Prolidase deficiency and systemic lupus erythematosus

Shrinath, M; Walter, John H.; Haeney, M R; Couriel, Jon; Lewis, Malcolm; Herrick, A.

doi:10.1136/adc.76.5.441

Cited by 73 publications

(56 citation statements)

References 12 publications

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“…Hypothetically, PD may cause the synthesis of antigenically immunoreactive, but biologically defective, C1q, that alters immune responses and thus predisposes to autoimmune disease (9). This association between SLE and prolidase deficiency has been reported previously in 7 cases (2 of our cases patient number 2 and 3 were previously briefly reported as part of a large series of PD patients) [7-10,16]. The clinical features and laboratory findings are presented in Table 1.…”

Section: Discussionsupporting

confidence: 77%

“…In reported cases of PD patients that developed SLE, most patients had some response to steroids and immunosuppressive therapy; however skin lesions were usually unresponsive to corticosteroid treatment. There were a few reports of mortalities in these patients, most of them related to infections [7-10]. …”

Section: Discussionmentioning

confidence: 99%

“…An association between prolidase deficiency and systemic lupus erythematosus has been previously reported in the literature [7-10]. Both prolidase deficiency and SLE are associated with disturbances in immune function and have many clinical features in common.…”

Section: Introductionmentioning

confidence: 94%

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Prolidase deficiency associated with systemic lupus erythematosus (SLE): single site experience and literature review

et al. 2012

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IntroductionProlidase deficiency (PD) is a rare autosomal recessive disorder which may have a wide spectrum of clinical features. These features include a characteristic facies, cognitive impairment, rashes or skin ulceration, splenomegaly, recurrent infections involving mainly the respiratory system, and iminodipeptiduria. The disorder is caused by a mutation in the PEPD gene.ObjectiveTo describe a cohort of unrelated PD patients from Northern Israel whose inborn error of metabolism was associated with systemic lupus erythematosus (SLE) and to identify in the medical literature all PD cases mimicked by and/or associated with SLE.MethodsThree patients with PD associated with SLE were clinically, biochemically and genetically investigated. These patients were from 3 unrelated consanguineous families residing in Northern Israel. A computer-assisted (PubMed) search of the medical literature from 1975 to 2011 was performed using the following key words: Prolidase deficiency, SLE, and systemic lupus erythematosus.ResultsAn association between PD and SLE was found in 10 PD patients. These 10 patients included three from our cohort of 23 PD patients, and seven out of just under 70 PD patients previously reported in the literature.ConclusionThe present findings underscore the relatively high incidence of the association between SLE and PD, suggesting that this association may not be coincidental. The phenotypic similarities between SLE and PD might suggest that the PEPD gene constitutes a modifier gene or a genetic risk factor in the causation of SLE.

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 99%

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Prolidase deficiency associated with systemic lupus erythematosus (SLE): single site experience and literature review

et al. 2012

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“…Serum prolidase activity was reported to be increased in patients with hypertension (Demirbag et al, 2007). Conversely, deficient/reduced serum prolidase activity was reported to be associated with lupuslike syndrome characterized by nonhealing skin ulcers owing to defective collagen turnover (Shrinath et al, 1997). Reduced serum prolidase activity was also reported in renal insufficiency (Evrenkaya et al, 2006) and uremia (Gejyo et al, 1983), because the kidney is the main source of prolidase (Liu et al, 2007).…”

Section: Discussionmentioning

confidence: 99%

The Association of Serum Prolidase Activity and Erectile Dysfunction

Savaş

Yeni

Çelik

et al. 2010

Journal of Andrology

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Prolidase is a cytosolic exopeptidase that cleaves iminodipeptides with carboxy-terminal proline or hydroxyproline and plays a major role in collagen turnover. Collagen is the essential content in atherosclerotic plaque, playing a key role in the stability/ instability and progression of endothelial dysfunction in the pathogenesis of erectile dysfunction (ED). Consequently, in this study we sought to determine serum prolidase activity and markers of oxidative stress, such as lipid hydroperoxide and total free sulfhydryl, in vasculogenic ED. We evaluated 92 patients with vasculogenic ED and 50 control subjects by clinical and laboratory investigations. We measured serum prolidase activity and serum total free sulfhydryl levels spectrophotometrically. Serum lipid hydroperoxide levels were determined with ferrous ion oxidationxylenol orange method. We assessed the association of serum prolidase activity with the presence and severity of vasculogenic ED and clinical characteristics, as well as laboratory parameters. We also assessed the association of serum prolidase activity with the variables according to the vascular status of patients with vasculogenic ED. The comparison included 92 vasculogenic ED patients grouped into 3 categories according to the vascular status of patients with ED-arterial insufficiency (n 5 26), veno-occlusive dysfunction (n 5 37), and mixed-type impotence (n 5 29)-and 50 controls. Receiver-operator characteristics (ROCs) were analyzed to find a cutoff value with the best sensitivity and lowest falsepositive rate. Serum prolidase activity (53.5 6 5.5 U/L vs 45.7 6 4.9 U/L, respectively; P , .001) and serum lipid hydroperoxide levels were significantly increased in patients with vasculogenic ED compared with controls, whereas serum total free sulfhydryl levels were significantly decreased in patients with vasculogenic ED compared with controls (P , .001). The lowest and highest mean serum prolidase activities were detected in control participants and in patients with arterial insufficiency, respectively (analysis of variance P , .001). The overall findings of this study support the predictive accuracy of the serum prolidase activity in our cohort, with a statistically significant ROC value of 0.78. Findings of this study have shown that serum prolidase activity is significantly associated with the presence and severity of vasculogenic ED, and elevated serum prolidase activity might be an independent predictor of ED.

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“…Previous clinical studies have shown increased serum prolidase activity, which indicates increased collagen turnover, in chronic liver disease, coronary artery disease (Myara et al 1984;Yildiz et al 2008). Conversely deficient/reduced serum prolidase activity was reported to be associated with lupus-like syndrome characterized by nonhealing skin ulcers owing to defective collagen turnover (Shrinath et al 1997). Reduced serum prolidase activity was also reported in renal insufficiency as the kidney is the main source of prolidase (Evrenkaya et al 2006; Liu et al 2007).…”

Section: Discussionmentioning

confidence: 98%