scholarly journals Chronic Myelomonocytic Leukemia Presenting With Polyserositis and Seropositivity for Rheumatoid Arthritis

2020 ◽  
Vol 8 ◽  
pp. 232470962096686
Author(s):  
Anthony Kunnumpurath ◽  
Sai Prasad Desikan ◽  
Charles McClain ◽  
Raman Desikan

Chronic myelomonocytic leukemia (CMML) is a rare clonal stem cell disorder associated with clinical and pathologic of myelodysplasia and myeloproliferation. Systemic autoimmune/inflammatory disorders (SAID) and polyserositis have been associated with CMML. These manifestations can be observed concomitantly, shortly before diagnosis or anytime along the course of illness. We report a case of myeloproliferative CMML who presented with polyserositis and positive serology for rheumatoid arthritis. Retrospective studies of myelodysplasia/CMML have reported 15% to 25% incidence of SAID. The most commonly observed disorders include systemic vasculitis, connective tissue diseases, polychondritis, seronegative arthritis, and immune thrombocytopenia. SAID does not confer adverse prognosis in retrospective studies. Polyserositis is less common; this may result from leukemic infiltrate or result from autoimmunity. Treatment of serositis includes steroids and cytoreductive agents. Serositis may confer poor prognosis and hypomethylating therapy may improve the outcome.

2009 ◽  
Vol 27 (5) ◽  
pp. 217-223 ◽  
Author(s):  
Elisa Menegatti ◽  
Annalisa Davit ◽  
Simona Francica ◽  
Daniela Berardi ◽  
Daniela Rossi ◽  
...  

Immune and inflammatory response activation is a common feature of connective tissue diseases and systemic vasculitis. The aim of our study was to evaluate the possible involvement of TNFα c.-308A > G, IL-10 c.-1082A > G, uteroglobin c.38A > G, TGFβ 1 c.869C > T and NFκB2 c.-1837T > C gene polymorphisms in susceptibility to connective tissue diseases. Our study cohort included 68 unrelated patients affected by rheumatoid arthritis (RA) (37 patients) and ANCA-positive [micropolyangiitis (mPA) 17 patients] or ANCA-negative systemic vasculitis [including 8 patients with Henoch-Schönlein purpura (HSP) and 6 patients with mixed cryoglobulinaemia (MC)] as well as 98 control subjects. Allele frequency analysis of uteroglobin c.38G > A polymorphism showed a significant increase in the c.38A allele in patients (p= 0.002). Genotype frequency analysis of uteroglobin and NF-κB2 gene polymorphisms in patients showed an increase in c.38GA and c.38AA genotypes in the uteroglobin gene (p=0.02) coupled with an increase in homozygous c.-1837CC in the NF-κB2 gene (p=0.02). Our data suggest that genetic variation in UG and NF-κB2 pathways could have effects in connective tissue disease susceptibility.


2020 ◽  
Vol 1-2 (211-212) ◽  
pp. 67-74
Author(s):  
Akmarzhan Rystanbayeva ◽  
◽  
Aigul Balmukhanova ◽  
Olga Mashkunova ◽  
Kuralay Kaynazarova ◽  
...  

To this date, uveitis associated with connective tissue diseases remain relevant due to serious complications that significantly reduce vision and thus worsen the quality of life of patients. Aim. To study the frequency and prevalence, as well as clinical manifestations of ophthalmopathology in connective tissue diseases. Material and methods. A retrospective and prospective study of 534 patients (144 men and 390 women) with connective tissue diseases was conducted, in which ophthalmological disorders were detected. The patients underwent standard rheumatological examination in the department. And also, ophthalmological examination with the determination of visual acuity, intraocular pressure and the use of biomicroscopy, ophthalmoscopy (or cycloscopy). If necessary and possible, additional studies were performed such as: ultrasound B-scan, optical coherence tomography, perimetry. The study examined the type of common disease, age of onset and gender of the patient. Of particular interest were patients with uveal inflammation, in which the localization and nature of the course of inflammation were evaluated. Results and discussion. The most common diseases with eye damage were rheumatoid arthritis (RA) – 129 (24.1%), systemic lupus erythematosus (SLE) – 92 (17.2%), inflammatory bowel disease (IBD) – 95 (17.8%), systemic scleroderma (SDS) – 54 (10.1%) and spondylarthritis (Spa) – 41 (7.8%). In a small percentage of cases, other connective tissue diseases were diagnosed, such as: mixed connective tissue diseases (MCTS) – 24 (4.5%), Overlap syndrome – 21 (3.9%), systemic vasculitis – 23 (4.3%), Behcet's syndrome -18 (3.4%), Sjogren disease – 14 (2.6%) and juvenile idiopathic arthritis - 7 (1.3%). All ophthalmic disorders were divided into four general groups and distributed as follows: inflammatory 27.7%, degenerative 4.9%, vascular 48.7%, side effects of glucocorticoids 18.7%. Uveitis accounted for 52 (9.7%) of all ophthalmopathology, occurring in connective tissue diseases. Unilateral acute anterior uveitis (AAU) was diagnosed in 13 (25%) patients with ankylosing spondylitis, of which 4 (7.8%) had a relapse in the paired eye. Simultaneously, bilateral AAU was detected in 3 (5.7%) patients with Behcet's syndrome. Chronic, recurrent posterior uveitis was diagnosed in 13 (25%) patients with Behcet's systemic vasculitis. All patients with Behcet's syndrome were male at the age of 31±3.1 years. The uveal process approximately started 3.2±1.16 years after the onset of systemic disease. Generalization of inflammation in all departments of uvea was detected in patients with PSA-5 (9.6%), AC – 3 (5.7%), DS – 2 (3.8%) and SLE – 2 (3.8%). In 5 (9.6%) patients, the process was bilateral. Inflammation of the paired eye by the type of AAU was observed in 1 patient and posterior uveitis - in 2 patients. Conclusions. Ophthalmic disorders in the structure of systemic diseases make up 20.2% and vary widely in their clinical manifestations. Chronic blepharoconjunctivitis, combined with rheumatoid arthritis (46%) is a common inflammation of the accessory structures of the eye. About 20% of patients have complications from general immunosuppressive therapy, leading to impaired visual function. The frequency of uveitis in the structure of ophthalmopathology associated with connective tissue disease is 9.7%. Uveitis associated with ankylosing spondylitis and systemic vasculitis of Behcet occurs in men at a younger age and is 31%. More than half (63%) of patients with uveitis have a severe course with the development of panuveitis and neuropathy. An increase in the thickness of the choroid (up to 2 mm) according to two-dimensional ultrasound is an early diagnostic criterion for the development of uveitis and the underlying disease of connective tissue. Keywords: inflammatory eye diseases, uveitis, connective tissue diseases, spondyloarthritis, Behcet's syndrome, complications.


2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Valentina Cestelli ◽  
Amelia Spinella ◽  
Federica Campomori ◽  
Carmela Esposito ◽  
Sara Ciaffi ◽  
...  

Vasculitis is a heterogeneous group of disorders characterized by the presence of necrotic inflammatory phenomena and destruction of blood vessels. Vasculitis is classified as primary (idiopathic) or secondary to infections, connective tissue diseases and drugs but can also be considered as a paraneoplastic phenomenon. Evidence shows that the increasing use of biological agents results in a growing number of reports of autoimmune diseases induced by these therapies. An inflammatory articular chronic disease such as rheumatoid arthritis may be complicated by extra-articular manifestations, such as cutaneous or systemic vasculitis. Herewith, we describe the case of a great vessels arteritis in a patient affected by rheumatoid arthritis in therapy with an anti-TNF agent (etanercept).


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