Concomitant Rheumatic Diseases Research Articles

Prevalence of concomitant rheumatologic diseases and autoantibody specificities among racial and ethnic groups in SLE patients.

Leveraging the Manhattan Lupus Surveillance Program (MLSP), a population-based registry of cases of systemic lupus erythematosus (SLE) and related diseases, we investigated the proportion of SLE with concomitant rheumatic diseases, including Sjögren's disease (SjD), antiphospholipid syndrome (APLS), and fibromyalgia (FM), as well as the prevalence of autoantibodies in SLE by sex and race/ethnicity. Prevalent SLE cases fulfilled one of three sets of classification criteria. Additional rheumatic diseases were defined using modified criteria based on data available in the MLSP: SjD (anti-SSA/Ro positive and evidence of keratoconjunctivitis sicca and/or xerostomia), APLS (antiphospholipid antibody positive and evidence of a blood clot), and FM (diagnosis in the chart). 1,342 patients fulfilled SLE classification criteria. Of these, SjD was identified in 147 (11.0%, 95% CI 9.2-12.7%) patients with women and non-Latino Asian patients being the most highly represented. APLS was diagnosed in 119 (8.9%, 95% CI 7.3-10.5%) patients with the highest frequency in Latino patients. FM was present in 120 (8.9%, 95% CI 7.3-10.5) patients with non-Latino White and Latino patients having the highest frequency. Anti-dsDNA antibodies were most prevalent in non-Latino Asian, Black, and Latino patients while anti-Sm antibodies showed the highest proportion in non-Latino Black and Asian patients. Anti-SSA/Ro and anti-SSB/La antibodies were most prevalent in non-Latino Asian patients and least prevalent in non-Latino White patients. Men were more likely to be anti-Sm positive. Data from the MLSP revealed differences among patients classified as SLE in the prevalence of concomitant rheumatic diseases and autoantibody profiles by sex and race/ethnicity underscoring comorbidities associated with SLE.

Interstitial Lung Disease in Primary Biliary Cholangitis: A Cohort Prospective Study.

Interstitial lung disease (ILD) has been recognized as an extrahepatic manifestation ofprimary biliary cholangitis (PBC), althoughlimited data are available on its prevalence and clinical significance. Therefore, we evaluated the occurrence and clinical features of ILD in a cohort of PBC patients. Ninety-three individuals without concomitant rheumatic diseases were enrolled in our prospective cohort study. All patients underwent chest high-resolution computed tomography (HRCT). Liver-related and lung-related survival wereassessed. A lung-related outcome was defined as death from ILD complications; a liver-related outcome was defined as liver transplantation or death from liver cirrhosis complications. HRCT findings suggestive ofILD were detected in 38 patients (40.9%). A sarcoid-like pattern of PBC-associated ILD was the most frequent, followed by subclinical ILD and organizing pneumonia. Patients with ILD were less likely to have liver cirrhosis and liver-related symptoms and presented with higher serum immunoglobulin M(IgM) and M2 subtype antimitochondrial antibodies (AMA-M2) positivity rates. In a multivariate analysis, the absence of liver disease symptoms at the disease presentation (OR 11.509; 95% CI 1.210-109.421; p = 0.033), the presence of hepatic non-necrotizing epithelioid cell granulomas (OR 17.754; 95% CI 1.805-174.631; p = 0.014), higher serum IgM (OR 1.535; 95% CI 1.067-2.208; p = 0.020) and higher blood leukocyte count (OR 2.356; 95% CI 1.170-4.747; p = 0.016) were independent risk factors associated with ILD in PBC. More than a third of patients with ILD showed no respiratory symptoms, and only one ILD-related death occurred during a follow-up of 29.0 months (IQR 11.5; 38.0). Patients with ILD had better liver transplant-free survival.ILD in PBC had a benign course and was associated with a lower liver disease severity. PBC-associated ILD should be included in a list of differential diagnoses of ILD.

Frequency of rheumatic diseases in patients with familial Mediterranean fever

Purpose: Mutations in the Mediterranean FeVer (MEFV) gene, which causes familial Mediterranean fever (FMF), may also cause the emergence of other specific rheumatic diseases. This study aims to determine the frequency of other rheumatologic diseases in paediatric FMF patients, evaluate whether there are clinical and genetic differences between those with and without concomitant rheumatologic diseases, and compare the data with previous studies.
 Materials and methods: The files of FMF patients who were followed up at the paediatric rheumatology department were reviewed retrospectively. Demographic data, MEFV mutations, treatment, disease severity scores, and concomitant rheumatic diseases were recorded from the files.
 Results: There were 303 FMF patients (154 female/149 male). The mean age at diagnosis was 7.04±3.9 years. The mean disease duration was 5.33±3.13 years. In the cohort, 41 FMF patients (13.5%) were diagnosed with another rheumatic disease. There were 22 cases of juvenile idiopathic arthritis (53.6%), seven cases of vasculitis (17%), six cases of periodic fever aphthous stomatitis and adenitis syndrome (14.6%), three cases of Behçet disease (7.3%), two cases of acute rheumatic fever (4.8%), and one case of systemic lupus erythematosus (2.4%). 32 of these 41 FMF patients (78%) had the M694V mutation (homozygous in 11, heterozygous in 21). Disease activity scores PRAS and ISSF scores were higher in FMF with rheumatic diseases (p=0.002 and p

The use of autologous platelet-rich fibrin matrix combined with meniscal repair in the treatment of parameniscal cyst: clinical results and cyst recurrence after 2-year of follow up

PurposeParameniscal cysts are associate with horizontal meniscal tears. Arthroscopic meniscal repair and the excision of the cyst by mini-open approach represent a valid treatment. However, the recurrence of cyst is still a current issue. Therefore, biological factors may be considered to promote the biological repair and avoid recurrence. The aim of the present study was to report the clinical results and the rate of recurrence of the cyst after minimum 2-year of follow up in a cohort of patients treated by meniscal repair and autologous platelet-rich fibrin matrix augment.MethodsPatients with lateral parameniscal cyst undergoing arthroscopic meniscal repair and autologous platelet-rich fibrin matrix augment between 2016 and 2019 were retrospectively reviewed in March 2021. Inclusion criteria were absence of prior surgery on the affected knee with minimum 2-year of follow-up. Exclusion criteria were concomitant ligament lesions, rheumatic diseases and knee osteoarthritis. After reviewing the database, each selected patient was contacted and asked to participate in the study; at the follow-up evaluation all patient signed an informed consent. Tegner-Lysholm knee score, IKDC and NRS were collected before surgery and at follow-up.ResultsThis study included 15 patients (8 male) with mean age of 32.8 years old. No recurrence of the cysts was observed. The Tegner-Lysholm knee score and IKDC subjective scores increased respectively from 41.3 ± 5.4 and 37.6 ± 5.1 at baseline to 92.3 ± 4.6 and 89.4 ± 2.6 at the final follow up. Concerning pain relief, the Numeric Pain Rating Scale (NRS) displayed a significant improvement reaching at the follow up a score of 1,3 ± 1.1 in comparison to 6.8 ± 0.9 at the baseline.ConclusionSurgical management of symptomatic lateral parameniscal cyst with cyst excision, autologous PRP membrane application and meniscus repair demonstrated excellent subjective clinical outcome with any cyst reoccurrence.Level of evidenceIII, retrospective cohort study.

97P Comparative serum metabolome analysis as a diagnostic tool in concomitant rheumatic and malignant diseases

Rheumatic and musculoskeletal diseases (RMDs) and malignancies are both caused by a dysfunctional immune system. Research on this immunological intersection offers the opportunity for better patient centred care and scientific progress. Herein, a diagnostic classification model based on changes in serum metabolome was explored for its suitability for early diagnosis of a concomitant malignancy in RMD patients.

Rheumatologic diseases impact the risk of progression of MGUS to overt multiple myeloma

Monoclonal gammopathy of undetermined significance (MGUS), a premalignant condition, is associated with various chronic inflammatory rheumatic diseases (RDs) and is frequently observed as an incidental finding during routine work-up. The association of MGUS and chronic RDs is well established, but the impact of RDs on the risk of transformation into overt multiple myeloma (MM) has not been evaluated so far. MGUS patients diagnosed between January 2000 and August 2016 were identified and screened for concomitant RDs. RDs were grouped into antibody (Ab)-mediated RDs and non-Ab–mediated RDs (polymyalgia rheumatica, large-vessel giant cell arteritis, spondyloarthritis, and gout). Progression to MM was defined as a categorical (yes/no) or continuous time-dependent (time to progression) variable. Of 2935 MGUS patients, 255 (9%) had a concomitant RD. MGUS patients diagnosed with non-Ab–mediated RDs had a doubled risk of progression compared with those without a concomitant RD (hazard ratio, 2.1; 95% CI, 1.1-3.9; P = .02). These data translate into a 5-year risk of progression of 4% in MGUS patients without rheumatologic comorbidity, 10% in those with concomitant non-Ab–mediated RDS, and 2% in those with Ab-mediated RDs. By using the complex risk stratification model that includes myeloma protein (M-protein) concentration, immunoglobulin type, and level of free light chain ratio as variables, patients with non-Ab–mediated RDs (n = 57) had the highest risk for progression (hazard ratio, 6.8; 95% CI, 1.5-30.7; P = .01) compared with patients with Ab-mediated RDs (n = 77). Chronic inflammatory diseases have an impact on the risk of MGUS progressing into overt MM, with a doubled risk of transformation observed in patients with non-Ab–mediated RDs. Future research can elucidate whether comorbidities such as RDs should be included in currently applied prognostic MGUS scores.

Basic immunology may lead to translational therapeutic rationale: SARS-CoV-2 and rheumatic diseases.

COVID‐19 pandemia is a major concern for patients and healthcare systems. The fear of infection by patients with concomitant rheumatic diseases (either adult or children) and connective tissue diseases is arising worldwide, because of their immunological background and immunological therapies. Analysing the basic biology of single diseases, the data suggest that there is an "immunological umbrella" that seems to protect against the infection, through IFN type 1 and NK cell function. To date, reports from China, United States and Europe did not reveal an higher risk of infection, either for rheumatoid arthritis, juvenile idiopathic arthritis nor for lupus erythematosus. Antimalarials, anti‐IL6‐Anti‐IL6 receptor, anti‐IL1, anti‐GM‐CSF receptor and JAK1/2/3 inhibitors, are under investigation in COVID‐dedicated clinical trials to control the inflammation raised by SARS‐CoV‐2 infection. Initial reports on the occurrence of autoimmune phenomena in the convalescence phase of SARS‐CoV‐2 infection suggests that the immunological consequences of the infection need to be strictly understood. Reporting of the study conforms to broad EQUATOR guidelines (Simera et al January 2010 issue of EJCI).

Minimally invasive surgical treatment of patients with temporomandibular joint disorders and concomitant rheumatic diseases

Background: Nowadays there is no common strategy for TMD rheumatoid patients.

Familial Mediterranean fever is no longer a rare disease in Japan.

BackgroundThe aim of this study was to evaluate the clinical manifestations and prevalence of familial Mediterranean fever (FMF) in Japanese patients with unexplained fever and rheumatic manifestations.MethodsWe enrolled 601 patients with unexplained fever or suspected FMF throughout Japan between 2009 and 2015. Patients were divided into three groups according to Tel Hashomer criteria: sure FMF, probable FMF, and non-FMF patients, including definitive rheumatic diseases. Mutation detection in exons 1, 2, 3, and 10 of the FMF gene MEFV was performed by direct sequencing.ResultsA total of 192 patients (31.9 %) were diagnosed with FMF according to FMF diagnostic criteria. These could be divided into sure FMF (56.3 %, n = 108) and probable FMF (43.7 %, n = 84) patients. Fever, abdominal symptoms, and thoracic symptoms were significantly more common in FMF than non-FMF patients. Among FMF patients, 26 (13.5 %) had concomitant rheumatic diseases. Most FMF patients (94.3 %, 181/192) carried at least one MEFV mutation. Allele frequencies of M694I (13.5 % vs 0 %) and E148Q (39.1 % vs 24.8 %) mutations were significantly higher in FMF compared with healthy subjects. Allele frequencies of common MEFV mutations in FMF patients were M694I (13.5 %), P369S (8.6 %), R408Q (8.1 %), G304R (2.9 %), R202Q (4.4 %), E148Q (39.1 %), L110P (11.7 %), and E84K (3.1 %). Patients with a sure FMF phenotype had a higher frequency of MEFV exon 10 mutation (M694I) and a lower frequency of MEFV exon 3 mutations (P369S, R408Q) compared with those with a probable FMF phenotype.ConclusionThe high prevalence of FMF in Japanese patients with unexplained fever was confirmed in the present study. FMF should be suspected in cases of unexplained fever or non-specific rheumatic manifestations, and mutational analysis of MEFV could be useful to predict the clinical phenotypes of FMF in Japan.

Systemic staging for urate crystal deposits with dual-energy CT and ultrasound in patients with suspected gout

Objective of the study is to compare the diagnostic accuracy for detecting monosodium urate crystal deposits between dual-energy CT (DECT) and ultrasound (US). Sixty consecutive patients (49 men, mean age 62 years) with clinically suspected gout were included in this case-control study. DECT and US of feet, knees, hands and elbows were performed in all patients. Polarisation microscopy of synovial fluid or a score incorporating serum uric acid level, first MTP joint involvement, gender, previous patient-reported arthritis attack, cardiovascular diseases, joint redness and onset within 1 day was used as standard of reference. Standard of reference classified 39 patients as gout positive. Sixteen patients had gout and a concomitant rheumatic disease. Sensitivities for diagnosis of gout disease were 84.6 % (33/39) for DECT and 100 % (39/39) for US. Specificities were 85.7 % (18/21) for DECT and 76.2 % (16/21) for US. Positive and negative predictive values were 91.7 % (33/36) and 75.0 % (18/24) for DECT, 88.6 % (39/44) and 100 % (16/16) for US, respectively. Urate crystals were detected most frequently in MTP1 joints (DECT 20/78, US 58/78), any other toe joints (DECT 25/78, US 62/78) and knees (DECT 41/78, US 31/78). The volumetry of DECT computed a mean urate crystal deposit load of 2.1 cm(3) (SD 9.6 cm(3)). A mean effective dose of ≤0.5 mSv was estimated. DECT is more specific for the diagnosis of gout than US. However, it fails to detect small urate crystal deposits. It might be particularly useful for patients with ambivalent findings, concomitant rheumatic diseases and with non-conclusive joint aspiration.

THU0243 Standardized mortality ratios and predictors of survival in 215 southern chinese patients with inflammatory myopathies

ObjectivesTo examine the standardized mortality ratios (SMRs) and predictive factors for malignancy in a cohort of southern Chinese patients with inflammatory myopathies (IM).MethodsAll patients who were diagnosed to have polymyositis...

Use of etanercept in human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) patients

Etanercept (Enbrel, Amgen, Thousand Oaks, CA), a soluble p75 tumor necrosis factor receptor:FC (TNFR:FC) fusion protein for plasma cytokines, specifically tumor necrosis factor-alpha (TNF-alpha), is used in the treatment of immune-mediated rheumatic diseases. To our knowledge, the use of etanercept in patients with human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) is relatively uncommon. The main purpose of this short review is to examine the safety of etanercept in patients with HIV/AIDS. A Medline search was conducted using the keywords etanercept and HIV and/or AIDS for any published articles between 1966 to the present (September 2004). A case report, one case series, and one clinical trial pertained to the use of etanercept in HIV patients. No reports were found on the use of etanercept in AIDS. In addition, two case reports were found documenting the use of infliximab in HIV patients. Preliminary reports indicate that the administration of etanercept does not appear to increase the morbidity or mortality rates in HIV. The inhibition of TNF-alpha may actually improve the symptoms of HIV/AIDS-associated aphthous ulcers, cachexia, dementia, fatigue, and fever, as well as help manage concomitant rheumatic diseases and psoriasis. The use of etanercept shows promise for applications in disease management in patients with HIV/AIDS. Continued research efforts are necessary to establish the long-term safety and efficacy of etanercept and other biologic agents in this patient population.