Autoimmune Disease (AD) in Patients with Myelodysplastic Syndrome (MDS): A Retrospective Single Institution Study.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4736-4736
Author(s):  
Jeyanthi Ramanarayanan ◽  
Alan N. Baer ◽  
Minoo Battiwalla ◽  
Laurie A. Ford ◽  
Meir Wetzler ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Systemic Vasculitis ◽  
Multiorgan Failure ◽  
Clinical Manifestations ◽  
Response To Therapy ◽  
Entire Cohort ◽  
Double Stranded Dna ◽  
Inflammatory Bowel ◽  
The Impact

Abstract Autoimmune disease (AD) can manifest uncommonly either at the time of diagnosis of MDS or during its course. When present, AD generally responds to immunosuppressive therapies, but cytopenias and immunosuppression associated with MDS compromise delivery of these therapies. Few studies have investigated the impact of co-existing AD on the course and outcome of patients with MDS. Our objective was to evaluate the clinical manifestations, laboratory characteristics, response to therapy and survival of MDS patients with AD. Records of patients evaluated at Roswell Park Cancer Institute with pathologically demonstrated MDS between 1993 and 2003 (n=277) were reviewed and patients with evidence of AD were identified. Patients with laboratory abnormalities without disease manifestations were excluded, as were patients with therapy-related MDS following treatment for AD. 13 patients (4%) were identified with co-existing MDS and AD. The initial presentation was AD in 6 (46%) and MDS in 4 (31%), while 3 patients (23%) had near-simultaneous diagnoses of both conditions. The spectrum of AD in these patients included systemic vasculitis in 3 patients, systemic lupus erythematosus in two and rheumatoid arthritis, temporal arteritis, cryoglobulinemia, aphthous stomatitis, pyoderma gangrenosum, inflammatory bowel disease, erythema nodosum and Evans syndrome in one patient each. Anti-double stranded DNA (levels ≥ 40.0 u/ml; normal range 0.0–3.5u/ml), ANA (≥1:160), cold agglutinins, low C3 and elevated ESR (≥100mm/hr) were the serological abnormalities detected at the time of AD diagnosis. Eleven of 13 patients were female, and median age at diagnosis of MDS was 65 years, while the entire cohort was 44% female (p=0.005) and had a median age of 71 yrs at diagnosis. FAB subtypes were RA (n=7), RAEB (n=3), CMMoL (n=2) and RARS (n=1). Cytogenetics were normal in 5 patients; abnormalities in the other 8 patients included −7, +8, and del(5q). The median survival of patients from diagnosis of MDS was 48 months and the survival from diagnosis of AD was 46 months. Known causes of death in 6 patients included sepsis, intracranial hemorrhage, lung cancer and transplant-associated multiorgan failure. Based on this study, AD occurs in 4% of MDS patients, predominantly affects female patients, and has heterogeneous clinical manifestations.The pathobiologic implication of the occurrence of AD at the same time or after the diagnosis of MDS is that the dysplastic clone might be responsible for the induction of immune dysregulation.

Influence of Obesity on Clinical Manifestations and Response to Therapy in Cutaneous Leishmaniasis caused by Leishmania braziliensis

2021 ◽  
Author(s):  
Tainã Lago ◽  
Lucas Carvalho ◽  
Mauricio Nascimento ◽  
Luiz H Guimarães ◽  
Jamile Lago ◽  
...  
Keyword(s):  
Cutaneous Leishmaniasis ◽  
Clinical Presentation ◽  
Clinical Manifestations ◽  
Healing Time ◽  
Response To Therapy ◽  
Leishmania Braziliensis ◽  
Cutaneous Lesions ◽  
Cytokine Levels ◽  
Cure Rates ◽  
The Impact

Abstract Background Cutaneous leishmaniasis (CL) caused by L. braziliensis is characterized by a single ulcer or multiple cutaneous lesions with raised borders. Cure rates below 60% are observed in response to meglumine antimoniate therapy. We investigated the impact of obesity on CL clinical presentation and therapeutic response. Methods A total of 90 age-matched CL patients were included (30 obese, 30 overweight and 30 with normal BMI). CL was diagnosed through documentation of L. braziliensis DNA by PCR or identification of amastigotes in biopsied skin lesion samples. Serum cytokine levels were determined by chemiluminescence. Antimony therapy with Glucantime (20mg/kg/day) was administered for 20 days. Results Obese CL patients may present hypertrophic ulcers rather than typical oval, ulcerated lesions. A direct correlation between BMI and healing time was noted. After one course of Antimony, cure was achieved in 73% of patients with normal BMI, 37% of overweight subjects, yet just 18% of obese CL patients (p<0.01). Obese CL cases additionally presented higher leptin levels than overweight patients or those with normal BMI (p<0.05). Conclusions Obesity modifies the clinical presentation of CL and host immune response, and is associated with greater failure to therapy.

scholarly journals Clinical Manifestations and Mechanisms of Autoimmune Disease-Related Multiple Cerebral Infarcts

2019 ◽  
Vol 28 (8) ◽  
pp. 1045-1052
Author(s):  
Li-Li Sun ◽  
Wen-Xiong Tang ◽  
Min Tian ◽  
Lu Zhang ◽  
Zun-Jing Liu
Keyword(s):  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Lupus Erythematosus ◽  
Transcranial Doppler ◽  
Clinical Manifestations ◽  
Cerebral Infarcts ◽  
Acute Infarction ◽  
Multiple Stroke ◽  
Underlying Mechanisms ◽  
Embolic Signals

It is important to investigate the clinical characteristics and identify the stroke mechanisms of patients with autoimmune disease-related stroke, which are necessary for early etiology diagnosis, accurate treatment and preventive strategies. In this article we retrospectively studied eight cases of acute ischemic stroke associated with autoimmune diseases, and without competing conventional stroke etiologies. The characteristics of stroke (clinical and radiological features), the laboratory tests especially serum D-dimer levels (as a marker of hypercoagulable state), and embolic signals on transcranial Doppler were evaluated for all eight patients. High-resolution magnetic resonance imaging (HRMRI), which can help to evaluate vasculitis was performed in four patients. The possible underlying mechanisms of these cases were discussed based on these manifestations. As a result, autoimmune diseases in our study included systemic lupus erythematosus ( n=5), mixed connective tissue disease ( n=1), central nervous system vasculitis ( n=1), and Takayasu arteritis ( n=1). All eight patients presented with acute infarction lesions in ≥2 vascular territories. Most patients presented with numerous small and medium infarction lesions located in the cortical and subcortical areas. Multiple stroke mechanisms were involved in these cases, including hypercoagulability ( n=4), cardiac embolism ( n=1) and vasculitis ( n=3). Embolic signals could be detected on transcranial Doppler in all three stroke mechanisms. In conclusion, our study revealed the characteristics of autoimmune disease-related stroke. For patients with multiple acute cerebral infarcts within non-single arterial territories, autoimmune disease is an important etiology not to be neglected. Multiple stroke mechanisms were involved in these cases.

Systemic lupus erythematosus—clinical features and aetiopathogenesis

2013 ◽  
pp. 923-937
Author(s):  
Caroline Gordon
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Differential Diagnosis ◽  
Autoimmune Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Immune Complexes ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Life Threatening ◽  
Symptoms And Signs

Systemic lupus erythematosus (SLE or lupus) is a multisystem, autoimmune disease associated with the formation of autoantibodies that form pathological immune complexes and activate a number of inflammatory pathways. The disease is characterized by remissions and relapses (flares) that can present with a variety of clinical manifestations. The symptoms and signs may range from mild features that can be treated easily to organ and even life threatening manifestations requiring potent immunosuppression. This chapter will review the epidemiology and pathology of lupus, then the clinical features including differential diagnosis and investigation of adult patients with SLE. Finally the classification, diagnosis, monitoring and outcome of lupus patients will be discussed.

scholarly journals Recent advances in the management of systemic lupus erythematosus

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 970 ◽  
Author(s):  
Savino Sciascia ◽  
Massimo Radin ◽  
Dario Roccatello ◽  
Giovanni Sanna ◽  
Maria Laura Bertolaccini
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Side Effects ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Therapeutic Strategies ◽  
Systemic Lupus ◽  
Systemic Involvement ◽  
Phase Ii And Iii ◽  
Chronic Autoimmune Disease

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease presenting highly heterogeneous clinical manifestations and multi-systemic involvement. Patients are susceptible to relapse­ and remission, thus making management challenging. Moreover, a considerable number of side effects may occur with conventional therapies; therefore, there is clearly a need for new therapeutic strategies. Since the pathogenesis of SLE is highly complex, it is far from being fully understood. However, greater understanding of the pathways and of the cellular and molecular mediators involved in SLE is being achieved. Emerging evidence has allowed the development of new biological therapeutic options targeting crucial molecular mediators involved in the pathogenesis of SLE. This literature review analyzes the availability of biological and target-directed treatments, phase II and III trials, and new therapies that are being developed for the treatment of SLE.

scholarly journals Anemia in Crohn’s Disease—The Unseen Face of Inflammatory Bowel Disease

Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1046
Author(s):  
Mihaela Dranga ◽  
Lucian Vasile Boiculese ◽  
Iolanda Popa ◽  
Mariana Floria ◽  
Oana Gavril ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Disease Activity ◽  
Bowel Disease ◽  
Clinical Manifestations ◽  
World Health ◽  
Tertiary Center ◽  
Inflammatory Bowel ◽  
The Impact

Background and Objectives: Anemia is the most frequent complication of inflammatory bowel diseases. Clinically, anemia can affect important quality-of-life (QoL) components, such as exercise capacity, cognitive function, and the ability to carry out social activities. The disease activity has a significant impact on QoL, mainly due to clinical manifestations, which are more severe during the periods of disease activity. Our aim was to estimate the impact of anemia on QoL in patients with Crohn’s disease. Material and Methods. We made a prospective study on 134 patients with Crohn’s disease (CD) in a Romanian tertiary center. The CD diagnosis was established by colonoscopy and histopathological examination. In particular cases, additional examinations were required (small bowel capsule endoscopy, computed tomography enterography, and magnetic resonance enterography). Anemia was defined according to the World Health Organization’s definition, the activity of the disease was assessed by Crohn’s disease activity index (CDAI) score, and the QoL was evaluated by Inflammatory Bowel Disease Questionnaire 32 (IBDQ 32). Results: 44.8% patient had anemia, statistically related to the activity of the disease and corticoids use. We found a strong association between QoL and disease activity on all four sub-scores: patients with more severe activity had a significantly lower IBDQ (260.38 ± 116.96 vs. 163.85 ± 87.20, p = 0.001) and the presence of anemia (127.03 vs. 148.38, p = 0.001). In multiple regression analyses, both disease activity and anemia had an impact on the QoL. Conclusions: Anemia has high prevalence in the CD in northeastern region of Romania. Anemia was more common in female patients, in patients undergoing corticosteroid treatment, and in those with active disease. Both anemia and disease activity had a strong negative and independent impact on QoL.

scholarly journals Lepromatous leprosy mimicking systemic lupus erithematosus: a case report

2019 ◽  
Vol 28 (1) ◽  
pp. 77-81
Author(s):  
Niken Kusumaningrum ◽  
Schandra Purnamawati ◽  
Dwi Retno Adi Winarni ◽  
Hardyanto Soebono
Keyword(s):  
Lupus Erythematosus ◽  
Correct Diagnosis ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Lepromatous Leprosy ◽  
Test Results ◽  
Systemic Lupus ◽  
Double Stranded Dna ◽  
Morphological Index ◽  
Dna Antibodies

The clinical manifestations of leprosy are highly variable, and the disease is notorious for being “a great imitator” of several other conditions. Leprosy may manifest with a variety of phenomena resembling those of autoimmune diseases. Herein, we report a 33-year-old male presenting with wounds on his left leg and hyperpigmented skin lesions all over his body. Six years earlier, the patient was diagnosed with systemic lupus erythematosus (SLE). However, therapy for SLE did not control his symptoms; instead, the patient developed features of leprosy, such as anesthetic skin lesions, nerve enlargement, and tenderness. Tests for antinuclear antibodies and anti-double stranded DNA antibodies were negative. Slit-skin smear showed a bacterial index of 6+ and morphological index of 10 %. Lupus band test results were negative. Histological findings were compatible with lepromatous leprosy. The clinical and serological similarities between leprosy and SLE may lead to erroneous diagnosis. Thus, clinicians should be aware of this characteristic for correct diagnosis.

Parasitic infection and autoimmunity

Lupus ◽  
2009 ◽  
Vol 18 (13) ◽  
pp. 1144-1148 ◽  
Author(s):  
G. Zandman-Goddard ◽  
Y. Shoenfeld
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune Response ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Molecular Mimicry ◽  
Uv Light ◽  
Parasitic Infection ◽  
Systemic Lupus ◽  
Parasitic Load ◽  
The Impact

Systemic lupus erythematosus is the prototypic multi-system autoimmune disease characterized by the production of multiple autoantibodies. The development of disease depends on a genetic predisposition and exposure to environmental factors including UV light, drugs, and infections. The association of parasitic infection and the development of autoimmune disease in general and lupus in particular remains elusive. In this paper, we review the recent evidence for protection from autoimmunity by parasites, models of parasite-related autoimmunity, molecular mimicry, the impact of parasitic molecules on the immune response and the association between parasitic load and the degree of autoimmunity.

scholarly journals Myeloperoxidase-antineutrophil Cytoplasmic Antibodies (MPO-ANCA) and Proteinase 3-ANCA without Immunofluorescent ANCA Found by Routine Clinical Testing

2015 ◽  
Vol 42 (5) ◽  
pp. 847-852 ◽  
Author(s):  
Deepak A. Rao ◽  
Kevin Wei ◽  
Joseph F. Merola ◽  
William R. O’Brien ◽  
Samuel U. Takvorian ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Systemic Vasculitis ◽  
Real Life ◽  
Retrospective Chart Review ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Antibody Assays ◽  
Missed Diagnosis ◽  
Inflammatory Bowel ◽  
New Diagnosis ◽  
Antibody Tests

Objective.Concurrent testing for serum antineutrophil cytoplasmic antibodies (ANCA) by indirect immunofluorescence (IF) and by antiproteinase 3 (PR3)/antimyeloperoxidase (MPO) antibody assays may identify patients with PR3-ANCA or MPO-ANCA despite a negative IF (IF-negative MPO/PR3-positive); however, the significance of this result is not clear. We sought to determine whether IF-negative, MPO/PR3-positive results identified any cases of clinically meaningful systemic vasculitis at our institution.Methods.We conducted a retrospective chart review of all IF-negative, MPO/PR3-positive patients identified at our institution over a 2-year period.Results.Of the 2345 samples tested over 2 years, 1998 were IF-negative. Among these IF-negative samples, 49 samples (2.5%) derived from 38 patients tested positive for MPO-ANCA or PR3-ANCA. Only 1 IF-negative, MPO/PR3-positive patient was subsequently diagnosed with ANCA-associated vasculitis (AAV). Eleven IF-negative, MPO/PR3-positive patients (29%) had been previously diagnosed and treated for AAV, all with positive IF and antibody tests prior to treatment. Four patients had evidence of cutaneous vasculitis not attributed to AAV, while several of the remaining IF-negative, MPO/PR3-positive patients had other immunologic disorders, including systemic lupus erythematosus (5 patients) and inflammatory bowel disease (3 patients).Conclusion.In this real-life cohort assayed simultaneously by IF and multiplexed bead assays, the detection of MPO-ANCA or PR3-ANCA without a positive IF rarely led to a new diagnosis of systemic vasculitis, and was more likely to occur in the context of a non-vasculitic inflammatory condition. Our results suggest that concurrent IF and MPO/PR3 testing may be of limited use in preventing a missed diagnosis of new-onset AAV.

scholarly journals AB1051 KIKUCHI FUJIMOTO DISEASE, IS IT SLE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1815.3-1816
Author(s):  
J. Camins-Fàbregas ◽  
V. Ortiz-Santamaria ◽  
N. Busquets-Pérez ◽  
A. Cuervo ◽  
I. Cañas Alcántara ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Response To Treatment ◽  
Systemic Autoimmune Disease ◽  
Histiocytic Necrotizing Lymphadenitis ◽  
Systemic Autoimmune Diseases ◽  
Necrotizing Lymphadenitis

Background:Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by adenopathies and fever. It raises a broad differential diagnosis that includes lymphoproliferative disorders, infections and systemic autoimmune diseases, and diagnostic confirmation is always by histology, which shows histiocytic necrotizing lymphadenitis. Although its course is generally benign and self-limited, it can be associated both at the time of diagnosis and during follow-up with systemic autoimmune diseases, the most frequent of which is systemic lupus erythematosus (SLE).Objectives:To describre the clinical and analytical characteristics of patients diagnosed with KFD and the development of systemic autoimmune disease.Methods:Patients diagnosed with KFD during the 1990s and 2020s are collected in a regional hospital (Granollers General Hospital). The clinic is documented at the diagnosis of EKF, the appearance of systemic autoimmune disease during follow-up and its clinical and analytical characteristics.Results:A total of 7 patients with EKF were diagnosed. All of them women with a mean age at diagnosis of 30 years. Diagnosis was made in all cases with compatible clinical symptoms, fever and lymphadenopathy, and lymph node biopsy confirming histiocytic necrotizing lymphadenitis. At the time of diagnosis, a patient was also diagnosed with SLE. During the follow-up, 4 of the 6 remaining patients developed clinical manifestations compatible with SLE (3 of them with systemic manifestations and a case of subacute cutaneous lupus. The mean time of onset of SLE was 34 months (between 6 months and 5 years). All of them received treatment with hydroxychloroquine, with good response to treatment.The clinical and analytical characteristics are presented in Table 1 below.Conclusion:In our center, 5 of the 7 patients (71%) diagnosed with EKF developed manifestations compatible with SLE. The importance of the diagnosis of EKF lies precisely in the possible association with systemic autoimmune disease, the most common being SLE, so it is recommended that patients be monitored to identify those who develop associated autoimmune disease.Disclosure of Interests:None declared

SAT0179 ADHERENCE TO HYDROXYCHLOROQUINE INFLUENCES THE INCIDENCE OF ORGAN DAMAGE DURING FOLLOW-UP IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1031.1-1031
Author(s):  
J. H. Kang ◽  
S. E. Choi ◽  
H. Xu ◽  
D. J. Park ◽  
S. S. Lee
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Clinical Manifestations ◽  
Medication Possession Ratio ◽  
Organ Damage ◽  
Systemic Lupus ◽  
Laboratory Findings ◽  
The Impact

Background:Objectives:Hydroxychloroquine (HCQ) is a cornerstone drug in patients with systemic lupus erythematosus (SLE), decreasing the risk of flares and comorbidities and improving survival. This study investigated the effects of HCQ adherence on clinical manifestations, disease activity, and organ damage in Korean patients with SLE.Methods:Data on 299 SLE patients were obtained from the Korean Lupus Network registry. Demographic variables, clinical manifestations, laboratory findings, PGA, and SLEDAI-2000 and SLICC damage index scores were recorded at the time of enrollment and repeated annually for 4 consecutive years. Patients were divided into two groups according to the level of HCQ adherence. Adherence was defined by the medication possession ratio and dichotomized as ≤ 80% vs. > 80%. Univariate and multivariate analyses were performed to assess the impact of adherence to HCQ on clinical outcomes.Results:Of the 299 patients, 31 (10.4%) showed poor drug adherence during the follow-up period. Patients with poor HCQ adherence were older (P=0.011), less insured (P=0.024), experienced lower employment (P=0.033), and had a higher rate of comorbidities such as hypertension (P=0.048) and depression (P<0.001). The non-adherent group had higher mean and changed SLICC damage index scores than the adherent group across all 4 years. In the multivariate analysis, HCQ non-adherence was significantly associated with older age (OR, 1.043; 95% CI, 1.006–1.081; P=0.021), depression (OR, 11.98; 95% CI, 1.099–130.6; P=0.042), and an annual increase in the SLICC damage index score (OR, 2.275; 95% CI, 1.369–3.779; P=0.002).Conclusion:HCQ adherence might be influenced by age and depressive mood. Additionally, the poor adherence to HCQ in SLE patients was correlated with higher cumulative organ damage. Therefore, patients with SLE should be educated to take HCQ appropriately to improve their clinical outcome in clinical practice.Disclosure of Interests:None declared

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