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Sjogrens, lungs, autoimmune

Jitesh Ahuja, Deepika Arora, Jeffrey P Kanne, Travis S Henry, J David Godwin
Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia...
November 2016: Radiologic Clinics of North America
Masato Kono, Yutaro Nakamura, Katsuhiro Yoshimura, Yasunori Enomoto, Yoshiyuki Oyama, Hironao Hozumi, Noriyuki Enomoto, Tomoyuki Fujisawa, Naoki Inui, Etsuko Hamada, Thomas V Colby, Masato Maekawa, Takafumi Suda
BACKGROUND: The aim of this study was to evaluate the incidence and clinical features of patients who developed collagen vascular disease (CVD) after an initial diagnosis of idiopathic nonspecific interstitial pneumonia (NSIP). METHODS: We conducted a retrospective review of 72 consecutive patients with NSIP who were diagnosed by surgical lung biopsy in our institution (idiopathic NSIP, n = 35; CVD-NSIP, n = 37 at initial diagnosis). No patients fulfilled the American College of Rheumatology criteria for a diagnosis with CVD within six months after the diagnosis of idiopathic NSIP...
August 2016: Respiratory Medicine
Maureen Rischmueller, Joanna Tieu, Susan Lester
Primary Sjögren's syndrome (pSS) is a relatively common autoimmune systemic rheumatic disease. In addition to sicca syndrome and swollen salivary glands, systemic features manifest in the majority of patients, and are severe in 15%, particularly affecting the joints, skin, lungs, and peripheral nervous system. A recent meta-analysis estimated a pooled relative risk of 13.76 for the development of non-Hodgkin lymphoma, particularly in pSS patients who have parotid enlargement, vasculitis, cryoglobulinemia, and antibodies to Ro and La...
February 2016: Best Practice & Research. Clinical Rheumatology
Małgorzata Bartosiewicz
The connective tissue diseases are a variable group of autoimmune mediated disorders characterized by multiorgan damage. Pulmonary complications are common, usually occur after the onset of joint symptoms, but can also be initially presenting complaint. The respiratory system may be involved in all its component: airways, vessels, parenchyma, pleura and respiratory muscles. Lung involvement is an increasing cause of morbidity and mortality in the connective tissue diseases. Clinical course is highly variable - can range from mild to rapidly progressive, some processes are reversible, while others are irreversible...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Aleksandra Plavsic, Rada Miskovic, Jasna Bolpacic, Branka Šuštran, Aleksandra Peric-Popadic, Mirjana Bogic
Sjögren's syndrome is an autoimmune disease of unknown etiology where immune response to self-antigens is believed to result from interactions between genetic and environmental factors. We describe the case of a patient who has been diagnosed with Sjögren's syndrome based on typical clinical and immunological parameters. The clinical picture was dominated by the respiratory symptoms, and radiographic and multislice computed tomography examination of the chest showed certain changes characteristic of pneumoconiosis...
June 15, 2015: Open Access Macedonian Journal of Medical Sciences
Aikaterini Liapi, Alice Horisberger, Spertini François, Camillo Ribi
Sjögren's syndrome (SS) is an autoimmune disease leading to mucosal dryness. It may also involve joints, nerves, kidneys and lungs. Patients with SS are also at increased risk for lymphoma. Diagnosis of SS relies on clinical, biological, histological and radiological criteria, after exclusion of other causes. Initial work-up may be performed in general practice, by serology (antinuclear and anti-SSA/SSB antibodies, rheumatoid factor) and by measuring lacrimal and salivary flow. Antibodies may be within normal range in up to one third of patients and when present are not specific for SS...
April 6, 2016: Revue Médicale Suisse
Victor C Kok, Jorng-Tzong Horng, Guo-Dung Hung, Jia-Li Xu, Tzu-Wei Hung, Yu-Ching Chen, Chien-Lung Chen
BACKGROUND: Recent studies indicate that chronic insomnia is associated with the development of certain somatic diseases. Whether it would be associated with the development of an autoimmune disease (AID) was unknown. OBJECTIVE: We aimed to examine the association and quantify the magnitude of risk for AID in individuals suffering from chronic insomnia requiring sleep-inducing pills. DESIGN: This was a population-based, nationwide longitudinal study...
September 2016: Journal of General Internal Medicine
Claudio De Felice, Silvia Leoncini, Cinzia Signorini, Alessio Cortelazzo, Paolo Rovero, Thierry Durand, Lucia Ciccoli, Anna Maria Papini, Joussef Hayek
Rett syndrome (RTT) is a devastating neurodevelopmental disease, previously included into the autistic spectrum disorders, affecting almost exclusively females (frequency 1:10,000). RTT leads to intellective deficit, purposeful hands use loss and late major motor impairment besides featuring breathing disorders, epilepsy and increased risk of sudden death. The condition is caused in up to 95% of the cases by mutations in the X-linked methyl-CpG binding protein 2 (MECP2) gene. Our group has shown a number of previously unrecognized features, such as systemic redox imbalance, chronic inflammatory status, respiratory bronchiolitis-associated interstitial lung disease-like lung disease, and erythrocyte morphology changes...
April 2016: Autoimmunity Reviews
Chun-Ting Chen, Yu-Chen Tseng, Chih-Wei Yang, Hsuan-Hwai Lin, Peng-Jen Chen, Tien-Yu Huang, Yu-Lueng Shih, Wei-Kuo Chang, Tsai-Yuan Hsieh, Heng-Cheng Chu
The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis...
January 2016: Medicine (Baltimore)
Che-Wen Yang, Yung-Tsu Cho, Kai-Lung Chen, Yi-Chun Chen, Hsiang-Lin Song, Chia-Yu Chu
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse drug reactions characterized by different extents of epidermal necrosis and mucosal breakdown. A limited number of studies have reported the long-term patterns of SJS and TEN complications in patient populations over long follow-up periods. The aim of this retrospective study was to collect data on long-term sequelae in patients admitted for SJS, SJS/TEN overlap, or TEN between 1998 and 2012. Among all 102 patients eligible for analysis, the 2 most common sequelae were cutaneous and ocular problems, both with incidences of 44...
May 2016: Acta Dermato-venereologica
Delia Reina, Daniel Roig Vilaseca, Vicenç Torrente-Segarra, Dacia Cerdà, Ivan Castellví, Cèsar Díaz Torné, Mireia Moreno, Javier Narváez, Vera Ortiz, Rosana Blavia, Montserrat Martín-Baranera, Hèctor Corominas
UNLABELLED: Primary Sjögren syndrome (PSS) is a chronic inflammatory autoimmune disease. Interstitial lung disease (ILD) can be an extraglandular complication. OBJECTIVE: To evaluate the clinical characteristics of patients diagnosed with PSS with ILD. METHODS: Multicentre cohort study with 25 patients diagnosed with PSS and ILD. Data of PSS, prognostic factors, pulmonary involvement variables, complementary tests that suggest a worse diagnosis and treatment given were collected...
July 2016: Reumatología Clinica
Ethan M Balk, Amy Earley, Esther A Avendano, Gowri Raman
BACKGROUND: Silicone gel breast implants were removed from the U.S. market for cosmetic use in 1992 owing to safety concerns. They were reintroduced in 2006, with a call for improved surveillance of clinical outcomes. PURPOSE: To systematically review the literature regarding specific long-term health outcomes in women with silicone gel breast implants, including cancer; connective tissue, rheumatologic, and autoimmune diseases; neurologic diseases; reproductive issues, including lactation; offspring issues; and mental health issues (depression and suicide)...
February 2, 2016: Annals of Internal Medicine
A V Arrossi, M Merzianu, C Farver, C Yuan, S H Wang, M O Nakashima, C V Cotta
BACKGROUND: Light chain deposition disease (LCDD) is usually a systemic disorder characterised by non-amyloid monoclonal immunoglobulin light chain deposition in tissues. Localised nodular pulmonary (NP) LCDD is a rare and poorly characterised entity and, owing to the difficulties in diagnosis, limited data are available. METHODS: We investigated the clinical, radiological and pathological characteristics of a series of six confidently diagnosed cases of NPLCDD...
June 2016: Journal of Clinical Pathology
Jon Nicholas Rubenstein, Colleen Beatty, Zoe Kinkade, Cara Bryan, Jeffery Paul Hogg, Laura F Gibson, Jeffrey A Vos
Extranodal Marginal Zone Lymphoma (ENMZL) of Mucosa-Associated Lymphoid Tissue (MALT) is a problematic and sometimes controversial diagnosis. While commonly seen in the stomach in the setting of chronic Helicobacter pylori infection, other extranodal sites, such as the lung, may also present with disease. ENMZL is clinically and morphologically heterogeneous; however, regardless of presentation, the etiology lies in the accumulation of lymphoid tissue in non-traditional sites. This phenomenon is typically secondary to an underlying inflammatory stimulus such as chronic infection or autoimmune states...
February 2015: Journal of Clinical & Experimental Pathology
Hossam Abdalla, Arindam Bagchi, Sabiha Bandagi
BACKGROUND: Lynch syndrome (LS) is an inherited colorectal cancer (CRC) syndrome accounting for about 3-5% of all cases and involves significantly higher risk of subsequent malignancies, colonic as well as extra-colonic. Increased risk of malignancies, especially lymphoid malignancies, have been described in patients with autoimmune diseases like rheumatoid arthritis (RA), systemic lupus erythematosus, and Sjögren's syndrome. Epidemiological studies demonstrated that hematopoietic, lung, skin, and prostate cancers are increased in RA, while breast and colon cancers are decreased, with an overall slight increase in all cancers...
2015: American Journal of Case Reports
Claire M Ellender, Catriona McLean, Trevor J Williams, Gregory I Snell, Helen M Whitford
A 33-year-old woman with past history of Sjögren's syndrome and systemic lupus erythematosus presented with dyspnea and syncope secondary to pulmonary hypertension. After progressive symptoms over 4 years, she received bilateral lung transplantation. Histopathology of the explanted lungs showed isolated pulmonary amyloid light-chain amyloidosis and pulmonary cysts. No evidence of systemic amyloidosis was found at the time of transplantation. Seven years post lung transplantation, she remains well with no evidence of systemic amyloidosis recurrence...
June 2015: Respirology Case Reports
Yoshinao Muro, Yuji Hosono, Kazumitsu Sugiura, Yasushi Ogawa, Tsuneyo Mimori, Masashi Akiyama
INTRODUCTION: Anti-PM/Scl antibodies are associated with polymyositis (PM)/systemic scleroderma (SSc) overlap syndromes and are also found in other systemic autoimmune diseases. Although anti-PM/Scl reactivity is found in 3-11% of PM or SSc patients and in approximately 25% of PM/SSc overlap patients, previous large studies of Japanese patients with scleroderma reported that anti-PM/Scl are not found in Japanese patients at all. The PM/Scl autoantigen complex comprises 11-16 different polypeptides; ELISA with PM1-α peptide, which is a major epitope of the PM/Scl complex, has frequently been used for the detection of these antibodies in recent studies...
2015: Arthritis Research & Therapy
M Fujimoto, K Ikeda, T Nakamura, T Iwamoto, S Furuta, H Nakajima
Increased risk of developing systemic lupus erythematosus (SLE) has been reported in patients with Klinefelter syndrome. Here, we describe a 16-year-old Japanese patient with trisomy X (47,XXX) who developed mixed connective tissue disease (MCTD) and Sjögren's syndrome. She had polyarthritis, edematous fingers with Raynaud's phenomenon, sicca syndrome, interstitial lung disease, possible myositis, and was positive for anti-nuclear antibody, anti-nRNP antibody and rheumatoid factor. This is the first report in the literature of a case of MCTD with female polysomy X, which further supports the link between the presence of extra X chromosome(s) and the development of autoimmune diseases...
October 2015: Lupus
Giuseppe Lopalco, Luca Cantarini, Antonio Vitale, Florenzo Iannone, Maria Grazia Anelli, Laura Andreozzi, Giovanni Lapadula, Mauro Galeazzi, Donato Rigante
A complex web of dynamic relationships between innate and adaptive immunity is now evident for many autoinflammatory and autoimmune disorders, the first deriving from abnormal activation of innate immune system without any conventional danger triggers and the latter from self-/non-self-discrimination loss of tolerance, and systemic inflammation. Due to clinical and pathophysiologic similarities giving a crucial role to the multifunctional cytokine interleukin-1, the concept of autoinflammation has been expanded to include nonhereditary collagen-like diseases, idiopathic inflammatory diseases, and metabolic diseases...
2015: Mediators of Inflammation
Yoshinao Muro, Yuji Hosono, Kazumitsu Sugiura, Yasushi Ogawa, Tsuneyo Mimori, Masashi Akiyama
INTRODUCTION: Anti-PM/Scl antibodies are associated with polymyositis (PM) / systemic scleroderma (SSc) overlap syndromes and are also found in other systemic autoimmune diseases. Although anti-PM/Scl reactivity is found in 3-11% of PM or SSc patients and in approximately 25% of PM/SSc overlap patients, previous large studies of Japanese patients with scleroderma reported that anti-PM/Scl are not found in Japanese patients at all. The PM/Scl autoantigen complex comprises 11-16 different polypeptides; ELISA with PM1-α peptide, which is a major epitope of the PM/Scl complex, has frequently been used for the detection of these antibodies in recent studies...
December 2015: Arthritis Research & Therapy
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