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Sjogren's syndrome;biopsy

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https://www.readbyqxmd.com/read/29321929/pleuritis-associated-with-primary-sjogren-syndrome
#1
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
https://www.readbyqxmd.com/read/29251022/impact-of-double-positive-for-anti-centromere-and-anti-ss-a-ro-antibodies-on-clinicopathological-characteristics-of-primary-sj%C3%A3-gren-s-syndrome-a-retrospective-cohort-study
#2
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 4 groups by ACA and anti-SS-A/Ro antibody positivity. Symptoms, laboratory and pathological data, and scleroderma-related data were compared among the groups...
December 18, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29245216/magnetic-resonance-imaging-of-the-spinal-cord-in-the-evaluation-of-3-patients-with-sensory-neuronopathies-diagnostic-assessment-indications-of-treatment-response-and-impact-of-autoimmunity-a-case-report
#3
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)
https://www.readbyqxmd.com/read/29243035/systemic-lupus-erythematosus-and-ocular-involvement-an-overview
#4
REVIEW
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
https://www.readbyqxmd.com/read/29241210/combination-cyclophosphamide-glucocorticoids-provide-better-tolerability-and-outcomes-versus-glucocorticoids-alone-in-patients-with-sjogren-s-associated-chronic-interstitial-nephritis
#5
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
https://www.readbyqxmd.com/read/29231165/polyvalent-immunoglobulins-with-vitamin-d3-and-vitamin-b12-in-the-treatment-of-sjogren-s-syndrome-in-a-vegetarian-with-stomatitis-glossodynia-xerostomia-and-elevated-antinuclear-antibodies-case-report%C3%A2
#6
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)...
December 12, 2017: International Journal of Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29209436/the-potential-relationship-between-flammer-and-sj%C3%A3-gren-syndromes-the-chime-of-dysfunction
#7
REVIEW
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
https://www.readbyqxmd.com/read/29208023/comparison-of-2002-aecg-and-2016-acr-eular-classification-criteria-and-added-value-of-salivary-gland-ultrasonography-in-a-patient-cohort-with-suspected-primary-sj%C3%A3-gren-s-syndrome
#8
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
https://www.readbyqxmd.com/read/29207757/vitamin-d-levels-and-associations-in-indian-patients-with-primary-sj%C3%A3-gren-s-syndrome
#9
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
https://www.readbyqxmd.com/read/29196420/fibrillary-glomerulonephritis-in-primary-sjogren-s-syndrome-a-rare-cause-of-renal-failure
#10
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
https://www.readbyqxmd.com/read/29196307/methotrexate-associated-lymphoproliferative-disease-with-multiple-pulmonary-nodules-in-a-patient-with-rheumatoid-arthritis
#11
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
https://www.readbyqxmd.com/read/29158045/-primary-sj%C3%A3-gren-syndrome-in-a-child
#12
I Majdoub, S Kallel, M Hsairi, M Snoussi, M Charfi, A Ben Halima, A Frikha, M Weli, F Safi, L Gargouri, N Ben Halima, A Mahfoudh, A Ghorbel, Z Bahloul
Sjögren syndrome is uncommon in children and occurs most often in association with autoimmune diseases (secondary Sjögren syndrome). We describe the clinical and biological features of a 7-year-old girl with primary Sjögren syndrome revealed by recurrent parotiditis. CASE REPORT: A 7-year-old girl was referred for investigation of multiple episodes of parotid swelling since age 4 years, without systemic symptoms. The examination was unremarkable except for enlarged and painless parotid glands...
November 17, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29154979/small-fiber-neuropathy-diagnosis-causes-and-treatment
#13
Damien Sène
Small fiber neuropathy, which affects the sensory A≏ and C fibers, is now a major diagnostic and therapeutic challenge. Nearly 7% of the general population have chronic neuropathic pain responsible for severe quality-of-life impairments. Awareness must therefore be raised among clinicians of the somatosensory and autonomic symptoms that can reveal small fiber neuropathy, appropriate diagnostic investigations, most common causes, and best treatment options for each patient profile. To help achieve this goal, the present review article discusses the clinical presentation of neuropathic pain and paresthesia and/or autonomic dysfunction due to involvement of nerves supplying exocrine glands and smooth muscle; normal findings from standard electrophysiological investigations; most informative diagnostic tests (epidermal nerve fiber density in a skin biopsy, laser-evoked potentials, heat- and cold-detection thresholds, electrochemical skin conductance); main causes, which consist chiefly of metabolic diseases (diabetes mellitus, glucose intolerance), dysimmunity syndromes (Sjögren's syndrome, sarcoidosis, monoclonal gammopathy), and genetic abnormalities (familial amyloidosis due to a transthyretin mutation, Fabry disease, sodium channel diseases); and the available symptomatic and etiological treatments...
November 15, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/29148407/minor-salivary-gland-fibrosis-in-sj%C3%A3-gren-s-syndrome-is-elevated-associated-with-focus-score-and-not-solely-a-consequence-of-aging
#14
Kerry M Leehan, Nathan P Pezant, Astrid Rasmussen, Kiely Grundahl, Jacen S Moore, Lida Radfar, David M Lewis, Donald U Stone, Christopher J Lessard, Nelson L Rhodus, Barbara M Segal, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVES: Evaluate the presence of minor salivary gland (SG) fibrosis in primary Sjögren's syndrome (pSS) as a function of disease pathology or a consequence of ageing. METHODS: Subjects with sicca symptoms attending a Sjögren's research clinic were classified by American European Consensus Group (AECG) criteria as either pSS or non-SS (nSS). Discovery (n=34 pSS, n=28 nSS) and replication (n=35 pSS, n=31 nSS) datasets were evaluated. Minor SG cross-sections from haematoxylin and eosin stained slides were imaged, digitally reconstructed and analysed for percent area fibrosis...
October 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29074164/defective-regulation-of-l1-endogenous-retroelements-in-primary-sjogren-s-syndrome-and-systemic-lupus-erythematosus-role-of-methylating-enzymes
#15
Clio P Mavragani, Adrianos Nezos, Irina Sagalovskiy, Surya Seshan, Kyriakos A Kirou, Mary K Crow
OBJECTIVE: To investigate whether altered DNA methylation contributes to the inappropriate expression of LINE-1 (L1) retroelements in primary Sjogren's syndrome (SS) and systemic lupus erythematosus (SLE). METHODS: Minor salivary glands (MSG) were obtained from 42 patients with primary SS [23 without adverse predictors for lymphoma development (SS-low risk), 7 SS-high risk and 12 complicated by B-cell lymphoma (SS-lymphoma)] and 17 sicca controls (SC). Additionally, kidney biopsy specimens and PBMCs were obtained from 23 and 73 lupus patients, respectively...
October 23, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29068087/dramatic-effect-of-hydroxychloroquine-on-lupus-alopecia
#16
Shohei Igari, Toshiyuki Yamamoto
Lupus alopecia is usually difficult to treat. We report a case of a 40-year-old woman with Sjögren's syndrome and atopic dermatitis who presented with discoid lupus erythematosus on the forearms and lupus erythematosus profundus with alopecia involving the scalp. A biopsy specimen taken from the discoid lupus erythematosus lesion on the forearm further exhibited a xanthomatous reaction, which however was not detected in another specimen from the lupus erythematosus profundus on the scalp. Treatment with oral hydroxychloroquine showed dramatic effects and complete hair regrowth was obtained 3 months later...
October 25, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/29067928/multi-organ-igg4-related-disease-demystifying-the-diagnostic-enigma
#17
S Bhardwaj, S Goyal, A K Yadav, A Goyal
IgG4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease entity, commonly creating confusion and diagnostic challenges. We present a case of a 25-year-old female who presented with bilateral orbital masses, lymphadenopathy, paraspinal and renal masses, which clinicoradiologically simulated lymphoma. The lymph node biopsy revealed interfollicular sheets of plasma cells creating confusion with Castleman's disease and marginal zone lymphoma. The orbital biopsy revealed ductular destruction, periductular plasma cells, and fibrosis, mimicking Sjogren's syndrome and Castleman's disease...
October 23, 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/29033144/impaired-anti-inflammatory-activity-of-ppar%C3%AE-in-the-salivary-epithelia-of-sj%C3%A3-gren-s-syndrome-patients-imposed-by-intrinsic-nf-%C3%AE%C2%BAb-activation
#18
Aigli G Vakrakou, Alexandros Polyzos, Efstathia K Kapsogeorgou, Dimitris Thanos, Menelaos N Manoussakis
Sjögren's syndrome (SS) patients manifest inflammation in the salivary glands (SG) and evidence of persistent intrinsic activation of ductal SG epithelial cells (SGEC), demonstrable in non-neoplastic SGEC lines derived from patients (SS-SGEC). The peroxisome-proliferator-activated receptor-γ (PPARγ) mediates important anti-inflammatory activities in epithelial cells. Herein, the comparative analysis of SG biopsies and SGEC lines obtained from SS patients and controls had revealed constitutively reduced PPARγ expression, transcriptional activity and anti-inflammatory function in the ductal epithelia of SS patients that were associated with cell-autonomously activated NF-κB and IL-1β pathways...
October 12, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29019884/utility-of-osteosclerotic-lesion-biopsy-in-diagnosis-of-poems-syndrome-a-case-report
#19
Daisuke Hara, Hisanao Akiyama, Saki Nukui, Takahiro Shimizu, Masahiro Hoshikawa, Yasuhiro Hasegawa
RATIONALE: We report a case of successful diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome based on monoclonality that was confirmed by an osteosclerotic lesion biopsy in a patient without pathognomonic symptoms or monoclonal gammopathy, probably because of comorbidities, which included systemic lupus erythematosus, rheumatoid arthritis, and Sjögren syndrome. PATIENT CONCERNS: A 57-year-old woman presented with an approximately 2-year history of numbness in the toes that had gradually spread, along with muscle weakness in both arms and legs...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28988489/fatty-infiltration-of-the-minor-salivary-glands-is-a-selective-feature-of-aging-but-not-sj%C3%A3-gren-s-syndrome
#20
Kerry M Leehan, Nathan P Pezant, Astrid Rasmussen, Kiely Grundahl, Jacen S Moore, Lida Radfar, David M Lewis, Donald U Stone, Christopher J Lessard, Nelson L Rhodus, Barbara M Segal, C Erick Kaufman, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVE: Determine the presence and assess the extent of fatty infiltration of the minor salivary glands (SG) of primary SS patients (pSS) as compared to those with non-SS sicca (nSS). METHODS: Minor SG biopsy samples from 134 subjects with pSS (n = 72) or nSS (n = 62) were imaged. Total area and fatty replacement area for each glandular cross-section (n = 4-6 cross-sections per subject) were measured using Image J (National Institutes of Health, Bethesda, MD)...
October 8, 2017: Autoimmunity
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