Discussionmentioning

confidence: 97%

A case of mixed connective tissue disease presenting with nephrotic syndrome

et al. 2004

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“…Hirohata et al . also reported a 39-year-old female with SSc who developed nephrotic syndrome (minimal change disease) four years later and was treated successfully with 60 mg of oral prednisolone daily without recurrence [ 9 ]. In yet another case in 2017, a 12-year-old female was diagnosed with nephrotic syndrome (FSGS) as the first manifestation of juvenile SSc [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

“…Renal involvement in SSc generally manifests with arterial hypertension with or without proteinuria. Although nephrotic syndrome has been reported in some SSc cases, it is rare and typically occurs due to amyloidosis or certain drugs rather than being directly linked to SSc [ 9 , 10 ]. Herein, we report a case of diffuse SSc complicated with nephrotic syndrome (focal segmental glomerulosclerosis) due to its rarity and for a better outlook in the management of these cases.…”

Section: Introductionmentioning

confidence: 99%

Nephrotic syndrome due to focal segmental glomerulosclerosis complicating scleroderma: a case report

Mehdipour Dalivand,

Hadjiabbasi,

Ramezanzadeh

et al. 2024

J Med Case Reports

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Background Systemic scleroderma (SSc) is an insidious autoimmune connective tissue disorder with multiorgan involvement. Renal involvement is one of the important causes of morbidity and mortality in scleroderma; however, nephrotic syndrome is reported rarely in association with SSc. We present a patient with SSc who developed focal segmental glomerulosclerosis (FSGS) as a complication of scleroderma. Case presentation A 59 year old Caucasian female patient, with a known history of diffuse systemic sclerosis from 8 years, presented to our clinic with symptoms of anasarca and weight gain. Her physical examination was unremarkable except for periorbital and extremity edema. Her biochemistry assessment revealed decreased serum albumin levels and elevated serum creatinine levels. A renal biopsy was performed, which showed histopathological patterns of FSGS type of nephrotic syndrome. After administration of high doses of steroid and rituximab in the course of her treatment for 6 months, her symptoms and proteinuria were improved without the occurrence of scleroderma renal crises. Conclusion SSc is a complex multisystemic autoimmune disorder. SRC is the most prominent renal involvement in SSc, but other renal pathologies may also occur. Each patient should be precisely investigated since managing these renal conditions can differ significantly. Nephrotic syndrome is a rare complication of SSc, which could be managed with prompt diagnosis and steroid administration.

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“…Antibodies to p25/p23 characterize a subset within the ACA-positive SSc patients, which is strongly associated with SjS symptoms (47). A case report of nephrotic syndrome in a patient with lcSSc and anti-ribosomal P antibodies was found (70). The association of anti-HMGCR antibodies and necrotizing autoimmune myositis (NAM) is wellknown (71,72).…”

Section: Autoabs In Sscmentioning

confidence: 99%

Clinical significance of rare serum autoantibodies in rheumatic diseases: a systematic literature review

Ceribelli¹,

Isailovic²,

Santis³

et al. 2018

J. Lab. Precis. Med

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The identification of serum autoantibodies is central in the diagnosis of systemic autoimmune rheumatic disease (SARD), and an increasing number of specificities have been detected in the past years.This allows an early diagnosis in the active phases of diseases, with the identification of specific disease subsets that may ultimately improve the disease outcomes. Thanks to the use of old and new laboratory techniques that are becoming increasingly available worldwide, the number of rheumatic patients with a specific autoantibody is increasing and this is improving also our knowledge of disease trigger mechanisms.The paradigmatic example is the plethora of serum autoantibodies described in polymyositis and dermatomyositis, coined myositis-specific antibodies (MSA) which include antibodies directed against tRNA synthetases, anti-SRP, anti-Mi-2, and anti-TIF-1γ and can discriminate disease subtypes, particularly when associated with the risk of cancer. As a further example, anti-HMGCR antibodies have been reported in several studies in association with necrotizing autoimmune myositis that may follow statin use. To clarify the current knowledge on these rare specificities, we performed a systematic literature review. We focused on the main features associated to specific autoantibodies that are rarely identified in rheumatic disease, to increase the awareness and scientific knowledge on these autoantibodies in different ethnic groups worldwide. Inclusion criteriaObservational studies, case reports and clinical trials were included. Exclusion criteriaArticles not concerning SARD, and reviews or editorials in languages different from English, if including children or animals, were excluded to limit the literature review to adults and because no funding was available for translation. The selection process was performed by two authors, based on titles, abstracts and subsequently full text papers. Figure 1 represents the flowchart of the selection process of this systematic literature review. Data extractionThe year of publication, study design, number of patients and demographic data were recorded. The outcome was defined by the identification of rare autoAbs and their prevalence and clinical significance in SARD. Articles were divided into categories depending on the disease.

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Systemic sclerosis complicated with nephrotic syndrome: relevance with antiribosomal P antibody

Cited by 8 publications

References 10 publications

A case of mixed connective tissue disease presenting with nephrotic syndrome

A case of mixed connective tissue disease presenting with nephrotic syndrome

Nephrotic syndrome due to focal segmental glomerulosclerosis complicating scleroderma: a case report

Clinical significance of rare serum autoantibodies in rheumatic diseases: a systematic literature review

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