• Childhood Kidney Diseases
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• 제26권1호
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• pp.18-24
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• 2022

Pediatric rheumatologic diseases are rare systemic diseases that can involve various organs, including the kidneys. Each rheumatologic disease can exhibit characteristic renal involvement, which requires proper treatment and diagnosis. In this review, we discuss renal involvement in classic rheumatologic diseases, including juvenile idiopathic arthritis, Sjogren's syndrome, systemic sclerosis, and juvenile dermatomyositis. Reviews addressing lupus nephritis and antineutrophil cytoplasmic antibody-associated renal disease are complex and tend to cover a wide array of topics, and thus were excluded from this review.

• Tuberculosis and Respiratory Diseases
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• 제69권5호
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• pp.375-380
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• 2010

Lymphoid interstitial pneumonia (LIP) is a rare benign lymphoproliferative interstitial lung disease. LIP has been associated with autoimmune disorders, HIV, viral infections, and so on. Once underlying systemic diseases have been excluded, a diagnosis of idiopathic LIP can be made. Although 6 cases of pathologically confirmed LIP have occurred in Korea, thus far none has been associated with primary Sjogren's syndrome. A 44-year-old man was admitted to hospital due to a dry cough and dypsnea on exertion that had been ongoing for 2 months. A chest radiography showed multiple and variable-sized cystic lesions, on both lungs and both interstitial infiltration and consolidation in both lower lung fields. Tests for autoantibody showed positive results of anti-nuclear antibody and anti-Ro/La antibody. The patient underwent a video assisted thoracoscopic surgery biopsy and pathologically confirmed LIP. We report the first known case of LIP-associated with primary Sjogren's syndrome in Korea.

• Clinical and Experimental Pediatrics
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• 제59권6호
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• pp.276-279
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• 2016

Sjogren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder with worldwide incidence of 0.4 per 100,000 people. It is characterized by the triad of congenital ichthyosis, spastic diplegia or quadriplegia, and mental retardation. Herein we report a 2-year-old male child with SLS, asthma, and recurrent pneumonia. SLS was confirmed by a molecular genetics study that revealed a deletion mutation in the ALDH3A2 gene. An ALDH3A2 gene mutation results in dysfunction of the microsomal enzyme fatty aldehyde dehydrogenase and impaired metabolism and accumulation of leukotriene B4, which is a key molecule and a pro-inflammatory mediator in developing allergic diseases, especially asthma. An increased level of leukotriene B4 has been reported in SLS patients. As far as we are aware, this is the first report of SLS associated with asthma and recurrent pneumonia. In conclusion, pediatricians should be aware of and evaluate patients with SLS for possible associated asthma and allergic disorders.

• 대한치과보존학회:학술대회논문집
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• 대한치과보존학회 2006년도 Spring Scientific Meeting(the 125th) of Korean Academy of Conservative Dentistry
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• pp.245-245
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• 2006

• International Journal of Oral Biology
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• 제46권4호
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• pp.190-199
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• 2021

Despite evidence that bacteria-sensing Toll-like receptors (TLRs) are activated in salivary gland tissues of Sjogren syndrome (SS) patients, the role of oral bacteria in SS etiopathogenesis is unclear. We previously reported that two SS-associated oral bacteria, Prevotella melaninogenica (Pm) and Rothia mucilagenosa (Rm), oppositely regulate the expression of major histocompatibility complex class I (MHC I) in human salivary gland (HSG) cells. Here, we elucidated the mechanisms underlying the differential regulation of MHC I expression by these bacteria. The ability of Pm and Rm to activate TLR2, TLR4, and TLR9 was examined using TLR reporter cells. HSG cells were stimulated by the TLR ligands, Pm, and Rm. The levels of MHC I expression, bacterial invasion, and viability of HSG cells were examined by flow cytometry. The hypoxic status of HSG cells was examined using Hypoxia Green. HSG cells upregulated MHC I expression in response to TLR2, TLR4, and TLR9 activation. Both Pm and Rm activated TLR2 and TLR9 but not TLR4. Rm-induced downregulation of MHC I strongly correlated with bacterial invasion and cell death. Rm-induced cell death was not rescued by inhibitors of the diverse cell death pathways but was associated with hypoxia. In conclusion, Pm upregulated MHC I likely through TLR2 and TLR9 activation, while Rm-induced hypoxia-associated cell death and the downregulation of MHC I, despite its ability to activate TLR2 and TLR9. These findings may provide new insight into how oral dysbiosis can contribute to salivary gland tissue damage in SS.

• BMB Reports
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• 제39권3호
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• pp.229-239
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• 2006

Sj$\"{o}$gren's syndrome (SS) is an autoimmune disorder that affects the salivary glands, leading to xerostomia, and the lacrimal glands, resulting in xerophthalmia. Secondary SS is associated with other autoimmune disorders such as systemic rheumatic diseases and systemic lupus erythematosis (SLE), which can affect multiple organs, including the epidermis. Recent studies have demonstrated that green tea polyphenols (GTPs) possess both anti-inflammatory and anti-apoptotic properties in normal human cells. Epidemiological evidence has indicated that, in comparison to the United States, the incidence of SS, clinical xerostomia and lupus is considerably lower in China and Japan, the two leading green tea-consuming countries. Thus, GTPs might be responsible, in part, for geographical differences in the incidence of xerostomia by reducing the initiation or severity of SS and lupus. Consistent with this, molecular, cellular and animal studies indicate that GTPs could provide protective effects against autoimmune reactions in salivary glands and skin. Therefore, salivary tissues and epidermal keratinocytes could be primary targets for novel therapies using GTPs. This review article evaluates the currently available research data on GTPs, focusing on their potential application in the treatment of the oral manifestations of SS and skin manifestations of SLE.

• 치과방사선
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• 제29권1호
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• pp.175-189
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• 1999

The purpose of this study was to established the characteristic radiographic features in salivary gland diseases by means of sialography and scintigraphy. Sialograms and scintigrams with diseases of salivary gland were examined. In this group were 5 salivary stones, 14 sialadenitis, 17 Sjogren's syndromes and 8 benign tumors. The obtained results were as follows; 1. In the configuration of the shape of main duct, those revealed that modified curvilinear and curvilinear types were predominant in Sjogren's syndromes but reverse sigmoid and angular types were in sialolithiasis and sialadenitis combined with sialodochitis. 2. In the configuration of the course of main duct, those revealed that smooth types were predominant in sialadenitis and irregular types were predominant in Sjogren's syndromes and benign tumors and irregular types were seen in all salivary stones and sialadenitis combined with sialodochitis, 3. In the type of intraglandular pattern, those revealed that destructive changes of salivary duct system and parenchyma were severe in sialadenitis and salivary stones and predominantly severe in Sjogren's syndromes. 4. The function of salivary gland was decreased severely in Sjogren's syndrome. and also decrease in salivary stone and sialadenitis. In benign tumor, the uptake of radioisotope was not seen in lesion and the function of salivary gland decreased in its remaining normal parenchyma.

• Imaging Science in Dentistry
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• 제51권2호
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• pp.129-136
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• 2021

Purpose: This study investigated the effects of 1 year of training on imaging diagnosis, using static ultrasonography (US) salivary gland images of Sjögren syndrome patients. Materials and Methods: This study involved 3 inexperienced radiologists with different levels of experience, who received training 1 or 2 days a week under the supervision of experienced radiologists. The training program included collecting patient histories and performing physical and imaging examinations for various maxillofacial diseases. The 3 radiologists (observers A, B, and C) evaluated 400 static US images of salivary glands twice at a 1-year interval. To compare their performance, 2 experienced radiologists evaluated the same images. Diagnostic performance was compared between the 2 evaluations using the area under the receiver operating characteristic curve (AUC). Results: Observer A, who was participating in the training program for the second year, exhibited no significant difference in AUC between the first and second evaluations, with results consistently comparable to those of experienced radiologists. After 1 year of training, observer B showed significantly higher AUCs than before training. The diagnostic performance of observer B reached the level of experienced radiologists for parotid gland assessment, but differed for submandibular gland assessment. For observer C, who did not complete the training, there was no significant difference in the AUC between the first and second evaluations, both of which showed significant differences from those of the experienced radiologists. Conclusion: These preliminary results suggest that the training program effectively helped inexperienced radiologists reach the level of experienced radiologists for US examinations.

• Journal of Oral Medicine and Pain
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• 제32권4호
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• pp.397-402
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• 2007

쉐그렌 증후군은 만성 자가 면역 질환으로 특히 눈물샘과 침샘에 영향을 주어 구강건조증, 건성각결막염, 이하선 확대 등을 나타낸다. 일반적으로, 특발성으로 발생하여 쉐그렌 증후군이 단독으로 나타나는 원발성 쉐그렌 증후군과 류마티스 관절염, 전신성 홍반성 낭창 등과 같은 다른 자가 면역 질환과 함께 나타나는 이차성 쉐그렌 증후군으로 분류할 수 있다. 쉐그렌 증후군의 경우, 구강 건조감으로 인한 불편감을 호소하는 대신, 불쾌한 맛, 이하선 부위의 붓는 느낌 등 구강건조증 이외의 증상을 호소하기도 한다. 따라서 구강건조증을 호소하는 환자에 있어서만 쉐그렌 증후군을 감별진단에서 고려할 것이 아니라, 쉐그렌 증후군의 관련 소견 중 구강건조증 이외의 증상이나 징후에 대해서도 충분히 주목하여, 관련 증상이나 징후를 보이는 경우 쉐그렌 증후군 관련 검사를 고려하고 시행하는 것이 필요하며, 2002년 수정된 쉐그렌 증후군 진단 기준에 부합되는 검사들을 통해 조기에 진단을 하는 것이 필수적일 것으로 사료된다. Manthorpe등은 처음 쉐그렌 증상이 발생한 때로부터 진단이 될 때까지 평균 10년이라는 긴 시간이 소요된다고 보고였는데, 이는 쉐그렌 증후군 진단이 조기에 잘 이루어지지 않는다는 것을 반영하는 것임을 알 수 있다. Kassan등에 의하면, 쉐그렌 증후군의 여러 전신적 증상 중 림프종의 발생 정도는 건강한 사람들에 비해 쉐그렌 증후군을 갖고 있는 환자의 경우 44배정도 높은 것으로 보고되고 있다. 따라서 쉐그렌 증후군 환자의 정확하고 신속한 진단은 쉐그렌 증후군과 관련하여 발생될 수 있는 합병증등을 예방, 관리하는데 있어서 중요한 의미를 지닌다고 할 수 있다.

• Journal of Oral Medicine and Pain
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• 제40권3호
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• pp.130-134
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• 2015

$Sj{\ddot{o}}gren's$ syndrome (SS) is an autoimmune disease characterized by an autoimmune exocrinopathy involving mainly salivary and lacrimal glands. Apart from manifestations due to involvement of exocrine glands, patients with SS can present with muscular and neurological manifestations. Here, we report a rare case of a 59-year-old woman with primary SS, who presented with severe general toothache and masticatory muscle myalgia successfully treated with clonazepam. Although it was not certain that these symptoms could be originated from focal muscle dystonia or neurological changes that are associated with primary SS, our case suggested that comprehensive evaluation including neuromuscular examinations in the oral and maxillofacial area is needed in patients with SS.