MO223MEMBRANOUS NEPHROPATHY ASSOCIATED TO ANKYLOSING SPONDYLITIS: CASE REPORT

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Aldjia LAMRI
Keyword(s):  
Ankylosing Spondylitis ◽  
Renal Involvement ◽  
Type I ◽  
Hla B27 ◽  
Ankylosing Spondilitis ◽  
History Of ◽  
Citrullinated Vimentin ◽  
Heat Shock Protein 65 ◽  
Pedal Edema ◽  
Protein Reduction

Abstract Background and Aims Ankylosing Spondylitis (AS) is a chronic inflammatory rheumatic disease diagnosed by the presence of the HLA-B27 antigen with joint and extra-articular manifestations. Its pathogenesis was initially based on auto-inflammatory phenomena, mainly involving innate immunity. However, many studies carried out in the recent years focus on its adaptive immunity aspect, especially autoimmune. These mechanisms seem to interact with each other, resulting in a complex pathophysiology. The general autoimmune characteristics of AS were investigated by Yuan and al, who reviewed recent reports of autoantibodies levels in AS patients. Their analysis shows that none of the autoantibodies considered in the study meet the criteria to be considered as a biomarker for the disease (including antibodies Anti- :CD74; beta2-Microglobulin; mutated Citrullinated Vimentin (MCV); Heat Shock Protein 65 (HSP65); 14-3-3 eta autoantibodies (14-3-3η); 1A-dependent autoantibody magnesium anti-protein phosphatase (PPM1A); sclerostin (SOST); and Anti-microbial antibodies). Renal involvement in AS is dominated by amyloidosis and IgA nephropathy. In rare cases, this disease has been associated with Membranous Nephropathy (MN). The pathogenetic link between the two disorders remains obscure. However, the recent connection of AS to autoantibodies potentially indicates the involvement of immune complexes formed from these autoantibodies in the development of MN. Method We report the case of a 36-years-old woman with a 7 years history of sacroiliitis, who also developed a pedal edema in May 2020. AS was diagnosed in 2014 by the presence of positive HLA-B27, and clinical and radiological manifestations of bilateral sacroiliitis without extra renal manifestations treated by indometacin during periods of pain . A pure nephrotic syndrome was revealed with a 24h proteinuria of 12g / 24h.The patient’s Albumin levels were at 16g/l, without HTA or hematuria, and with a correct renal function (Creatinine at 60mmol/l). A renal biopsy showed a MN with type I polytypical. Light microscopy showed a thickening of capillary loops, while IF staining revealed granular deposits of IgG along the capillary wall. Investigations of further secondary MNs were negative, and the patient was aPLA2R-negative. Results A treatment by corticosteroids was initiated prior biopsy to her referral , which resulted in pain relief and urinary protein reduction (prot 24h 1.5g/24h). Given her good response to this initial treatment, corticotherapy was maintained. As the levels of inflammation and discomfort were low, the patient did not wish to be treated by biotherapy. Conclusion This case suggests a secondary MN in association with AS.The discovery of new autoantibodies associated with AS opens up promising perspectives, and could potentially lead to the characterization of biomarkers for screening and monitoring this disease. However, more studies are needed in order to improve our understanding of the role played by possible immune complex diseases, (in particular MN), in relation to this Ankylosing spondilitis.

MRI Detection of Active Sacroiliitis in First Degree Relatives of Ankylosing Spondylitis Patients with Clinical and Laboratory Correlations

2021 ◽  
Author(s):  
Haron Obaid ◽  
Stephan Milosavljevic ◽  
Udoka Okpalauwaekwe ◽  
Brenna Bath ◽  
Catherine Trask ◽  
...  
Keyword(s):  
Back Pain ◽  
Ankylosing Spondylitis ◽  
Disease Activity ◽  
Hla B27 ◽  
Physical Test ◽  
First Degree Relatives ◽  
Pain Provocation ◽  
History Of ◽  
Active Sacroiliitis

Abstract Background. Detection of ankylosing spondylitis (AS) in the preclinical stage could help prevent long term morbidity in this patients’ population. The aim of this study was to examine the prevalence of active sacroiliitis in first-degree relatives of AS patients using MRI with clinical and laboratory correlations as these patients may benefit from MRI screening and early treatment.Methods. Seventeen first-degree relatives of AS patients were recruited prospectively. AS screening questionnaires (Ankylosing Spondylitis Disease Activity Score, Bath Ankylosing Spondylitis Disease Activity Index & Visual Analogue Scale), blood tests (C-Reactive Protein, HLA-B27), and an MRI of the SIJs were taken. Two musculoskeletal radiologists interpreted the MRI scans, and two physiotherapists applied four symptom provocation tests (Gaenslen's test, posterior pelvic pain provocation test, Patrick's Faber (PF) test and palpation of the long dorsal SIJ ligament test), and two functional movement tests (active straight leg raise and Stork test). Results. Seven (41%) of the 17 participants demonstrated MRI evidence of active sacroiliitis. Of the 7 participants with active sacroiliitis, two (29%) had no history of recent low back pain (LBP), two (29%) had negative HLA-B27, and one (14%) participant had neither back pain nor positive HLA-B27. The Cohen's Kappa score for the interobserver agreement between the radiologists was 1.00 (p-value <0.0001). Despite fair to strong between therapist agreement for the physical test outcomes (Kappa 0.26 to 1.00), the physical test results per se did not have any predictive association with a positive MRI.Conclusions. MRI detected active sacroiliitis in 41% of first-degree relatives of AS patients. The lack of a history of prior LBP or positive HLA-B27 in active sacroiliitis participants might suggest that MRI screening for this high-risk population is warranted; however, further larger studies are needed to help elucidate its cost-effectiveness and long-term benefits.

Influence of HLA-B*5703 and HLA-B*1403 on Susceptibility to Spondyloarthropathies in the Zambian Population

2008 ◽  
Vol 35 (11) ◽  
pp. 2236-2240 ◽  
Author(s):  
ROBERTO DÍAZ-PEÑA ◽  
MIGUEL ANGEL BLANCO-GELAZ ◽  
PANGANANI NJOBVU ◽  
ANTONIO LÓPEZ-VAZQUEZ ◽  
BEATRIZ SUÁREZ-ÁLVAREZ ◽  
...  
Keyword(s):  
Human Immunodeficiency Virus ◽  
Ankylosing Spondylitis ◽  
Reactive Arthritis ◽  
Hiv Positive ◽  
Sample Population ◽  
Type I ◽  
Hla B27 ◽  
New Findings ◽  
Immunodeficiency Virus ◽  
Sub Saharan

ObjectiveTo analyze the distribution of HLA-B alleles and to investigate their contribution in the susceptibility to spondyloarthropathies (SpA) in a sample population from Zambia, in order to determine a relationship between some HLA-B alleles and development of ankylosing spondylitis (AS), reactive arthritis (ReA), or undifferentiated SpA (uSpA).MethodsWe selected 72 patients with SpA and found that 46 had uSpA, 23 ReA, and 3AS.We also selected 92 matched controls; 55 of these had human immunodeficiency virus type I (HIV-I) infection.ResultsWe found a significant increase in the rate of uSpA and ReA with features of Reiter’s syndrome (RS) in HIV-positive individuals who carried the HLA-B*5703 allele (pc < 0.0001 and pc < 0.001, respectively). Among the significant new findings identified were the presence of B*1403 in 2 of the 3 AS patients (pc < 0.05, OR 47), confirming previous data in the Togolese population.ConclusionThe presence of B*5703 and HIV infection may not affect susceptibility to AS and ReA, but they do show an important influence in uSpA and RS. Our findings confirm that HLA-B* 1403 is the only factor to increase the risk of AS in a sub-Saharan African population, whereas HLA-B27 was virtually absent in patients with AS.

scholarly journals Undifferentiated seronegative spondyloarthritis with inflammatory bowel disease and a family history of psoriasis. Sicca syndrome

2013 ◽  
pp. 189-191
Author(s):  
Norma Marigliano ◽  
Domenico Galasso
Keyword(s):  
Inflammatory Bowel Disease ◽  
Psoriatic Arthritis ◽  
Ankylosing Spondylitis ◽  
Bowel Disease ◽  
Acute Inflammation ◽  
Clinical Manifestations ◽  
Hla B27 ◽  
History Of ◽  
Seronegative Spondyloarthritis ◽  
Inflammatory Bowel

Background: Seronegative spondyloarthritis is characterized by the presence of subcutaneous nodules, asymmetrical peripheral arthritis, sacroileitis with or without spondylitis, and rheumatoid-factor negativity. Other common clinical manifestations include oral ulcers, conjunctivitis, and cutaneous lesions such as psoriasis. Familial aggregation has also been described. According to the 1986 classification, corresponding clinical entities include ankylosing spondylitis, psoriatic arthritis, Reiter’s syndrome, arthritis associated with inflammatory bowel disease (IBD), and undifferentiated spondyloarthritis. The disease is also frequently associated with the HLA B27 antigen. From the clinical point of view, there are often incomplete forms of spondyloarthritis, such as reactive arthritis triggered by asymptomatic infections, psoriatic arthritis without psoriasis itself, initial phases of specific forms of spondyloarthritis or the phase of ankylosing spondylitis characterized by sacroiliac lesions, and all forms that remain undifferentiated for long periods of time. Moreover, there are close relations between arthropathy and IBDs, such as Crohn’s disease, ulcerative colitis, and Whipple’s syndrome. Recently, microscopic inflammatory bowel lesions and psoriatic arthritis have been described. Case report: A 30-year-old man (HLA B27-negative) who had been vaccinated against TBC and HBV presented with a 6-year history of recurrent episodes of predominantly left-sided sciatica. The pain was worse at night and during rest. He was suffering from bilateral sacroileitis without spondylitis. Three to five times a day, usually after eating, he passed watery feces containing mucous and small amounts of bright red blood. Colonoscopy revealed pancolitis with histological evidence of chronic inflammation interspersed with areas of acute inflammation, edema, hyperemia, and glandular distortion. One year later, the clinical manifestations and histological findings were essentially unchanged: glandular distortions, chronic and acute inflammation of the lamina propria and crypt microabscesses. There were no granulomas and no evidence of uveitis. The inflammatory index was positive; FR, ENA, ANA titers were negative. He began therapy with adalimumab (loading dose 80 mg followed by 40 mg every 15 days) and mesalazine (2.4 g per os), and the clinical manifestations of the disease improved significantly.

scholarly journals REASONS FOR DIAGNOSTIC DELAYS OF AXIAL SPONDYLOARTHRITIS

2019 ◽  
Vol 72 (9) ◽  
pp. 1607-1610
Author(s):  
Robert Zwolak ◽  
Dorota Suszek ◽  
Aleksandra Graca ◽  
Marcin Mazurek ◽  
Maria Majdan
Keyword(s):  
Back Pain ◽  
Ankylosing Spondylitis ◽  
Axial Spondyloarthritis ◽  
Diagnostic Delay ◽  
Connective Tissue Diseases ◽  
Inflammatory Back Pain ◽  
Hla B27 ◽  
History Of ◽  
Axial Symptoms ◽  
Diagnostic Delays

Introduction: The probability of development of axial spondyloarthritis (axSpA) is estimated to be above 90% among patients with chronic back pain, presence of HLA B27 antigen and positive family history of ankylosing spondylitis (AS), psoriasis, reactive arthritis, inflammatory bowel disease or uveitis. The nonradiographic axSpA and ankylosing spondylitis diseases’ activity has a comparable impact on the patients’ quality of life and from the practical point of view the approach to treatment of each of them is the same. The aim: The attempt to identify the reasons of diagnostic delays of AS among patients hospitalized in the Rheumatology and Connective Tissue Diseases Department in Lublin and to suggest the ways of improving the accuracy of diagnostic track among other healthcare providers than rheumatologists. Material and methods: We performed a retrospective analysis of the records of 82 patients’ with the established diagnosis of AS, hospitalized in the Rheumatology and Connective Tissue Diseases Department in Lublin in 2000-2019, and of 45 years of age and older. Results: From among 82 patients (28 women and 54 men) the diagnosis of AS after 45 years of age was established in 25 patients (10 women and 15 men) – group t, and in the other 57 patients (group n) the diagnosis was established before 45 years of age. On average the age at the time of diagnosis in the whole group (t+n) was 40,7±10,2 (18-76) years, the age at the beginning of inflammatory back pain (age of axial symptoms) was 30,9±8,5 (13-51) years and the diagnostic delay (period between first axial symptoms and diagnosis establishment) was 9,75±9,5 (0-46) years. We did not find any statistically significant associations between sex and age at the moment of diagnosis, age of the beginning of axial symptoms and the time of diagnostic delay. There was no significant difference of incidence of enthesitis, uveitis, arthritis, prevalence of family history of spondyloarthritis and CRP level between group t and n. Antigen HLA B27 was more frequently present in group t. Conclusions: Instead of the recognition progress and worldwide popularization of knowledge about axSpA, the diagnostic delays in this field are still estimated to last many years, the patients are looking for other specialists’ help, and they can be not knowledgeable of the inflammatory back pain criteria. Currently, HLA B27 antigen and C-reactive protein are the two most commonly used biomarkers for diagnostic and disease activity monitoring purposes of axSpA and magnetic resonance is the only “imaging biomarker” The presence of extra-axial symptoms does not improve the diagnostic sensitivity.

scholarly journals Frequency of HLA-B27 gene among patients with ankylosing spondylitis and its consequences on clinical manifestation

2019 ◽  
Vol 9 (3) ◽  
pp. 188-192
Author(s):  
Marjoa Humaira Mekhola ◽  
Mohammad Abdul Jalil Chowdhury ◽  
Mohammad Shamim Ahmed ◽  
Abed Hussain Khan ◽  
Shrebash Paul
Keyword(s):  
Back Pain ◽  
Ankylosing Spondylitis ◽  
Clinical Manifestation ◽  
Human Leukocyte ◽  
Cross Sectional Study ◽  
Hla B27 ◽  
Cross Sectional ◽  
Reactive Protein ◽  
History Of ◽  
High Level

Background: Ankylosing spondylitis is an inflammatory disease and leading case of back pain worldwide. It is thought that the gene human leukocyte antigen-B27 (HLA-B27) provides a strong tendency in people to develop ankylosing spondylitis. This study was designed to evaluate the frequency of HLA-B27 among patients with ankylosing spondylitis and clinical manifestation among the HLA-B27 positive and negative patients. Methods: A cross-sectional study was done in the Department of Medicine and Department Rheumatology of BSMMU, Shahbag, Dhaka, Bangladesh from 1st January 2016 to 30th June 2016. Results: Total 70 patients were included in this study, among those 54 were male and 16 were female and total 54 patients (77.14%) were HLA-B27 positive and 16 patients (22.86%) were HLA-B27 negative. Most HLAB27 positive patients had extra-articular manifestation, family history of low back pain and high level of BASDAI score, compared to those who were negative. Among the 54 positive patients, 9 (16.67%) had combination of tendinitis, enthesitis and uveitis and 2 (3.70%) had all four of extra-articular manifestation. Among the 16 negative patients, 2 (12.5%) had all four extra-articular manifestation. Among 16 negative patients 9 (56.25%) had raised erythrocyte sedimentation rate (ESR) and 5 (31.25%) had raised C-reactive protein (CRP). On the other hand, among 54 positive patients 48 (88.89%) had raised ESR and 44 (81.48%) had raised CRP. Conclusion: The association of HLA-B27 might be the reason of disease severity among the positive patients. Birdem Med J 2019; 9(3): 188-192

A brief history of ankylosing spondylitis

2016 ◽  
Author(s):  
Stefan Siebert ◽  
Sengupta Raj ◽  
Alexander Tsoukas
Keyword(s):  
Ankylosing Spondylitis ◽  
Clinical Features ◽  
Association Studies ◽  
Genome Wide Association ◽  
Genome Wide Association Studies ◽  
Hla B27 ◽  
The Past ◽  
Genome Wide ◽  
Clinical Radiology ◽  
History Of

The history of ankylosing spondylitis (AS) dates back to the discovery of skeletons with characteristic spinal changes. The disease was further defined by correlating pathological and clinical features, and the development of clinical radiology. Subsequent epidemiology and familial studies highlighted the association with other related conditions as part of the spondyloarthritides. The discovery of HLA-B27 confirmed this association. Over the past two decades, genome-wide association studies, and advances in imaging and immunology have yielded dramatic insights into the disease and the development of highly effective therapies.

P022 Genetic background predicts the extra-articular involvement in patients with enthesitis related arthritis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Hanene Ferjani ◽  
Cherif Ines ◽  
Kaouther Maatallah ◽  
Wafa Triki ◽  
Dorra Ben Nessib ◽  
...  
Keyword(s):  
Ankylosing Spondylitis ◽  
Family History ◽  
Disease Activity ◽  
Genetic Background ◽  
Morning Stiffness ◽  
Inflammatory Diseases ◽  
Hla B27 ◽  
Familial History ◽  
Enthesitis Related Arthritis ◽  
History Of

Abstract Background Enthesitis-related arthritis (ERA) represents 20% of all juvenile idiopathic arthritis subtype. Among the genetic risk factors for the development of ERA, HLA-B27 has been implicated as a major contributor. The frequency of HLA-B27 varies among population. HLA-B 27 status in ERA may influence the clinical phenotype and prognosis of the disease. The main objective of this study is to determine whether genetic background including HLA B27 and familial history of spondylarthritits (SpA) may influence the clinical features of ERA patients. Methods We conducted a retrospective study including patients with ERA, all fulfilling the International League of Associations for Rheumatology (ILAR) criteria. For all patients, we collected the following data: Age, family history of rheumatic inflammatory diseases, inflammatory bowel diseases (IBD), the presence of HLA-B27 antigen, the inflammatory biomarkers: Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP), the disease activity assessed by morning stiffness, night awakenings, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and the current treatment. We assessed, as well, the functional impact using the Bath Ankylosing Spondylitis Functional Index (BASFI) the Lequesne index. The population was divided into two groups: a group including patients positive to HLA-B27 antigen and/or with family history of rheumatic inflammatory diseases, psoriasis or inflammatorybowel diseases. The second group was defined as control group with patients negative to HLA-B27 antigen and without any family history of the diseases above. Results We included 40 ERA patients, mean age of onset 12,43 ± 3,003 years (6–16). The majority of them were male (n = 34). Twenty-eight patients had a genetic background. Among them, 7,5% of patients had a positive family history, 42,5% were positive to HLA-B27 antigen and 20% of them met both criteria. As shown in table 1, clinical manifestations were similar between the 2 groups. Enthesitis was more frequent in patients with HLAB27 without a significant difference. Regarding the disease activity, the number of night awakenings and the morning stiffness duration were comparable in the two groups. Six patients had a BASDAI score &gt; 4 with no difference between the two groups. Extra-articular manifestations were present in 15 patients. Among them 14 had a genetic background, reaching the significance threshold with P = 0,013. We counted 8 cases of uveitis, one case of IBD, 5 cases of lung disease and 1 case of cardiac involvement. Inflammatory markers were higher in the group with familial history of SpA and/or positive HLAB27. Indeed, the mean ESR value was 42,73 vs 29.9, P = 0,01. There were no correlations between BASFI score and a positive genetic background (P = 0,283). Only one patient was put on biologics. He has no family history and is negative to HLA-B27 antigen. Conclusion The frequency of HLAB27 was in line with the literature data. The genetic background did not influence the disease activity or the functional impairment in our population. However, a positive correlation was found between a positive familial history of SpA, HLAB27, and the presence of extra-articular manifestations as well as with a higher ESR value.

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