Clinical Characteristics and Risk Factors for Overlapping Rheumatoid Arthritis
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Autoimmune Pancreatitis: an Autoimmune Or Immunoinflammatory Disease?
10 The Open Autoimmunity Journal, 2011, 3, 10-16 Open Access Autoimmune Pancreatitis: An Autoimmune or Immunoinflammatory Disease? Yvonne Hsieh, Sudhanshu Agrawal, Leman Yel and Sudhir Gupta* Division of Basic and Clinical Immunology, University of California, Irvine, CA 92697, USA Abstract: Autoimmune pancreatitis (AIP) has been widely presumed to be an autoimmune disease that is characterized by elevated IgG and/or IgG4, the presence of autoantibodies, and an infiltration of lymphocytes and plasma cells with fi- brosis. However, no detailed immunological studies have been published. To define immunological changes in AIP in de- tail, and to review evidence for autoimmunity which may be antigen specific and may play a role in the pathogenesis of AIP, and therefore, to determine whether AIP is an autoimmune disease. A detailed immunological investigation for both innate and adaptive immune responses was performed in a patient with AIP. Review of literature was performed from Pub med, and Medline search. Immunological analysis of patient with AIP revealed increased production of proinflammatory IL-6, and IL-17, and increased NK cell activity. No organ-specific or non-specific antibodies were detected. There was no correlation between serum IgG4 with disease activity or response to steroid therapy. Review of literature revealed lack of auto-antigen-specific T and B cell responses in AIP, and autoantibodies are present only in a subset of patients, and are not specific to pancreatic tissue antigens. Therefore, we propose the term Immunoinflammatory pancreatitis rather than an autoimmune pancreatitis. Keywords: NK cells, IL-6, IL-17, autoantibodies, IgG4. INTRODUCTION collectively has been suggested to increase the sensitivity of detection of the disease [6]. -
73 Rheumatoid Factor in Patients with Systemic Lupus Erythematosus
Abstracts Lupus Sci Med: first published as 10.1136/lupus-2019-lsm.73 on 1 April 2019. Downloaded from Conclusions LIT responds appropriately in both directions of the following: LI, anti 2GP1 IgG/IgM, Anti aCL IgG/ to changes in physician (T2T) as well as patient relevant IgM. (DA and health status) outcomes among Spanish SLE Statistical Analysis: Epiinfo version 7.2.0.1. patients. Results We reviewed 147 clinical histories, 107 with RF Funding Source(s): None ordered. Female 93/107 (86.9%), mean age 41.6 years (SD ±13.8). Disease duration 98 months (SD ±80.6). Tobacco exposure 10/107 (9.35%). RF (+) 22/107 (20.5%), RF median titer 228.8 IU/ml (31–2825 IU/ml). – 73 RHEUMATOID FACTOR IN PATIENTS WITH SYSTEMIC RF level in patients with GMN was 142 IU/ml (39 191), – LUPUS ERYTHEMATOSUS without GMN 258 IU/ml (31 2825) p=0.46. RF level in patients with anti Ro (+) was 248 IU/ml (31– Diana G Garate*, Demelza D Yucra, Ruth Balcazar, Hamaui Adriana, Diana Dubinsky. 2825), Ro (-) 52.5 IU/ml (31–74) p=0.37. Sanatorio Guemes RF (-) subgroup showed statistically significant association 10.1136/lupus-2019-lsm.73 with female sex, discoid lesions and aPL presence. RF(+) was associated with Ab Ro, La and patients with RA. (Table 1). Background Rheumatoid factor (RF) in SLE is found in Not found significant association with: rash, photosensivity, around 25% of patients. Authors associate it with cutaneous oral ulcer, joint pain, arthritis, serositis, neurologic and hema- involvement, Sicca, anti Ro (+) and a protective role in glo- tological involvement. -
A Rare Case Report of Polyangiitis Overlap Syndrome: Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis Michele V
Quan et al. BMC Pulmonary Medicine (2018) 18:181 https://doi.org/10.1186/s12890-018-0733-2 CASE REPORT Open Access A rare case report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Michele V. Quan1* , Stephen K. Frankel2, Mehrnaz Maleki-Fischbach3 and Laren D. Tan1* Abstract Background: Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature. Case presentation: We conducted a PUBMED literature review using key words ‘granulomatosis with polyangiitis,’ ‘Wegener’s,’‘GPA,’‘eosinophilic granulomatosis with polyangiitis,’‘Churg-Strauss,’‘EGPA,’‘overlap syndrome,’‘Wegener’s with eosinophilia,’ and ‘GPA with eosinophilia’ in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described. Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. -
Psoriasis, a Systemic Disease Beyond the Skin, As Evidenced by Psoriatic Arthritis and Many Comorbities
1 Psoriasis, a Systemic Disease Beyond the Skin, as Evidenced by Psoriatic Arthritis and Many Comorbities – Clinical Remission with a Leishmania Amastigotes Vaccine, a Serendipity Finding J.A. O’Daly Astralis Ltd, Irvington, NJ USA 1. Introduction Psoriasis is a systemic chronic, relapsing inflammatory skin disorder, with worldwide distribution, affects 1–3% of the world population, prevalence varies according to race, geographic location, and environmental factors (Chandran & Raychaudhuri, 2010; Christophers & Mrowietz, 2003; Farber & Nall, 1974). In Germany, 33,981 from 1,344,071 continuously insured persons in 2005 were diagnosed with psoriasis; thus the one year prevalence was 2.53% in the study group. Up to the age of 80 years the prevalence rate (range: 3.99-4.18%) was increasing with increasing age and highest for the age groups from 50 to 79 years The total rate of psoriasis in children younger than 18 years was 0.71%. The prevalence rates increased in an approximately linear manner from 0.12% at the age of 1 year to 1.2% at the age of 18 years (Schäfer et al., 2011). In France, a case-control study in 6,887 persons, 356 cases were identified (5.16%), who declared having had psoriasis during the previous 12 months (Wolkenstein et al., 2009). The prevalence of psoriasis analyzed across Italy showed that 2.9% of Italians declared suffering from psoriasis (regional range: 0.8-4.5%) in a total of 4109 individuals (Saraceno et al., 2008). The overall rate of comorbidity in subjects with psoriasis aged less than 20 years was twice as high as in subjects without psoriasis. -
Gether with Anti -Synthetase, Ro52 and Jo-1-Double Positive: High Rate of Malignancies, Poorer E.G
Journal of Neuromuscular Diseases 5 (2018) 109–129 109 DOI 10.3233/JND-180308 IOS Press Review Current Classification and Management of Inflammatory Myopathies Jens Schmidt∗ Department of Neurology, Muscle Immunobiology Group, Neuromuscular Center, University Medical Center G¨ottingen, G¨ottingen, Germany Abstract. Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyogra- phy (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis. Keywords: Skeletal muscle, muscle inflammation, myositis, immunosuppression, neuroinflammation, autoimmunity INTRODUCTION requires testing of auto-antibodies, histological eval- uation of a skeletal muscle biopsy and further tests Inflammatory myopathies (synonym: idiopathic including muscle MRI and EMG. Novel diagnostic inflammatory myopathy, IIM) –in short: myositis– criteria have recently been established, but an update are rare conditions that can affect multiple organs will be required (see below for details). -
Celiac Disease and Nonceliac Gluten Sensitivitya Review
Clinical Review & Education JAMA | Review Celiac Disease and Nonceliac Gluten Sensitivity A Review Maureen M. Leonard, MD, MMSc; Anna Sapone, MD, PhD; Carlo Catassi, MD, MPH; Alessio Fasano, MD CME Quiz at IMPORTANCE The prevalence of gluten-related disorders is rising, and increasing numbers of jamanetwork.com/learning individuals are empirically trying a gluten-free diet for a variety of signs and symptoms. This review aims to present current evidence regarding screening, diagnosis, and treatment for celiac disease and nonceliac gluten sensitivity. OBSERVATIONS Celiac disease is a gluten-induced immune-mediated enteropathy characterized by a specific genetic genotype (HLA-DQ2 and HLA-DQ8 genes) and autoantibodies (antitissue transglutaminase and antiendomysial). Although the inflammatory process specifically targets the intestinal mucosa, patients may present with gastrointestinal signs or symptoms, extraintestinal signs or symptoms, or both, Author Affiliations: Center for Celiac suggesting that celiac disease is a systemic disease. Nonceliac gluten sensitivity Research and Treatment, Division of is diagnosed in individuals who do not have celiac disease or wheat allergy but who Pediatric Gastroenterology and Nutrition, MassGeneral Hospital for have intestinal symptoms, extraintestinal symptoms, or both, related to ingestion Children, Boston, Massachusetts of gluten-containing grains, with symptomatic improvement on their withdrawal. The (Leonard, Sapone, Catassi, Fasano); clinical variability and the lack of validated biomarkers for nonceliac gluten sensitivity make Celiac Research Program, Harvard establishing the prevalence, reaching a diagnosis, and further study of this condition Medical School, Boston, Massachusetts (Leonard, Sapone, difficult. Nevertheless, it is possible to differentiate specific gluten-related disorders from Catassi, Fasano); Shire, Lexington, other conditions, based on currently available investigations and algorithms. -
Conditions Related to Inflammatory Arthritis
Conditions Related to Inflammatory Arthritis There are many conditions related to inflammatory arthritis. Some exhibit symptoms similar to those of inflammatory arthritis, some are autoimmune disorders that result from inflammatory arthritis, and some occur in conjunction with inflammatory arthritis. Related conditions are listed for information purposes only. • Adhesive capsulitis – also known as “frozen shoulder,” the connective tissue surrounding the joint becomes stiff and inflamed causing extreme pain and greatly restricting movement. • Adult onset Still’s disease – a form of arthritis characterized by high spiking fevers and a salmon- colored rash. Still’s disease is more common in children. • Caplan’s syndrome – an inflammation and scarring of the lungs in people with rheumatoid arthritis who have exposure to coal dust, as in a mine. • Celiac disease – an autoimmune disorder of the small intestine that causes malabsorption of nutrients and can eventually cause osteopenia or osteoporosis. • Dermatomyositis – a connective tissue disease characterized by inflammation of the muscles and the skin. The condition is believed to be caused either by viral infection or an autoimmune reaction. • Diabetic finger sclerosis – a complication of diabetes, causing a hardening of the skin and connective tissue in the fingers, thus causing stiffness. • Duchenne muscular dystrophy – one of the most prevalent types of muscular dystrophy, characterized by rapid muscle degeneration. • Dupuytren’s contracture – an abnormal thickening of tissues in the palm and fingers that can cause the fingers to curl. • Eosinophilic fasciitis (Shulman’s syndrome) – a condition in which the muscle tissue underneath the skin becomes swollen and thick. People with eosinophilic fasciitis have a buildup of eosinophils—a type of white blood cell—in the affected tissue. -
A Case of Undifferentiated Connective Tissue Disease
Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 59-62 Case Report A case of Undifferentiated connective tissue disease Chatterjee A,1 Chatterjee K,2 Sarkar N,3 1Assistant Professor, 2Senior Resident, Department of Pediatrics, Calcutta National Medical College 3 Registrar, Apollo Gleaneagles Hospital,Kolkata. ABSTRACT Undifferentiated connective tissue disease is an overlap syndrome in which the features of more than one disease is present but their complete diagnosis is lacking. We are presenting an 11year child with fever, arthritis, polyserositis, myalgia, nephritis, sclerodactyly with positive anti-dsDNA and anti-Smith antibody. She improved with prednisolone and cyclophosphamide. Key Words: Undifferentiated connective tissue disease, polyserositis, arthritis, myalgia, sclerodactyly. INTRODUCTION and investigation showed ESR-95mm/hour. After Connective tissue disease result from autoimmune 4 months of fever ,she developed pain, swelling processes that lead to inflammation of target organs. and movement restriction of the large joints, it was Rarely,children develop overlap syndromes, one symmetric with no small joint involvement. She is such is mixed connective tissue disease(MCTD) the third child of a non-consanguinous marriage, where features of two or more major rheumatic with no significant past illness and no family history disorders are seen-juvenile rheumatoid of musculoskeletal disease. arthritis(JRA), systemic lupus erythematosus She was admitted to a hospital at 8 months of fever (SLE), juvenile dermatomyositis(JDM) and with joint pain and swelling, weakness and pallor, systemic sclerosis. Children may also have cervical lymphadenopathy and abdominal undifferentiated connective tissue disease in which distention. Investigation showed Hb-8.3gm/ manifestations strongly suggest but do not meet dL,ESR-60mm/hour, malaria parasite, sputum for diagnostic criteria for a specific rheumatic disease. -
Comorbidities in Psoriasis: the Recognition of Psoriasis As a Systemic Disease and Current Management
Review Derleme 71 DOI: 10.4274/turkderm.09476 Turkderm-Turk Arch Dermatol Venereology 2017;51:71-7 Comorbidities in psoriasis: The recognition of psoriasis as a systemic disease and current management Psoriazisde komorbiditeler: Psoriazisin sistemik hastalık olarak kabul edilmesi ve güncel yaklaşım Göknur Kalkan Ankara Yıldırım Beyazıt University Faculty of Medicine, Department of Dermatology, Ankara, Turkey Abstract Psoriasis, with a worldwide prevalence of 2-3%, is now assumed as a systemic chronic inflammatory disease accompanied by comorbidities while it was accepted as a disease limited only to the skin in the past. There are several classifications of the comorbidities which are more common in patients with moderate to severe psoriasis. Simply, comorbidities can be classified as classic, emerging, related to lifestyle, related to treatment. They can also be categorized as medical comorbidities, psychiatric/psychologic comorbidities, and behaviors contributing to medical and psychiatric comorbidities. In this review, providing early diagnosis and treatment of comorbidities, learning screening recommendations for early detection and long-term disease control and improvement in life quality by integrated, multidisciplinary approach were targeted. Keywords: Psoriasis, comorbidity, systemic disease Öz Tüm dünyada %2-3 oranında görülme sıklığına sahip psoriazis, geçmişte sadece deriye sınırlı kabul edilirken, günümüzde birçok komorbiditenin eşlik ettiği kronik sistemik enflamatuvar bir hastalık olarak ele alınmaktadır. Orta-şiddetli düzeyde -
Rheumatoid Factor: a Novel Determiner in Cancer History
cancers Commentary Rheumatoid Factor: A Novel Determiner in Cancer History Alessio Ugolini 1,2 and Marianna Nuti 1,* 1 Department of Experimental Medicine, “Sapienza” University of Rome, Viale Regina Elena 324, 00161 Rome, Italy; alessio.ugolini@moffitt.org 2 Department of Immunology, H. Lee Moffitt Cancer Center, Tampa, FL 33612, USA * Correspondence: [email protected] Simple Summary: Rheumatoid factors are autoantibodies that characterize different autoimmune diseases, in particular rheumatoid arthritis, but that can also be found in the sera of the general healthy population. They have been mainly studied in the context of autoimmune diseases, but some evidence have suggested an association between their presence and the predisposition to develop cancer as well as a facilitation of cancer growth and progression in oncologic patients. In this review, for the first time we thus analyze and discuss the possible roles that these autoantibodies can assume in tumor history, from determiners of a heightened susceptibility of developing cancer to drivers of a reduced response to immunotherapies. Abstract: The possible interplay between autoimmunity and cancer is a topic that still needs to be deeply explored. Rheumatoid factors are autoantibodies that are able to bind the constant regions (Fc) of immunoglobulins class G (IgGs). In physiological conditions, their production is a transient event aimed at contributing to the elimination of pathogens as well as limiting a redundant immune response by facilitating the clearance of antibodies and immune complexes. Their production can become persistent in case of different chronic infections or diseases, being for instance a fundamental marker for the diagnosis and prognosis of rheumatoid arthritis. -
Antisynthetase Syndrome and Rheumatoid Arthritis: a Rare Overlapping Disease
Case Report Annals of Clinical Case Reports Published: 15 Jul, 2020 Antisynthetase Syndrome and Rheumatoid Arthritis: A Rare Overlapping Disease Makhlouf Yasmine*, Miladi Saoussen, Fazaa Alia, Sallemi Mariem, Ouenniche Kmar, Leila Souebni, Kassab Selma, Chekili Selma, Zakraoui Leith, Ben Abdelghani Kawther and Laatar Ahmed Department of Rheumatology, Mongi Slim Hospital, Tunisia Abstract The association between Antisynthetase Syndrome (ASS) and rheumatoid arthritis is extremely rare. In this case report, we are describing a 16 years long standing history of seropositive RA before its uncommon association to an ASS. A 55-year-old female patient presented at the first visit with symmetric polyarthritis and active synovitis affecting both hands and ankles. Laboratory investigations showed positive rheumatoid factors, positive anti-CCP antibodies and negative ANA. The X-rays were consistent with typical erosive in hands. Thus, the patient fulfilled the ACR 1987 criteria of RA in 2000 at the age of 37. Methotrexate was firstly prescribed. However, it was ineffective after 4 years. Then, the patient did well with Leflunomide until January 2017, when she developed exertional dyspnea. High-resolution CT of the lung revealed Nonspecific Interstitial Pneumonia (NSIP). Autoantibodies against extractable nuclear antigens were screened and showed positive results for anti-Jo1 autoantibodies. She was diagnosed with ASS complicating the course of RA. Keywords: Antisynthetase syndrome; Rheumatoid arthritis; Overlap syndrome; Nonspecific interstitial pneumonia Key Points • Antisynthetase Syndrome should be considered as a clinical manifestation of overlap syndromes, particularly in active RA patients with pulmonary signs and anti-Jo-1 antibody. OPEN ACCESS • An early diagnosis of antisynthetase syndrome in an overlap syndrome is important, as *Correspondence: treatment may need adjustment. -
Sjögren's Syndrome and Sicca Symptoms in Patients with Systemic
al of Arth rn ri u ti o s J Horimoto et al., J Arthritis 2016, 5:1 Journal of Arthritis DOI: 10.4172/2167-7921.1000190 ISSN: 2167-7921 Research Article Open Access Sjögren’s Syndrome and Sicca Symptoms in Patients with Systemic Sclerosis Alex Magno Coelho Horimoto1*, Vinicius de Macedo Possamai2 and Izaias Pereira da Costa1 1Professor of Rheumatology, Medical University of Mato Grosso do Sul, Brazil 2Rheumatologist, Medical University of Mato Grosso do Sul, Brazil Abstract Introduction: Several autoimmune diseases can be accompanied by dysfunction of the salivary glands, regardless of the presence or absence of association with Sjögren’s syndrome (SS). A recent study by Maeshima et al. found salivary hyposecretion in 58.3% of patients with various connective tissue diseases, particularly systemic sclerosis (SSc). Objective: To determine the prevalence of SS and sicca symptoms in patients with SSc. Assess whether the presence of SS in patients with SSc causes worsening of the disease. Methods: 69 SSc patients periodically monitored in the rheumatology clinic at NHU / UFMS composed the study. All patients were questioned about sicca symptoms and clinical features. We evaluated the RF levels, ANA, anti-Ro / La. Results and discussion: 69 SSc patients were enrolled in the study, with average age of 51.2 years, women at 98.3% and white by 50%. Sicca symptoms were present in 48 patients (69.5%) with SSc; 43/69 patients (62.3%) with dry mouth and 46/69 patients (66,7%) with dry eye. Sicca symptoms observed predominantly in patients with diffuse disease (75%). The antinuclear antibody positivity was 95% and the rheumatoid factor (RF) was observed in 14 patients (23.3%).