scholarly journals Hematological Manifestations among Patients with Rheumatic Diseases

2020 ◽  
pp. 1-10
Author(s):  
Alina Klein ◽  
Yair Molad

<b><i>Background:</i></b> Rheumatic diseases have many hematological manifestations. Blood dyscrasias and other hematological abnormalities are sometimes the first sign of rheumatic disease. In addition, novel antirheumatic biological agents may cause cytopenias. <b><i>Summary:</i></b> The aim of this review was to discuss cytopenias caused by systemic lupus erythematosus and antirheumatic drugs, Felty’s syndrome in rheumatoid arthritis, and autoimmune hemolytic anemia, thrombosis, and thrombotic microangiopathies related to rheumatological conditions such as catastrophic antiphospholipid syndrome and scleroderma renal crisis. <b><i>Key Message:</i></b> The differential diagnosis of various hematological disorders should include rheumatic autoimmune diseases among other causes of blood cell and hemostasis abnormalities. It is crucial that hematologists be aware of these presentations so that they are diagnosed and treated in a timely manner.

Author(s):  
José L. Pablos ◽  
Lydia Abasolo-Alcázar ◽  
José M. Álvaro-Gracia ◽  
Francisco J. Blanco ◽  
Ricardo Blanco ◽  
...  

ABSTRACTBackgroundThe susceptibility of patients with rheumatic diseases, and the risks or benefits of immunosuppressive therapies for COVID-19 are unknown.MethodsWe performed a retrospective study with patients under follow-up in rheumatology departments from seven hospitals in Spain. We matched updated databases of rheumatology patients with SARS-CoV-2 positive PCR tests performed in the hospital to the same reference populations. Rates of PCR+ confirmed COVID-19 were compared among groups.ResultsPatients with chronic inflammatory diseases had 1.32-fold higher prevalence of hospital PCR+ COVID-19 than the reference population (0.76% vs 0.58%). Systemic autoimmune or immune mediated diseases (AI/IMID) patients showed a significant increase, whereas inflammatory arthritis (IA) or systemic lupus erythematosus (SLE) patients did not. COVID-19 cases in some but not all diagnostic groups had older ages than cases in the reference population. IA patients on targeted-synthetic or biological disease-modifying antirheumatic drugs (ts/bDMARD), but not those on conventional-synthetic (csDMARD), had a greater prevalence despite a similar age distribution.ConclusionPatients with AI/IMID show a variable risk of hospital diagnosed COVID-19. Interplay of aging, therapies, and disease specific factors seem to contribute. These data provide a basis to improve preventive recommendations to rheumatic patients and to analyze the specific factors involved in COVID-19 susceptibility.


2013 ◽  
Vol 7 (1) ◽  
pp. 87-95 ◽  
Author(s):  
Fozya Bashal

This article is a review of different management strategies for the hematological manifestations of systemic lupus erythematosus (SLE), the strategies include immunosuppressive drugs, some noval therapies and B-cell depletion for refractory thrombocytopenia in patients with SLE and in antiphospholipid antibody syndrome associated with SLE. The researcher questions the validity of the current classic treatment modes and the article explores the relationships between SLE hematological manifestations and the level of morbidity and mortality burden and focuses on the pathophysiology, diagnostic approaches and management strategies of these manifestations. The researcher focuses on hematological abnormalities because they are the commonest among most manifestations in SLE seen in Anemia, leucopenias and thrombocytopenia. They commonly result from an immune mediated bone marrow failure, excessive peripheral cells destruction or certain drugs and infections. There is also an association between anti-phospholipid antibody syndrome (APS) and SLE referred to as secondary APS or SLE-APS. Furthermore, it was recently found that mycophenolatemofetil acts as corticosteroids and as cyclophosphamide sparing agent. Although there is no specific therapy for cytopenias in SLE, corticosteroids remain the mainstay in the treatment of these patients along with less used other conventional treatment options such as azathioprine, cyclophosphamide and human normal immunoglobulin. There are other novel therapies such as thrombopoietin receptor agonists in thrombocytopenia and the use of autologous hematopoitic stem cells transplantation in refractory SLE-APS that are under review. Some of these therapies include thrombopoietin receptor agonists in thrombocytopenia and the use of autologous hematopoitic stem cells transplantation in refractory SLE-APS. The study concludes that treatment of hematological abnormalities is challenging because the treatment itself can cause undue complications sometimes such as granulocytosis due to infection or the use of high doses of steroids and may occur during acute exacerbations of SLE. It is important to take these factors into consideration for disease therapy and management. Publication Abstract: This article is a review of different management strategies for the hematological manifestations of systemic lupus erythematosus (SLE). The strategies include immunosuppressive drugs, some novel therapies and B-cell depletion for refractory thrombocytopenia in patients with SLE and in anti-phospholipid antibody syndrome associated with SLE. The researcher questions the validity of the current classic treatment modes and the article explores the relationships between SLE hematological manifestations and the level of morbidity and mortality burden while it focuses on the pathophysiology, diagnostic approaches and management strategies. The study concludes that hematological abnormalities are the commonest among most manifestations in SLE, and that their treatment is challenging because the treatment itself can cause undue complications sometimes such as granulocytosis due to infection or the use of high doses of steroids and may occur during acute exacerbations of SLE. It is important to take these factors into consideration for disease therapy and management.


2014 ◽  
Vol 13 (1) ◽  
pp. 49-53
Author(s):  
Md. Abdur Rouf ◽  
Abdul Ahad Mohammed Ryhan Uddin ◽  
AMM Ehteshamul Hoque ◽  
Mohammad Ismail ◽  
Rajat Sanker Roy Biswas ◽  
...  

Hematological abnormalities are frequently encountered in patients with SLE. It was aimed to study the pattern and frequency of hematological abnormalities in patients with SLE. Forty patients were prospectively analyzed who were suffering from SLE in the CMCH over a period of one year. All patients were evaluated according to the case record form with particular reference to hematological manifestations. Most patients were females in the child bearing age. Hematological manifestations were documented in87.5% of patients. Anemia was the most common hematological abnormality detected (57.5%). Iron deficiency anemia(IDA) 60%, Anemia of chronic disease(ACD) 26.9% and Autoimmune hemolytic anemia(AIHA) 8.7 % were the most common cause of anemia detected. The data demonstrated that while hematological manifestations are very common in SLE but they are rarely life threatening.DOI: http://dx.doi.org/10.3329/cmoshmcj.v13i1.19420


1997 ◽  
Vol 77 (05) ◽  
pp. 0856-0861 ◽  
Author(s):  
N Abuaf ◽  
S Laperche ◽  
B Rajoely ◽  
R Carsique ◽  
A Deschamps ◽  
...  

SummaryIn HIV-1 infection, an increased prevalence of anticardiolipin autoantibodies (aCL) and lupus anticoagulant (LA) has been described. In order to see if these antibodies are isolated or, like in autoimmune diseases, associated with hematological disorders and with antibodies to other phospholipids and to proteins of coagulation, we investigated 3 groups of patients: 1. 342 HIV-1 infected patients, 2. 145 control patients including 61 systemic lupus erythematosus (SLE) patients, 58 patients with a connective tissue disease, 15 patients with stroke, 11 patients with syphilis and 3.100 blood donors. In HIV-1 infection antiprothrombin (aPrT) antibodies were present in 25% of patients, the prevalence of antiphosphatidylcholine antibodies (aPC) (50%) was almost as high as aCL (64%), and 39% had both antibodies. Absorption on liposomes of the latter revealed an heterogeneous mixture of aCL and aPC or cross-reacting antibodies. In contrast with SLE, anti-β2-glycoprotein I (4%), LA (1%), biological false positive test for syphilis (0.3%), thrombosis (p <0.001) were uncommon. In HIV-1 infection, antiphospholipid antibodies do not associate with features linked to them in SLE or syphilis.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 862.2-863
Author(s):  
M. K. Chung ◽  
J. S. Park ◽  
H. S. Lim ◽  
C. H. Lee ◽  
J. Lee

Background:Rheumatoid arthritis (RA) predominantly affects women and has a significant impact on childbearing. Several population-based studies identifying incidence, prevalence, and medication use of RA have been reported, yet epidemiological studies focusing on women with RA in childbearing years are missing.Objectives:We aimed to identify the incidence, prevalence and medication use of RA among Korean women in childbearing years.Methods:From National Health Insurance Service (NHIS) data (2009-2016), containing inpatient and outpatient claim information for approximately 97% of the Korean population, we identified 9,217,139 women aged between 20-44 years. Incidence and prevalence of RA in the specific sociodemographic group of women in childbearing age were analyzed, and the prevalence of medication prescription were compared between women with RA and controls without rheumatic diseases such as RA, systemic lupus erythematosus, and ankylosing spondylitis. Individuals with RA were defined by the presence of International Classification of Disease, 10th revision code, M05. The medication use was defined as receiving > 90days prescriptions of NSAIDs, corticosteroids (CSs), and conventional synthetic (cs) disease modifying antirheumatic drugs (DMARDs) or > 1day prescription of biologic (b) DMARDs.Results:Total 24,590 women with RA were identified. The average incidence of RA during 2011-2016 among women in childbearing years was 24.1/100,000 person-years (PYs) (95% CI 20.91-27.31) with a yearly increase from 20.99/100,000 PYs in 2011 to 28.38/100,000 PYs in 2016. The average prevalence of RA during 2009-2016 among women in childbearing years was 105.2/100,000 PYs (95% CI 99.0-111.5) with a minimum of 95.7/100,000 PYs in 2009 and a maximum of 110.5/100,000 PYs in 2016. There were increasing trends in both incidence and prevalence of RA according to age among women in childbearing years peaking in the age group of 40-44 years. The prescriptions of NSAIDs, CSs, csDMARDs and bDMARDs were more frequent in women with RA than controls (NSAIDs; 94.21% vs 21.79%, CSs; 83.65% vs 4.28%, csDMARDs; 91.23% vs 0.41%, bDMARDs; 0.11% vs 0%, p<0.001).Conclusion:The incidence and prevalence of RA are high among Korean women in childbearing years, and medication use was significantly more frequent in this specific population than controls. High disease burden is imposed upon women in childbearing years.References:[1] Won S, Cho SK, Kim D, Han M, Lee J, Jang EJ, Sung YK, Bae SC: Update on the prevalence and incidence of rheumatoid arthritis in Korea and an analysis of medical care and drug utilization. Rheumatol Int 2018, 38(4):649-656.[2] Smeele HTW, Dolhain R: Current perspectives on fertility, pregnancy and childbirth in patients with Rheumatoid Arthritis. Seminars in arthritis and rheumatism 2019, 49(3s):S32-s35.Table 1.Medication use among women with RA and controls in childbearing age between 20-44 years during 2009-2016Control(n=155,486)RA(n=23,756)n(%)n(%)PNSAIDs33,887(21.79)22,380(94.21)<.0001Steroids6,653(4.28)19,871(83.65)<.0001csDMARDs634(0.41)21,673(91.23)<.0001bDMARDs0(0.00)27(0.11)<.0001RA, rheumatoid arthritis; NSAID, non-steroidal anti-inflammatory drug; cs, conventional synthetic; b, biologic; DMARDs, disease modifying antirheumatic drugsDisclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1256.3-1257
Author(s):  
S. Lambova

Background:Videocapillaroscopy is the gold standard for evaluation of nailfold capillaries and the major tool used for differentiation of primary and secondary Raynaud’s phenomenon (RP) in rheumatology practice. However, nowadays, there are also accessible alternatives such as USB capillaroscopes, which offer the opportunity to apply capillaroscopic examination at a significantly lower price.Objectives:The aim of the current study was to study the utility of USB capillaroscope (Dinolite) via assessment of capillaroscopic images obtained by patients with primary and secondary RP in rheumatic diseases.Methods:The study represents analysis of capillaroscopic images of 32 patients with RP – primary and secondary in the context of SSc or other rheumatic diseases i.e., undifferentiated connective tissue disease (UCTD) and systemic lupus erythematosus (SLE). All the patients had signed an informed consent for participation in a study of their capillaroscopic, laboratory and clinical associations. The study represents retrospective analysis of the capillaroscopic images obtained from 8 fingers (II-V bilaterally) using USB capillaroscope (Dinolite) at magnification 200x. Capillary diameters were measured (arterial, venous and apical loop) as well as the number of capillaries per millimeter. The capillaroscopic images were categorized into the following groups i.e., I. Absence of microangiopathy: i) normal pattern, ii) nonspecific changes (dilated capillaries with arterial diameter > 0.015mm, venous > 0.020mm; haemorhhages and/or other nonspecific changes), II. Presence of microangiopathy i.e., “scleroderma”/”scleroderma-like” pattern. Presence of giant capillaries with capillary diameter >0.050mm was considered as a sufficient criterion for classifying the image as “scleroderma”/”scleroderma-like” pattern. For “scleroderma” type images in SSc patients staging of Cutolo et al (2000) was used i.e., “early”, ”active”, ”late” phase (1).Results:Images suitable for analysis with good visibility that permits analysis of the major capillaroscopic parameters were available in all patients. Among 32 included patients, 9 patients were with SSc, 12 cases with primary RP, and 10 patients with secondary RP in other CTD (7 patients with UCTD and 3 patients with SLE). „Scleroderma“ pattern was detected in 6 patients with SSc and in all these cases the capillaroscopic images were classifiable into one of the three distinct phases i.e., “early”, ”active” and ”late” phase. Presence of microvascular changes (“scleroderma-like” pattern) was detected also in 5 among the 10 patients with other CTD i.e., UCTD and SLE. In primary RP patients capillaroscopy revealed either normal pattern or nonspecific findings but without features of microangiopathy.Conclusion:Good capillaroscopic images, which could be analyzed and interpreted, are usually obtained using USB capillaroscope. This permits evaluation of the major capillaroscopic parameters. The available software although less sophisticated vs those of videocapillaroscopes, gives the opportunity for measurement of capillary diameters, mean capillary density, etc. The images received from USB capillaroscope are easily classified into “scleroderma”, “scleroderma-like”, non-specific changes and normal pattern. The most important conclusion from capillaroscopy is about presence or absence of microangiopathy. This was easily detected via USB capillaroscope that could be suggested as an ideal alternative for videocapillaroscopes in every day rheumatology practice especially at low budget cases. Measurements of capillary diameters and capillary density provide quantitative data that make these devises also appropriate for scientific research.References:[1]Cutolo M, Sulli A, Pizzorni C AS. Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol. 2000;27(1):155–60.Disclosure of Interests:None declared.


Lupus ◽  
2021 ◽  
pp. 096120332110310
Author(s):  
Mehmet Ersin ◽  
Mehmet Demirel ◽  
Mehmet Ekinci ◽  
Lezgin Mert ◽  
Çiğdem Çetin ◽  
...  

Objective Osteonecrosis (ON), also known as avascular necrosis, is characterized by the collapse of the architectural bone structure secondary to the death of the bone marrow and trabecular bone. Osteonecrosis may accompany many conditions, especially rheumatic diseases. Among rheumatic diseases, osteonecrosis is most commonly associated with systemic lupus erythematosus (SLE). We assessed prevalence and distribution pattern of symptomatic ON in patients with SLE and compare the natural courses of hip and knee ON. Methods 912 SLE patients admitted between 1981 and 2012 were reviewed. SLE patients with symptomatic ON were retrospectively identified both from the existing SLE/APS database. The prevalence of symptomatic ON was calculated; with ON, the joint involvement pattern was determined by examining the distribution of the joints involved, and then the data about the hip and knee joints were entered in the Kaplan-Meier analysis. Kaplan-Meier methods were used to calculate 5- and 10-year rates of ON-related hip (the hip group) and knee survival (the knee group). Results Symptomatic ON developed in various joints in 97 of 912 patients with SLE, and the overall prevalence of ON was detected as 10.6%. The mean age at the time of SLE and ON diagnoses were 27.9 ± 9.9 (14–53) and 34.2 ± 11.3 (16–62) years, respectively. The mean duration from diagnosis of SLE to the first development of ON was 70.7± 60.2 (range = 0–216) months. The most common site for symptomatic ON was the hips (68%, n=66), followed by the knees (38%, n = 37). According to Kaplan-Meier analysis, hip and knee joint survival rates associated with 5-year ON were 51% and 88%, and 10-year survival rates were 43% and 84%, respectively. Conclusion We observed that the prevalence of symptomatic ON in patients with SLE was 10.6%. With the estimated 10-year survival rates of 40% versus 84% for the hip and knee joints, respectively, hip involvement may demonstrate a more aggressive course to end-stage osteoarthritis than the knee involvement.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1277.3-1278
Author(s):  
T. Oton ◽  
L. Carmona ◽  
J. L. Andréu Sánchez

Background:Methotrexate (MTX) is currently a mainstream drug in the treatment of rheumatic diseases. However, the response to MTX is not universal and may be conditioned by a number of factors, among which adherence could be crucial.Objectives:The aim of this study is to explore adherence to MTX in patients with rheumatic diseases, facilitators and perceived when taking and maintaining the prescription.Methods:A qualitative study of content analysis was performed. Focus groups with patients taking either oral or subcutaneous MTX (being the main or coadjuvant treatment) for any rheumatic disease was performed. The groups were moderated by a rheumatologist that was unknown for the patients. The speech was recorded and transcribed. Subsequently, an inductive coding was performed with the help of Atlas.ti and main themes and sub-themes were extracted, with examples of verbatim anonymized speech.Results:Three focus groups were conducted, with a total of 12 participants, of whom eight were women, seven had rheumatoid arthritis, three had psoriatic arthritis, one had spondyloarthritis, and one had systemic lupus erythematosus. All patients reported an adequate adherence to treatment. The barriers identified were: information in the leaflet, technical language in the consults, difficult access to doctor´s appointment, social environment, side effects and the subcutaneous device. As facilitators, the following aspects were discussed: good predisposition of the physician, reliable graphic information, role of associations and partners support.The unmet needs detected were: problems with travelling, protocols for eventualities, absence of a plan of care, neglection of “non-physical” symptoms, disinformation on side effects and training in complementary aspects.Conclusion:Getting reliable information was the main barrier identified. The environment and side effects may also negatively impact on adherence. Shared decision making is a goal to be achieved in the future in these patients.Disclosure of Interests:Teresa Oton Consultant of: Novartis Farmaceutica, SA, Pfizer, S.L.U., Merck Sharp & Dohme España, S.A., Roche Farma, S.A, Sanofi Aventis, AbbVie Spain, S.L.U., and Laboratorios Gebro Pharma, SA (All trhough institution), Loreto Carmona Grant/research support from: Novartis Farmaceutica, SA, Pfizer, S.L.U., Merck Sharp & Dohme España, S.A., Roche Farma, S.A, Sanofi Aventis, AbbVie Spain, S.L.U., and Laboratorios Gebro Pharma, SA (All trhough institution), José Luis Andréu Sánchez: None declared


2021 ◽  
pp. 95-102
Author(s):  
Everardo Arias Torres ◽  
Yongen Chang ◽  
Sheetal Desai ◽  
Ian Chang ◽  
Jonathan E. Zuckerman ◽  
...  

Thrombotic microangiopathies (TMAs) involve multiple organ systems due to the presence of microangiopathic hemolysis. One such condition, atypical hemolytic uremic syndrome (aHUS), is a complement-mediated process that is part of a spectrum of disorders that have underlying complement dysfunction of the alternative pathway due to overactivity or decreased self-nonself discrimination by innate immunity. Complement-amplifying conditions such as pregnancy may unmask a diagnosis of aHUS. We present an important case of a pregnant 23-year-old Hispanic female who presented in mid-gestation (21 weeks) with an initial diagnosis of systemic lupus erythematosus (SLE) complicated by aHUS. She met clinical criteria for aHUS on presentation and was found to have a pathogenic CFHR1–3 homozygous deletion. She has been treated with intravenous and oral steroids, cyclophosphamide, subsequently also with plasma exchange, and finally with eculizumab with partial improvement in renal function. This case adds to the emerging literature showing that SLE and aHUS (or complement-mediated TMA) can be successfully treated with C5 blockade.


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