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Undifferentiated Connective Tissue Disease

Arsenio Spinillo, Fausta Beneventi, Elena Locatelli, Vèronique Ramoni, Roberto Caporali, Claudia Alpini, Giulia Albonico, Chiara Cavagnoli, Carlomaurizio Montecucco
BACKGROUND: The burden of pregnancy complications associated with well defined, already established systemic rheumatic diseases preexisting pregnancy such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma is well known. Systemic rheumatic diseases are characterized by a long natural history with few symptoms, an undifferentiated picture or a remitting course making difficult a timely diagnosis. It has been suggested that screening measures for these diseases could be useful but the impact of unrecognized systemic rheumatic disorders on pregnancy outcome is unknown...
October 18, 2016: BMC Pregnancy and Childbirth
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
A Bortoluzzi, F Furini, F Campanaro, M Govoni
OBJECTIVES: The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999-2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria...
October 4, 2016: Lupus
Gabriele Valentini, Antonella Riccardi, Serena Vettori, Rosaria Irace, Michele Iudici, Salvatore Tolone, Ludovico Docimo, Marialuisa Bocchino, Alessandro Sanduzzi, Domenico Cozzolino
The aim of the study was to evaluate CXCL4 levels in undifferentiated connective tissue disease at risk for SSc (UCTD-SSc-risk) and confirm its increase and investigate its prognostic value. Serum CXCL4 levels were measured in 45 patients and 24 controls. CXCL4 was significantly higher in UCTD-SSc-risk patients than in controls. It resulted higher in patients with a shorter disease duration and in those lacking capillaroscopic alterations. We confirm that CXCL4 levels are increased in UCTD-risk-SSc patients...
September 20, 2016: Clinical and Experimental Medicine
Nafeesah Fatimah, Ahmad Ussaid, Aflak Rasheed
Undifferentiated connective tissue diseases usually present with arthralgias, sicca symptoms, Raynaud's phenomenon and leucopenia. This case presents the atypical presentation of an undifferentiated connective tissue disease with extensive cutaneous involvement of fingers and toes leading to gangrene with absence of typical rheumatological symptoms. The autoimmune profile showed positive ANA and anti-Ro/SS-A. Thromboembolism was ruled out on the basis of transthoracic and transesophageal echo. She was treated with I/V corticosteroids and cyclophosphamide that halted the disease progression...
August 2016: Oxford Medical Case Reports
M D Lobo, M Y Adeel, C B Wolff, P O Julu, M Shah, D M Collier, M Saxena, C Floyd, T Brier, V Kapil, O Mukhtar, A Radunovic, N Sutcliffe, F Sharif
OBJECTIVE: Profound BP variability (BPV) is a major cause of cardiovascular morbidity and poor quality of life as there are no optimal pharmacological strategies to help patients. We hypothesised that in a patient with baroreflex dysfunction and preserved efferent baroreflex pathway, carotid sinus stimulation may help control BP, BPV and heart rate variability (HRV). DESIGN AND METHOD: A 52 year old man was referred with profound HR and BPV. Home SBPs were in a range of 60-250 mmHg and DBPs were 40-130 mmHg and heart rate (HR) of 60-200 bpm (confirmed with ABPM, see Figure) despite multiple medications including felodipine 30 mg daily, terazosin 16 mg daily, doxazosin 8 mg daily, bisoprolol 20 mg daily and butrans patch 17...
September 2016: Journal of Hypertension
Simon Krabbe, Cigdem Gül, Bjarne Andersen, Niels Tvede
This case report describes a patient with arthritis of the large joints, bilateral sacroiliitis, and positive anti-SSA and anti-dsDNA antibody, who received sulfasalazine and shortly thereafter became critically ill. He developed toxic epidermal necrolysis, hemolytic anemia, lymphopenia, markedly elevated ferritin, and muscle wasting. A diagnosis of systemic lupus erythematosus was made, and mycophenolate mofetil and systemic glucocorticoids brought this severe disease under control. Toxic epidermal necrolysis-like lesions and hemophagocytic syndrome have been reported as manifestations of systemic lupus erythematosus...
2016: Case Reports in Rheumatology
Natalia C O Ciang, Nídia Pereira, David A Isenberg
In 1972, Sharp et al. described a new autoimmune rheumatic disease that they called MCTD, characterized by overlapping features of SSc, SLE, PM/DM, high levels of anti-U1snRNP and low steroid requirements with good prognosis. MCTD was proposed as a distinct disease. However, soon after the original description, questions about the existence of such a syndrome as well as disputes over the features initially described began to surface. The conundrum of whether MCTD is a distinct disease entity remains controversial...
July 19, 2016: Rheumatology
Athol U Wells, Vincent Cottin
PURPOSE OF REVIEW: There is growing speculation that idiopathic nonspecific interstitial pneumonia (NSIP) is, in reality, a grouping of separate disorders with a common histologic pattern. In this review, distinct clinical, imaging, and serologic features providing support for this premise are detailed and discussed. RECENT FINDINGS: The diagnosis of idiopathic NSIP is often uncertain because of its clinical and imaging diversity. In a landmark study of inter-multidisciplinary group diagnostic variation, there were striking discrepancies between seven expert groups (κ = 0...
September 2016: Current Opinion in Pulmonary Medicine
M Infantino, F Meacci, V Grossi, M Manfredi, F Li Gobbi, P Sarzi-Puttini, F Atzeni, M Benucci
Anti-nuclear antibody (ANA) positivity suggests CTD but can also lead to a diagnosis of UCTD when a patient does not fulfill the CTD diagnostic criteria. An anti-dense fine speckled (DFS) immunofluorescence (IIF) pattern can be observed when using an ANA test on HEp-2 cells and is due to the presence of antibodies to the nuclear DFS70 antigen that has rarely found in CTD. Serological testing for anti-DFS70 antibodies could therefore play a very interesting negative predictive role in stratifying patients on the basis of the evolution of UCTD to CTD...
July 18, 2016: Immunologic Research
Anna Masiak, Julia Kulczycka, Zenobia Czuszyńska, Zbigniew Zdrojewski
OBJECTIVES: Inflammatory myopathies are a group of idiopathic, heterogeneous systemic diseases affecting predominantly skeletal muscles, though they can also involve the skin and internal organs. The association between cancer and idiopathic inflammatory myopathies, particularly dermatomyositis, which is termed cancer-associated myositis (CAM), has been reported in the medical literature. A newly described autoantibody to a 155-kDa nuclear protein, identified as transcription intermediary factor 1-gamma (TIF1-γ), has proven useful for cancer screening in patients with dermatomyositis...
2016: Reumatologia
Peri Hickman Pepmueller
Autoimmune diseases often have overlapping symptoms and laboratory somewhat unfamiliar to the non-rheumatologist. Characteristic signs, symptoms, and autoantibodies define specific connective tissue diseases. Some patients have some characteristic symptoms, but cannot be definitively classified. Still other patients meet criteria for more than one specific connective tissue disease. These patients can be confusing with regard to diagnosis and prognosis. Clarification of each patient's condition can lead to improved patient care...
March 2016: Missouri Medicine
Chiara Cattaneo, Margherita Oberti, Cristina Skert, Angela Passi, Mirko Farina, Alessandro Re, Paola Tozzi, Erika Borlenghi, Giuseppe Rossi
Adult onset hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which can develop as a complication of many disorders. Early diagnosis is essential in order to avoid a fatal outcome. To confirm the diagnosis of acquired HLH made in a single institution series of adult patients with HLH-04 criteria, we applied the HScore and evaluated prognostic factors associated with clinical outcome. The median age of 35 patients was 54 (range 17-81), M/F ratio was 20/15. In 26/35 (74.3%) patients, an underlying haematological disease was present (2 Multicentric Castleman Disease, 10 B-cell Non-Hodgkin Lymphoma [NHL] and 14 T/NK-cell NHL); an autoimmune disorder was observed in four (11...
June 3, 2016: Hematological Oncology
Raúl Menor Almagro, Juan Francisco Rodríguez Gutiérrez, María Auxiliadora Martín-Martínez, María José Rodríguez Valls, Concepción Aranda Valera, José Luís de la Iglesia Salgado
OBJECTIVE: To determine the dilution titles at antinuclear antibodies (ANA) by indirect immunofluorescence observed in cell substrate HEp-2 and its association with the diagnosis of systemic connective tissue disease in ANA test requested by a Rheumatology Unit. METHOD: Samples of patients attended for the first time in the rheumatology unit, without prior ANA test, between January 2010 and December 2012 were selected. The dilution titers, immunofluorescence patterns and antigen specificity were recorded...
May 21, 2016: Reumatología Clinica
Michael Samycia, Collette McCourt, Kam Shojania, Sheila Au
BACKGROUND: The Dermatology and Rheumatology Treatment Clinic is a novel multidisciplinary clinic where patients are concomitantly assessed by a rheumatologist and dermatologist. OBJECTIVES: To determine the number of patients seen in clinic, patient demographics, and most common diagnoses. METHOD: A retrospective review was performed over a 2-year period. Data collected included patient age, sex, dermatologic diagnosis, rheumatologic diagnosis, biopsies performed, and number of follow-up visits...
September 2016: Journal of Cutaneous Medicine and Surgery
Justin M Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N Husain, Steven Montner, Jonathan H Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij, Mary E Strek
Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint...
June 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
P Elfving, O Marjoniemi, H Niinisalo, A Kononoff, L Arstila, E Savolainen, J Rutanen, O Kaipiainen-Seppänen
Objective of the study was to evaluate the annual incidence and distribution of autoimmune connective tissue diseases and vasculitides during 2010. All units practicing rheumatology in the Northern Savo area, Finland, participated in the study by collecting data on newly diagnosed adult patients with autoimmune connective tissue disease or vasculitis over 1-year period. Seventy-two cases with autoimmune connective tissue disease were identified. The annual incidence rates were as follows: systemic lupus erythematosus 3...
July 2016: Rheumatology International
Jonathan Bevan, Michael Richardson
We describe the case of a patient with a long history of undifferentiated connective tissue disease who developed headache, ataxia and orofacial dyskinesia attributed to a diagnosis of systemic lupus erythematosus (SLE). Gross elevation of the concentration of several plasma tumour markers (CEA, CA-125, CA19-9, CA15-3) was detected in the absence of malignancy. These markers fell significantly within a month of starting immunosuppressive therapy alongside clinical improvement. Caution should be taken in the interpretation of plasma tumour markers in patients with connective tissue disease...
2016: BMJ Case Reports
Yang Li, Ming' en Lyu, Feng Xue, Wenjie Liu, Yating Hao, Yue Guan, Boyang Sun, Cuicui Lyu, Xueping Gu, Rongfeng Fu, Yueting Huang, Wei Liu, Yunfei Chen, Xiaofan Liu, Lei Zhang, Renchi Yang
OBJECTIVE: To deepen the understanding of clinical manifestations and treatment of patients with positive lupus anticoagulant (LAC). METHODS: The clinical data of 2 patients were analyzed and related literature were reviewed. RESULTS: Case 1, a 31-year-old female, diagnosed as lupus anticoagulant positive, secondary to undifferentiated connective tissue disease, was presented with menorrhagia and thrombocytopenia. Anti-nuclear antibody (ANA) was positive 1:1000 (homogeneous type) with anti-double stranded DNA positive, and dRVVT LA1/LA2 was 3...
February 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
A Şahin, A Yetişgin, M Şahin, Y Durmaz, A K Cengiz
BACKGROUND: In humans, it has been suggested that low-level mean platelet volume (MPV) may be related to secondary thrombosis due to inflammation. For this reason, MPV can be used as a marker showing inflammation in the body. OBJECTIVES: To evaluate the association of MPV with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Disease Activity Score-28 (DAS-28) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in patients with rheumatic diseases...
May 6, 2015: West Indian Medical Journal
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