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Sjogren's syndrome;biopsy

Lara A Aqrawi, Janicke Liaaen Jensen, Gunnvor Øijordsbakken, Ann-Kristin Ruus, Ståle Nygård, Marit Holden, Roland Jonsson, Hilde Kanli Galtung, Kathrine Skarstein
A characteristic feature of primary Sjögren's syndrome (pSS) is the destruction of salivary and lacrimal glands mediated by mononuclear cell infiltration. Adipocytes can also occupy a large portion of the salivary gland (SG) tissue area, although little is known about their significance in pSS. We have previously investigated adipose tissue infiltration in SG biopsies from pSS patients and non-SS sicca controls. Our findings indicated the distinct incidence of adipose tissue replacement in pSS patients, where adipocytes were detected in interleukin (IL) 6 rich regions...
March 5, 2018: Autoimmunity
Jolien F van Nimwegen, Martha S van Ginkel, Suzanne Arends, Erlin A Haacke, Bert van der Vegt, Nicole Sillevis Smitt-Kamminga, Fred K L Spijkervet, Frans G M Kroese, Alja J Stel, Elisabeth Brouwer, Arjan Vissink, Hendrika Bootsma
Objectives: To validate the ACR-EULAR classification criteria for primary SS (pSS), and compare them to the American-European Consensus Group (AECG) and ACR criteria in a Dutch prospective diagnostic cohort. Methods: Consecutive patients (n = 129) referred for suspicion of pSS underwent a multidisciplinary evaluation, including a labial and/or parotid gland biopsy. Patients with an incomplete work-up (n = 8) or associated systemic auto-immune disease (n = 7) were excluded...
February 12, 2018: Rheumatology
J-W Kim, H Lee, S-H Park, S-K Kim, J-Y Choe, J K Kim
OBJECTIVE: Salivary gland ultrasonography (SGUS) has been applied in the diagnosis of Sjögren's syndrome (SS). The aim of this study is to investigate the association of SGUS findings with clinical, histological, and serologic features of SS. METHODS: A total of 104 patients with suspected SS underwent SGUS for evaluation of salivary gland involvement. Patients with primary SS were determined according to the classification criteria for SS. The parenchymal inhomogeneity of bilateral parotid and submandibular glands was graded from 0 (homogeneity) to 4 (gross inhomogeneity)...
February 7, 2018: Scandinavian Journal of Rheumatology
Jeffery E Illman, Simone B Terra, Allison J Clapp, Katie N Hunt, Robert T Fazzio, Sejal S Shah, Katrina N Glazebrook
OBJECTIVES: This article reviews our experience and describes the literature findings of granulomatous diseases of the breast and axilla. METHODS: After approval of the Institutional Review Board was obtained, the surgical pathological records from January 2000 to January 2017 were searched for the keyword granulomatous. Clinical, imaging and histology findings were reviewed by both a fellowship-trained radiologist and a breast-imaging consultant radiologist, reviewing 127 patients (age range, 32-86 years; 126 women and 1 man)...
February 5, 2018: Insights Into Imaging
Shino Fujimoto, Hiroshi Kawabata, Nozomu Kurose, Haruka Kawanami-Iwao, Tomoyuki Sakai, Takafumi Kawanami, Yoshimasa Fujita, Toshihiro Fukushima, Yasufumi Masaki
RATIONALE: TAFRO syndrome is a newly proposed disorder that manifests as thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. In this report, we describe the development of severe TAFRO syndrome-like systemic symptoms during the clinical course of juvenile-onset Sjögren's syndrome in a 32-year-old woman. PATIENT CONCERNS: The patient was admitted due to dyspnea, fever, polyarthralgia, and generalized edema. She had been diagnosed with Sjögren's syndrome at the age of 14 years, based on histopathological examination of a biopsy of the minor salivary glands and the development of Raynaud's phenomenon, with no follow-up treatment required...
December 2017: Medicine (Baltimore)
Zhihua Zhou, Weiqiang Jiang, Ming Wang, Yongyuan Liu, Wei Zhang, Manping Huang, Donghui Liang
RATIONALE: Purpura is a common dermatologic manifestation in Sjögren syndrome (SS). When a patient presents with sicca symptoms, the diagnosis of SS is not difficult. PATIENT CONCERNS: Here, we reported a case of a 52-year-old Chinese woman who initially presented with nonpalpable purpura on both lower extremities, and these lesions had developed soon after prolonged sitting. In the past 2 years, she had repeated cutaneous nonpalpable purpura 4 times. She had no sicca symptoms, dry eyes, or dry mouth...
December 2017: Medicine (Baltimore)
Yuhua Wang, Guohua Zhang, Lingling Zhang, Miansong Zhao, Hongdong Huang
Sjögren syndrome (SS) is characterized by dysfunction of the exocrine glands, particularly the salivary and lacrimal glands. Thus, labial salivary gland biopsy is useful method for diagnosing SS. The aim of the present study was to investigate the microRNA (miRNA or miR) profile of labial salivary glands obtained from SS patients and to examine the correlation of miR-181a and -16 levels with the pathological grade in SS. miRNA expression in labial salivary gland tissues was profiled in 3 female patients with primary SS and 3 female patients with non-SS sicca syndrome using microarray analysis...
January 2018: Experimental and Therapeutic Medicine
Valter R Fonseca, Vasco C Romão, Ana Agua-Doce, Mara Santos, Dolores López-Presa, Ana Cristina Ferreira, João Eurico Fonseca, Luis Graca
OBJECTIVES: To investigate whether the balance of blood T follicular helper (Tfh) and Foxp3+ T follicular regulatory (Tfr) cells can inform on ectopic lymphoid neogenesis and disease activity in primary Sjögren's syndrome (SS). METHODS: We prospectively recruited 56 patients with clinical suspicion of SS, 16 of which subsequently fulfilled American European Consensus Group primary SS classification criteria and were compared with 16 patients with non-Sjögren sicca syndrome...
January 23, 2018: Arthritis & Rheumatology
Stefan Markus Weiner
Renal involvement is common and heterogenous in connective tissue diseases and has a main influence on prognosis and mortality. In systemic lupus erythematosus proliferative glomerulonephritis is the most common manifestation, while in primary Sjogren's syndrome interstitial nephritis with tubular dysfunction is the predominant pathological feature. In systemic sclerosis the most serious renal manifestation is scleroderma renal crisis characterized by abrupt onset of hypertension and acute kidney injury associated with an increase in plasma renin activity...
January 2018: Deutsche Medizinische Wochenschrift
Chiaki Hosoda, Yusuke Hosaka, Kai Ryu, Akira Kinoshita, Keisuke Saito, Kazuyoshi Kuwano
We herein present a case of a 71-year-old woman with primary Sjogren's syndrome (SjS), who developed bilateral pleural effusion and ground glass opacity during treatment with low-dose prednisolone. The pleural effusion and bronchoalveolar lavage fluid revealed elevation of lymphocytes. Thoracoscopic pleural biopsy showed infiltration of lymphocytes with no evidence of other diseases, confirming SjS-related pleuritis. Therefore, we initiated 20 mg prednisolone and pleural effusion was rapidly resolved. Our results indicate that SjS can be rarely complicated with pleuritis...
February 2018: Respirology Case Reports
Yasunori Suzuki, Hiroshi Fujii, Hideki Nomura, Ichiro Mizushima, Kazunori Yamada, Masakazu Yamagishi, Mitsuhiro Kawano
OBJECTIVES: The purpose of our study was to define the clinical characteristics of anti-centromere antibody and anti-SS-A/Ro antibody (ACA/SS-A) double positive Sjögren's syndrome (SS) and to clarify the clinical impact of these antibodies. METHODS: We examined 108 patients (6 males, mean age 57.9 years) with SS who underwent labial salivary gland biopsy. The patients were divided into four groups by ACA and anti-SS-A/Ro antibody positivity. Symptoms, laboratory and pathological data, and scleroderma-related data were compared among the groups...
January 9, 2018: Modern Rheumatology
Julius Birnbaum, Aliya Lalji, Ezequiel A Piccione, Izlem Izbudak
RATIONALE: Sensory neuronopathy can be a devastating peripheral nervous system disorder. Profound loss in joint position is associated with sensory ataxia, and reflects degeneration of large-sized dorsal root ganglia. Prompt recognition of sensory neuronopathies may constitute a therapeutic window to intervene before there are irreversible deficits. However, nerve-conduction studies may be unrevealing early in the disease course. In such cases, the appearance of dorsal column lesions on spinal-cord MRI can help in the diagnosis...
December 2017: Medicine (Baltimore)
Rosanna Dammacco
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control...
December 14, 2017: Clinical and Experimental Medicine
Yuqi Shen, Jingyuan Xie, Li Lin, Xiao Li, Pingyan Shen, Xiaoxia Pan, Hong Ren, Nan Chen
BACKGROUND: Steroid therapy has become an effective option for patients with primary Sjogren's syndrome with tubulointerstitial nephritis (TIN), while the use of cytotoxic agents is still debated. Our study aimed to compare the clinical outcomes of patients treated with cyclophosphamide (CTX) combined with glucocorticoids with those of patients treated with glucocorticoids alone. METHODS: All patients with primary Sjogren's syndrome with chronic TIN admitted to the Division of Nephrology, Ruijin Hospital, from January 1, 2002, to April 30, 2016, and treated with steroids alone or combined with CTX were included...
2017: American Journal of Nephrology
Clemens Cuny, Barbara Vaerst, Jennis Gabrielpillai, Aykut Tahtali, Sven Balster, Reinhard Lissner, Barry G Woodcock
BACKGROUND: Sjogren's syndrome, involving sicca symptoms with xerostomia, stomatitis, and considerable pain is a difficult-to-treat autoimmune disease where the treatment options are limited and, as in the case of methotrexate, have a low therapeutic index. CASE REPORT: This case report concerns a male patient, aged 75 years and vegetarian, with Sjogren's syndrome subsequently confirmed by salivary gland biopsy. Serum antinuclear antibodies (ANA) were elevated (1 : 320)...
January 2018: International Journal of Clinical Pharmacology and Therapeutics
Babak Baban, Olga Golubnitschaja
Flammer syndrome (FS) is a term to blanket a cluster of vascular and nonvascular signs and symptoms linked to primary vascular dysregulation (PVD), increased sensitivity to various stimuli (stress, drugs, etc.) and altered sense regulation such as pain, smell and thirst perception. On one hand, disruption of blood barrier and homeostasis of the body are the main targets of vascular irregularity. Inflammation and immune disorders including autoimmunity are considered as a consequence of the abnormal vascular regulation processes...
December 2017: EPMA Journal
Maëlle Le Goff, Divi Cornec, Sandrine Jousse-Joulin, Dewi Guellec, Sebastian Costa, Thierry Marhadour, Rozenn Le Berre, Steeve Genestet, Béatrice Cochener, Sylvie Boisrame-Gastrin, Yves Renaudineau, Jacques-Olivier Pers, Alain Saraux, Valérie Devauchelle-Pensec
BACKGROUND: The objective was to evaluate concordance between 2002 American-European Consensus Group (AECG) and 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (pSS) and to assess how salivary gland ultrasonography (SGUS) might improve the classification of patients. METHODS: Patients with suspected pSS underwent a standardised evaluation, including SGUS, at inclusion into the single-centre Brittany DIApSS cohort...
December 6, 2017: Arthritis Research & Therapy
Pulukool Sandhya, Gowri Mahasampath, Puneet Mashru, Joseph Dian Bondu, Victoria Job, Debashish Danda
Introduction: Vitamin D is a steroid hormone belonging to the class of secosteroids with myriad immune functions and has been implicated in aetiopathogenesis of various autoimmune diseases. Although, there have been various studies showing the association of vitamin D in rheumatoid arthritis and lupus in different populations, there have been limited studies on vitamin D and primary Sjögren's Syndrome (pSS). There are no studies on association of vitamin D and pSS from any tropical country including Indian subcontinent...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
Rahul Sehgal, Syed M Sajjad, Jiwan K Thapa
Renal involvement in primary Sjogren's syndrome (pSS) varies in severity and prevalence. Although previously felt to be uncommon, kidneys can be involved in up to 25%-30% of pSS patients. Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that can occur in association with another autoimmune condition or malignancy. The diagnosis relies on renal biopsy findings of haphazardly arranged fibrils in all glomerular compartments and distinction from other forms of fibrillary glomerulopathies such as renal amyloidosis and immunotactoid glomerulopathy...
December 1, 2017: Clinical Medicine & Research
Wei-Fu Lai, Yen-Po Chin, Chih-Wei Liu, Chang-Youh Tsai
A 62-year-old woman with rheumatoid arthritis and secondary Sjögren's syndrome took methotrexate (MTX) 5 mg three times a week regularly but gradually developed an intermittent fever, oral ulcers and productive cough with mucopurulent sputum for about 2 weeks. Image study found multiple nodular lesions and lymphadenopathies in bilateral lungs. Empirical antibiotics for 1 week failed to alleviate the fever. A transbronchial biopsy in the right fourth bronchus showed infiltration of abnormally enlarged lymphoid cells with a surface marker of CD20, some of which also stained positively in situ with Epstein-Barr virus-encoded small RNA and some CD3(+) cells...
December 1, 2017: BMJ Case Reports
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