Common Connective Tissue Disease Research Articles

Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus

Pulmonary arterial hypertension (PAH) is a severe complication of systemic lupus erythematosus (SLE), and SLE-PAH is the most common connective tissue disease (CTD)-associated PAH in Chinese patients.1 The prognosis of...

Effect of Boswellia on Adjuvant Induced Rheumatoid Arthritis in Experimental Animals

<p>Arthritis is the most common systemic connective tissue disease. About 1% of the world's population is affected by RA; women are three times more often than men. Onset is most frequent between the ages of 40 and 50 years, but people of any age can be affected. Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks flexible joints. Although the cause of rheumatoid arthritis is unknown, autoimmunity plays an important role in both its chronicity and progression<strong>.</strong></p><p><strong></strong>The new trend of medical treatment of rheumatoid arthritis seeks new drugs with more efficacy and fewer side effects. Since tumor necrosis factor alpha (TNF-α) as well as other cytokines act as key players in the development of arthritis.</p><p>Studies in the United States and Europe have shown that their use is less common in clinical settings, but has become increasingly more in recent years as scientific evidence about the effectiveness of herbal medicine has become more widely available. The present work investigates the effect of Boswellia on Adjuvant induced RA in Experimental Animals.</p>

A study of cutaneous manifestations of systemic lupus erythematosus in Malwa region of India

<p class="abstract"><strong>Background:</strong> Systemic lupus erythematosus (SLE) is an autoimmune disease. SLE is the most common connective tissue disease. Its prevalence varies according to geographical and racial background from 3/10 000 in Caucasians to 20/10 000 in Afro-Caribbeans. Around 90% of affected individuals are women, and the peak age at onset is between 20 and 30 years. Limited studies are available in India which have documented the frequency of cutaneous manifestations of SLE, so, this study was planned to evaluate the frequency of skin manifestations of SLE, in a tertiary care centre of Malwa region of India.</p><p class="abstract"><strong>Methods:</strong> All demographic data including age, sex, weight was collected on a pre-designed proforma. All the patients were evaluated for cutaneous manifestations of SLE i.e. malar rash, discoid rash, oral ulcers, photosensitivity etc. All the information was recorded in designed proforma.<strong></strong></p><p class="abstract"><strong>Results:</strong> Among 100 SLE subjects, 82 (82%) patients had skin manifestations, 18 (18%) patients had oral ulcer and 12 (12%) patients had raynaud’s phenomenon at the time of presentation. Among dermatological manifestations, 42% patients had photosensivity, 35% patients had malar rash, 30% patients had discoid rash, 10% patients had alopecia and 2% patients had bullous lesion.</p><p class="abstract"><strong>Conclusions:</strong> Skin lesions in patients with lupus may be specific or nonspecific. This study covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should be involved with other specialties to provide optimal care of SLE patient.</p>

Laboratory diagnostics and pathogenesis of rheumatoid arthritis — the past and the present

Rheumatoid arthritis (RA) is one of the most common systemic connective tissue diseases of autoimmune origin and is characterised by chronic inflammation of joints. The aetiology of this disease has not been fully understood yet. A crucial role in the development of RA is played by multiple genetic (shared epi-tope, HLA-DR genes, family predisposition) and environmental factors (smoking, female-specific factors, bacteria, viruses and mucosal inflammation, periodontal and lung diseases). The purpose of this article is to review the latest data on the pathogenesis of this disease and biomarkers used in the diagnostics of RA. RA is associated with the production of autoantibodies, among which rheumatoid factor (RF) and anti-citrullinated protein (anti-CCP) antibodies are included in the new classification criteria of RA. So far, anti-CCP is the best marker of RA; its high sensitivity and specificity have been helpful in diagnostics and monitoring disease activity and the development of more aggressive disease, as well as the pharmacological treatment used. Recently, anti-carbamylated (anti-carP) antibodies and calprotectin have been described in RA, which also appears to be promising in the diagnostics of this disease.

Prevalence, Characterisation and Diagnosis of Common Connective Tissue Diseases from Coimbatore Region in Tamil Nadu

Prevalence, Characterisation and Diagnosis of Common Connective Tissue Diseases from Coimbatore Region in Tamil Nadu

Severe Thrombocytopenia in Patient with Dermatomyositis.

ABSTRACT: Dermatomyositis (DM) is part of a heterogeneous group of systemic diseases called idiopathic inflammatory myopathies. As in other autoimmune connective tissue diseases (CTD), abnormalities of hematopoietic tissue and/or peripheral blood cells may develop and represent an important prognostic factor. Most common CTD associated with thrombocytopenia (TP) are systemic lupus erythematosus and antiphospholipid syndrome. DM-related TP is less frequent and may develop in the context of an underlying malignancy. Severe TP related to myositis is a very rare occurrence. We report a case of a male patient diagnosed with acute DM, debilitating muscle weakness and rapid development of severe TP.

Systemic Lupus Erythematosus Is Associated With Increased Adverse Postoperative Renal Outcomes and Mortality: A Historical Cohort Study Using Administrative Health Data.

Systemic lupus erythematosus (SLE) is a common autoimmune connective tissue disease that mainly harms kidneys, heart, lungs, and nervous system. Effects of surgical stimulus and anesthesia combined with SLE-related pathologies may increase morbidity and mortality. Therefore, we aimed to evaluate the association between SLE (versus none) and postoperative renal, cardiac, and in-hospital mortality complications among patients undergoing major surgeries. We obtained censuses of 2009 to 2011 inpatient hospital discharges across 7 states and conducted a retrospective cohort study by using International Classification of Diseases and Injuries, Version 9, diagnosis codes, procedure codes, and present-on-admission indicators. We included patients who had major surgery and matched each SLE discharge up to 4 control discharges for potential confounders. We assessed the association between matched SLE patients and controls on in-hospital renal complications, cardiovascular complications, and in-hospital mortality using separate logistic regression models. Among 8 million qualifying discharges, our sample contained 28,269 SLE patients matched with 13,269 controls. SLE was associated with a significantly higher risk of postoperative renal complications, with an estimated odds ratio (99% CI) of 1.33 (1.21, 1.46); P < .001. In addition, SLE was significantly associated with a higher risk of in-hospital mortality, with an estimated odds ratio (99% CI) of 1.27 (1.11, 1.47); P < .001. However, we found no significant association between SLE and cardiac complications, with an estimated odds ratio (99% CI) of 0.98 (0.83, 1.16), P = .79. This is, by far, the largest clinical study for postoperative outcomes of SLE patients with adequately powered statistical analyses. We concluded that SLE was associated with a higher risk of renal complications and in-hospital mortality but not cardiac events after major surgery. In SLE patients, more aggressive measures should be taken to prevent renal injury in the perioperative period.

Pulmonary manifestations in systemic lupus erythematosus

Systemic lupus erythematosus is the most common connective tissue disease that is associated with pulmonary manifestations. Although lupus has the potential to affect any organ, lung involvement is observed during the course of the disease in most cases and it is prognostic for outcome. Pulmonary manifestations in lupus can be classified into five groups based on the anatomical involvement: pleura, lung parenchyma, bronchi and bronchioli, lung vasculature and respiratory muscles can be involved. The most common respiratory manifestations attributable to lupus are pleuritis with or without pleural effusion, pulmonary vascular disease, upper and lower airway dysfunction, parenchymal disease, and diaphragmatic dysfunction (shrinking lung syndrome). In this article the authors summarize lung involvement of lupus, its diagnosis, therapy and prognosis. Orv. Hetil., 2016, 157(29), 1154-1160.

Research progress in the clinical application of autoantibodies in juvenile idiopathic arthritis

Juvenile idiopathic arthritis is a common connective tissue disease of children.The rate of disability is high.Therefore, early diagnosis is important.There are some study on the value of antibodies in juvenile idiopathic arthritis, such as rheumatoid factor, antiperinuclear factor, antikeratin antibody, antibodies to cyclic citrullinated peptides and so on.This review introduces the progress of them. Key words: Arthritis, juvenile, idiopathic; Rheumatoid factor; Antiperinuclear factor; Antikeratin antibody; Citrulline

Immunological markers of rheumatoid arthritis

Rheumatoid arthritis (RA) is the most common connective tissue disease of autoimmune origin. The disease is characterized by chronic inflammation leading to bone erosions and organ involvement. RA is a progressive disease. It affects the quality of life, leading to disability and death mainly due to premature cardiovascular disease. Early diagnosis and appropriate treatment are essential for prognosis and quality of life improvement. In 2010 the American College of Rheumatology (ACR) and The European League Against Rheumatism (EULAR) established new RA classification criteria. Besides clinical symptoms it includes two immunologic criteria: rheumatoid factor (RF) and anti-citrullinated protein antibodies (anti-CCP antibodies). RF is the first well-known RA immunologic marker. It is observed in 80-85% of patients with RA. Elevated serum level of RF has been associated with increased disease activity, radiographic progression, and the presence of extraarticular manifestations. The sensitivity of RF is 50-90%, and specificity is 50-95%. Anti-CCP antibodies appear to be a more specific marker than RF. They are often present at the very beginning of the disease, or even years before the first symptoms. The prognostic value of anti-CCP antibodies is well established. High serum level of anti-CCP correlates with poor prognosis and early erosions of the joints. The sensitivity of anti-CCP2 is 48-80%, and specificity is 96-98%. New immunologic markers include anti-carbamylated protein antibodies (anti-CarP) and antibodies against heterogeneous nuclear ribonucleoproteins (anti-hnRNP A2/B1, RA33). Scientists aim to identify a highly sensitive and specific biomarker of the disease that not only has diagnostic and prognostic value but also may predict the response to treatment.

Mixed connective tissue disease.

The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3.8 per 100,000 and the mean annual incidence to be 2.1 per million per year, supporting the notion that MCTD is the least common CTD. Little is known about the etiology of MCTD, but recent genetic studies have confirmed that MCTD is a strongly HLA (​human leukocyte antigen)-linked disease, as the HLA profiles of MCTD differ distinctly from the corresponding profiles of ethnically matched healthy controls and other CTDs. In the first section of this review, we provide an update on the clinical, immunological, and genetic features of MCTD and discuss the relationship between MCTD and the other CTDs. Then we proceed to discuss the recent advances in therapy and our current understanding of prognosis and prognostic factors, especially those that are associated with the more serious pulmonary and cardiovascular complications of the disease. In the final section, we discuss some of the key, unresolved questions related to anti-RNP-associated diseases and indicate how these questions may be approached in future studies.

Interstitial Lung Disease and Rheumatoid Arthritis: A Review

Interstitial lung diseases (ILDs) are a group of diffuse parenchymal lung disorders that are classified according to different clinical, radiological and histopathological features. Interstitial lung disease can be primary or it can be due to a secondary cause, such as an underlying connective tissue disease (CTD). Rheumatoid arthritis (RA) is the most common CTD, and ILD can be found in a quarter of RA patients. Interstitial lung disease has a significant impact on RA patients in terms of their burden of disease and quality of life. Despite this it remains largely understudied and pathogenesis is unclear. Newer imaging techniques include ultrasound and 18F-FDG PET/CT. Therapeutics that show promise include mycophenolate mofetil and rituximab. Paradoxically, some of the agents that are good in treating articular manifestations in RA patients can result in the worsening or development of ILD.

Pulmonary manifestations in systemic lupus erythematosus: association with disease activity.

Background and objectiveAlthough systemic lupus erythematosus (SLE) is the most common connective tissue disease affecting the lung, few studies have assessed risk factors that predict pulmonary manifestations. The objectives of the present study were to determine the prevalence of lung manifestations in SLE patients from Western Saudi Arabia by analysing results from high-resolution computed tomography (HRCT) scans and to identify independent risk factors for lung involvement.MethodsThis was a 10-year retrospective study involving 184 SLE patients. We examined all HRCT lung abnormalities and determined whether findings were associated with the presence of lupus nephritis (LN), SLE disease activity (as defined by SLE Disease Activity Index 2000 item scores ≥4 for any and all items) or levels of complement and anti-double-stranded DNA (anti-dsDNA).ResultsWe identified 61 patients (33%) with pulmonary involvement, and 52 (85%) of these subjects showed HRCT abnormalities. The most common HRCT findings were pleural effusion, consolidation and atelectasis (58%, 42% and 42%, respectively). There was a significant association between abnormal HRCT results and hypocomplementemia, high levels of anti-dsDNA and disease activity (P < 0.05), particularly with regard to pleuropericardial effusion and consolidation. Pulmonary abnormalities were significantly higher within the first five years after SLE diagnosis (P < 0.001). However, neither disease duration nor LN was associated with increased risk.ConclusionsLung manifestations were frequent in SLE patients from Saudi Arabia, with pleural effusion, consolidation and atelectasis being the most common. Low complement levels, high anti-dsDNA levels and disease activity were significantly associated with abnormal HRCT findings (all P < 0.001).

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review.

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

Systemic lupus erythematosus in male: two case reports

Systemic lupus erythematosus (SLE) is the most common multisystem connective tissue disease. Around 90% of affected individuals are women, with peak onset in the second and third decades. Tissues of all system are damaged by pathogenic auto-antibodies and immune complexes. We report here two cases of SLE in male patient, presented with typical features of SLE. Though the disease is rare in male, but such type of manifestations should be investigated properly to exclude SLE. DOI: http://dx.doi.org/10.3329/bjmed.v24i2.20222 Bangladesh J Medicine 2013; 24 : 82-85

AB0922 Pulmonary Involvement in Connective Tissue Diseases - A Retrospective Analysis of A Multidisciplinary Approach

BackgroundThe lung is a frequent target of connective tissue diseases (CTDs) and all components of the respiratory system are at risk. Interstitial lung disease (ILD) is frequently seen in CTDs,...

SAT0307 Overlap Myositis – the Most Common Form of Autoimmune Myositis

BackgroundAutoimmune myositis (AIM) is a syndrome characterized by proximal muscle weakness, involvement of the cellular and humoral immune systems in skeletal muscle pathology, and presence of autoantibodies (Abs) (myositis-specific (MSAs)...

Connective tissue disease in pregnancy

Connective tissue diseases (CTD) include a variety of chronic multisystem disorders with a high percentage of autoimmune conditions. Many of these conditions affect women of childbearing age and, therefore, pregnancy poses an important challenge for doctors looking after such women. Knowledge of medication safety, the effect of pregnancy on such diseases and vice versa, together with preconception counselling and multidisciplinary team care, are the basic pillars needed to provide the best obstetric and medical care to these women. In this review, we discuss the management of the most common autoimmune CTD before, during and after pregnancy, along with the most relevant issues regarding appropriate medication.

FRI0302 Sjögren’s syndrome and associated organ-specific autoimmune diseases: clinical, immunological and histological profile. argentine study group of sjögren´s syndrome. gessar.

BackgroundSjögren’s Syndrome is the most common connective tissue disease that appears in association with other autoimmune diseases.ObjectivesTo determine if significant differences exist in the clinical, immunological and histological profile between...

FEV1 over time in patients with connective tissue disease-related bronchiolitis

Fibrosis or inflammation of the bronchioles is a well-known manifestation of connective tissue disease (CTD). However, the natural history of CTD-related bronchiolitis is largely unknown. We analyzed consecutive patients evaluated at National Jewish Health (Denver, CO) from 1998 to 2008 with CTD and surgical lung biopsy-confirmed bronchiolitis. Linear mixed effects models were used to estimate the longitudinal postbronchodilator FEV1 %predicted (%pred) course and differences between subjects with or without constrictive bronchiolitis (CB). Of 28 subjects with a mean age of 53±9 years, fourteen (50%) had CB. The most common CTD diagnosis was rheumatoid arthritis (n=14; 50%). There were no significant differences in demographics, smoking status, underlying CTD diagnoses, 6-minwalk distance, dyspnea score or drug therapy between subjects with CB and those with cellular bronchiolitis. Three subjects with CB (11%) and four with cellular bronchiolitis (14%) died. Compared with subjects with CB, those with cellular bronchiolitis had higher mean FEV1 %pred at all times. There were no significant differences in FEV1 %pred slope within- or between-groups (CB vs. cellular bronchiolitis) preceding surgical lung biopsy or afterward. Subjects with CTD-related CB had lower FEV1 %pred values than those with CTD-related cellular bronchiolitis at all time points, but FEV1 %pred remained stable over time in both groups regardless of therapy received.