American College Of Rheumatology Classification Research Articles

The performance of the 2022 ACR/EULAR classification criteria for Takayasu's arteritis as compared to the 1990 ACR classification criteria in a Chinese population.

In this study, we studied the performance of the 2022 American College of Rheumatology (ACR)/ European Alliance of Associations for Rheumatology (EULAR) classification criteria for Takayasu's arteritis (TAK) as compared to the 1990 ACR classification criteria in a Chinese population. The sensitivity, specificity, positive predictive value, negative predictive value, accuracy and the area under the receiver operating characteristics curve (AUC) of the above two criteria were compared. The sensitivity (92.6%), positive predictive value (95.6%), negative predictive value (94.6%), accuracy (95.0%) and AUC (0.981) of the 2022 criteria were superior to those of the 1990 criteria (45.7%, 91.5%, 70.5%, 75.0% and 0.874, respectively), and the difference of AUC was statistically significant (Z = 5.362, P < 0.001). In addition, we included new imaging modalities in the 1990 criteria, whose sensitivity, positive predictive value, negative predictive value, accuracy and AUC were significantly improved, but still lower than those of the 2022 criteria, the difference in AUC was also statistically significant (Z = 2.023, P = 0.043). The 2022 criteria for TAK exhibited superior performance compared with the 1990 criteria and may be more appropriate for the Chinese population. Incorporating additional imaging modalities could enhance the classification performance of the 1990 criteria even further.

Patients with clinically diagnosed hand OA not fulfilling the ACR classification criteria are in an earlier disease phase and more often have thumb base OA

ObjectiveTo investigate the performance of the American College of Rheumatology (ACR) classification criteria for hand osteoarthritis. DesignLongitudinal data up to four years from a cohort of consecutive patients with primary hand osteoarthritis diagnosed by their rheumatologist (Hostas study) were used to classify presence or absence of hand osteoarthritis according to the 1990 ACR criteria (traditional format: one major and 4 minor ACR criteria) (ACR+/ACR−). Demographics, Australian/Canadian osteoarthritis hand index (AUSCAN) pain and function were obtained. Hand radiographs were scored according to Kellgren-Lawrence; radiographic osteoarthritis was defined as Kellgren-Lawrence ≥2 in ≥1 CMC1 joint or ≥2 DIP/PIP/MCP joints. ResultsOf 538 patients (mean age 61 years, 86.1% women) 485 (90.1%) fulfilled ACR criteria at baseline. Except for the minor criterion swelling of <3 MCP joints, all criteria differed between the groups. ACR− patients were younger, with higher BMI, a shorter time since diagnosis, and less bony enlargements, joint deformities and radiographic osteoarthritis, except for radiographic CMC1 osteoarthritis which was seen more often in ACR− patients. No difference in AUSCAN pain or function was seen between ACR− versus ACR+ patients. After follow-up 37/53 (69.8%) converted to ACR+, 2/53 (3.8%) did not, and 14/53 (26.4%) were lost to follow-up. ConclusionsIn clinical practice the majority of patients fulfill the ACR classification criteria, but those in an earlier disease phase, with less signs of hand osteoarthritis or with primarily thumb base osteoarthritis are less likely to fulfill them. New classification criteria also including earlier disease stages and with attention for hand osteoarthritis subtypes are required.

Clinical subgroup clustering analysis in a systemic lupus erythematosus cohort from Western Pennsylvania.

Systemic lupus erythematosus (SLE) is a complex heterogenous autoimmune disease that can affect multiple organs. We performed clinical clustering analysis to describe a lupus cohort from the University of Pittsburgh Medical Center. A total of 724 patients who met the American College of Rheumatology (ACR) classification criteria for SLE were included in this study. Clustering was performed using the ACR classification criteria and the partitioning around medoid method. Correlation analysis was performed using the Spearman's Rho test. Patients with SLE in our cohort identify three district clinical disease subsets. Patients in cluster 1 were significantly more likely to develop renal and hematologic involvement, and had overrepresentation in African-American and male lupus patients. Clusters 2 and 3 identified a milder disease, with a significantly less likelihood of organ complications. Patients in cluster 2 are characterized by malar rash and photosensitivity, while patients in cluster 3 are characterized by oral ulcers, which is present in ~90% of patients within this cluster. The presence of photosensitivity or oral ulcers appears to be protective against the development of lupus nephritis in our cohort. We describe a large cohort of SLE from Western Pennsylvania and identify three distinct clinical disease subgroups. Clustering analysis might help to better manage and predict disease complications in heterogenous diseases like lupus.

Biomechanical Properties of Heel Pad, Metatarsal Head Soft-Tissue and Foot Ulcers in Patients with Systemic Sclerosis: A Case Control Study.

Background:Systemic sclerosis is a chronic disease of connective tissue accompanied by fibrosis of the skin and inner organs and an increased risk of foot ulcers. Biomechanical indices such as soft-tissue thickness and compressibility may correlate with the risk of this phenomenon.Objective:The aim of this study was to assess heel pad and first metatarsal head (MTH) soft-tissue thickness and compressibility index (CI) in scleroderma patients compared to matched healthy individuals. Not all patients had foot ulcers.Methods:Heel pad thickness in standing (loaded) and lying (unloaded) positions were measured in 40 scleroderma patients by means of a lateral foot radiograph. CI was measured as the ratio of loaded to unloaded thickness. The Soft-tissue thickness of the first MTH was measured by ultrasound. Results were compared with 40 healthy controls of matched age and body mass index. All patients’ diagnoses were made based on the American College of Rheumatology classification criteria.Results:Forty scleroderma patients (36 females, 4 males) with the following demographics were studied; mean age (SD) 45(12), mean body mass index 25.5 (4), and mean disease duration=10(9.6) years; only 8 (20%) had digital ulcers. Patients’ heel pad thickness and CI in the dominant side and MTH soft-tissue thickness on both sides were significantly different compared to the control group. Comparison of results in patients with and without foot ulcers also showed a significant difference in soft-tissue thickness. Thickness was negatively associated with disease duration, but the CI did not change over time.Conclusion:Soft-tissue thickness of the foot decreases in scleroderma patients and is associated with foot ulcers and digital ulcers in the hands.

Serum irisin level in rheumatoid arthritis patients: Relationship to disease activity, subclinical atherosclerosis, and cardiovascular risk factors

Aim of the workTo assess serum irisin level in rheumatoid arthritis (RA) patients, determine the relationship between irisin levels, disease activity and cardiovascular (CV) risk factors, and to evaluate its performance in predicting subclinical atherosclerosis and disease activity. Patients and methods60 RA patients and 30 controls were recruited for serological enzyme linked immunosorbant assay (ELISA) testing of irisin levels. Disease activity score (DAS28), health assessment questionnaire disability index (HAD-DI) and American college of rheumatology classification of functional status were assessed. Levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), glycated hemoglobin (HbA1c), insulin and lipid profile were measured. Homeostasis Model Assessment of Insulin Resistance (HOMA-IR) was calculated. Carotid intimal medial thickness (C-IMT) was measured and echocardiography performed. ResultsSerum irisin levels were significantly lower in patients (9.8 ± 10.6 ng/ml) vs. controls (20.5 ± 13.8 ng; p < 0.001). Irisin levels inversely correlated significantly with C-IMT, body mass index (BMI), HOMA-IR, disease activity and disability. Patients with CV involvement showed significantly lower serum irisin level, increased disease activity and disability. Classifying patients based on cut-off values of DAS28 into low, moderate, and high disease activity, a significant difference in irisin levels was found, being lowest among highly active patients. Irisin performed as an excellent independent indicator of subclinical atherosclerosis (p < 0.001) and high disease activity (p < 0.001) in RA patients. ConclusionIn RA, decreased irisin were significantly associated with increased CV risk and performed better than traditional yardsticks in identifying disease activity. It may act as an independent indicator of subclinical atherosclerosis within RA patients.

Imaging Tests in the Early Diagnosis of Giant Cell Arteritis.

Early recognition of giant cell arteritis (GCA) is crucial to avoid the development of ischemic vascular complications, such as blindness. The classic approach to making the diagnosis of GCA is based on a positive temporal artery biopsy, which is among the criteria proposed by the American College of Rheumatology (ACR) in 1990 to classify a patient as having GCA. However, imaging techniques, particularly ultrasound (US) of the temporal arteries, are increasingly being considered as an alternative for the diagnosis of GCA. Recent recommendations from the European League Against Rheumatism (EULAR) for the use of imaging techniques for large vessel vasculitis (LVV) included US as the first imaging option for the diagnosis of GCA. Furthermore, although the ACR classification criteria are useful in identifying patients with the classic cranial pattern of GCA, they are often inadequate in identifying GCA patients who have the extracranial phenotype of LVV. In this sense, the advent of other imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and positron emission tomography (PET)/CT, has made it possible to detect the presence of extracranial involvement of the LVV in patients with GCA presenting as refractory rheumatic polymyalgia without cranial ischemic manifestations. Imaging techniques have been the key elements in redefining the diagnostic work-up of GCA. US is currently considered the main imaging modality to improve the early diagnosis of GCA.

Prevalence of Systemic Lupus Erythematosus in the United States: Estimates From a Meta-Analysis of the Centers for Disease Control and Prevention National Lupus Registries.

Epidemiologic data on systemic lupus erythematosus (SLE) are limited, particularly for racial/ethnic subpopulations in the US. This meta-analysis leveraged data from the Centers for Disease Control and Prevention (CDC) National Lupus Registry network of population-based SLE registries to estimate the overall prevalence of SLE in the US. The CDC National Lupus Registry network includes 4 registries from unique states and a fifth registry from the Indian Health Service. All registries defined cases of SLE according to the American College of Rheumatology (ACR) 1997 revised classification criteria for SLE. Case findings spanned either 2002-2004 or 2007-2009. Given the heterogeneity across sites, a random-effects model was used to calculate the pooled prevalence of SLE. An estimate of the number of SLE cases in the US was generated by applying sex/race-stratified estimates to the 2018 US Census population. In total, 5,417 cases were identified as fulfilling the ACR SLE classification criteria. The pooled prevalence of SLE from the 4 state-specific registries was 72.8 per 100,000 person-years (95% confidence interval [95% CI] 65.3-81.0). The prevalence estimate was 9 times higher among females than among males (128.7 versus 14.6 per 100,000), and highest among Black females (230.9 per 100,000), followed by Hispanic females (120.7 per 100,000), White females (84.7 per 100,000), and Asian/Pacific Islander females (84.4 per 100,000). Among males, the prevalence of SLE was highest in Black males (26.7 per 100,000), followed by Hispanic males (18.0 per 100,000), Asian/Pacific Islander males (11.2 per 100,000), and White males (8.9 per 100,000). The American Indian/Alaska Native population had the highest race-specific SLE estimates, both among females (270.6 per 100,000) and among males (53.8 per 100,000). In 2018, an estimated 204,295 individuals (95% CI 160,902-261,725) in the US fulfilled the ACR classification criteria for SLE. A coordinated network of population-based SLE registries provides more accurate estimates of the prevalence of SLE and the numbers of individuals affected with SLE in the US in 2018.

The Challenge of Very Early Systemic Sclerosis: A Combination of Mild and Early Disease?

To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients. We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom. One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation. This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.

Complement Activation in Patients With Probable Systemic Lupus Erythematosus and Ability to Predict Progression to American College of Rheumatology-Classified Systemic Lupus Erythematosus.

ObjectiveTo evaluate the frequency of cell‐bound complement activation products (CB‐CAPs) as a marker of complement activation in patients with suspected systemic lupus erythematosus (SLE) and the usefulness of this biomarker as a predictor of the evolution of probable SLE into SLE as classified by the American College of Rheumatology (ACR) criteria.MethodsPatients in whom SLE was suspected by lupus experts and who fulfilled 3 ACR classification criteria for SLE (probable SLE) were enrolled, along with patients with established SLE as classified by both the ACR and the Systemic Lupus International Collaborating Clinics (SLICC) criteria, patients with primary Sjögren's syndrome (SS), and patients with other rheumatic diseases. Individual CB‐CAPs were measured by flow cytometry, and positivity rates were compared to those of commonly assessed biomarkers, including serum complement proteins (C3 and C4) and autoantibodies. The frequency of a positive multianalyte assay panel (MAP), which includes CB‐CAPs, was also evaluated. Probable SLE cases were followed up prospectively.ResultsThe 92 patients with probable SLE were diagnosed more recently than the 53 patients with established SLE, and their use of antirheumatic medications was lower. At the enrollment visit, more patients with probable SLE were positive for CB‐CAPs (28%) or MAP (40%) than had low complement levels (9%) (P = 0.0001 for each). In probable SLE, MAP scores of >0.8 at enrollment predicted fulfillment of a fourth ACR criterion within 18 months (hazard ratio 3.11, P < 0.01).ConclusionComplement activation occurs in some patients with probable SLE and can be detected with higher frequency by evaluating CB‐CAPs and MAP than by assessing traditional serum complement protein levels. A MAP score above 0.8 predicts transition to classifiable SLE according to ACR criteria.

Clinical and laboratory manifestations of systemic lupus erythematosus in patients with neuropsychiatric disorders (eurasian renaissance register)

Objective : to investigate the clinical and laboratory manifestations of systemic lupus erythematosus (SLE) with nervous system lesions in a Kyrgyz cohort. Subjects and methods . The prospective study enrolled 460 patients with SLE who fulfilled American College of Rheumatology (ACR) 1987 and SLICC 2012 criteria, and had been followed up at Academician M. Mirrahimov National Center for Cardiology and Therapy, from January 2012 to December 2017 according to the Eurasian RENAISSANCE Register program. The 1999 ACR classification criteria were used to evaluate the neuropsychiatric manifestations of SLE (NPSLE). A psychiatrist diagnosed neuropsychiatric disorders according to the ICD-10. A psychologist identified cognitive impairment, by using the specific Mini-Mental State Examination (MMSE) (a minischeme for examining a patient's mental status). Results and discussion . Various NPSLEs were detected in 103 (22.39%) of the 460 patients. According to the 1999 ACR criteria, 103 patients were diagnosed as having 155 different NPSLEs, including 123 (79.35%) patients who had central nervous system (CNS) disorders and 32 (20.65%) with peripheral nervous system (PNS) damages. There were 76 (61.79%) focal 47 (38.21%) diffuse CNS disorders. Most patients with diffuse NPSLEs were observed to have psychosis, the main manifestations of which were visual and auditory hallucinations (72.34%). The patients with focal NPSLEs had cerebrovascular disease (43.42%) with increase of level of antinuclear antibodies, antibodies against double-stranded DNA and hypocomplementemia (95.56, 86.52, and 73.85%, respectively). Patients with CNS lesions were significantly more likely than those with PNS to have lupus nephritis and hematological disorders (leukopenia, lymphopenia, and thrombocytopenia), as well as high immunological activity. Conclusion . Most patients with diffuse NPSLEs were observed to have psychosis with visual and auditory hallucinations (72.34%), and those with focal NPSLEs had cerebrovascular disease (43.42%) with a high frequency of immunological disorders. Lupus nephritis and hematological disorders with high immunological activity were significantly more common in patients with CNS lesions than in those with PNS ones.

Acupuncture in the treatment of fibromyalgia: what has changed in the last 10 years?

In the last 10 years, fibromyalgia diagnosis has been transformed by the American College of Rheumatology (ACR) classification criteria. These changes have significantly impacted outcomes in treatment recommendations, including many non-pharmacological therapies, notably acupuncture. This review presents what has changed on acupuncture recommendations for the treatment of fibromyalgia after alterations performed by ACR classification criteria. A literature search was performed to identify published studies between January 01, 2008 and June 30, 2018. Published studies were identified using an initial search of PubMed database. We searched for trials published using the terms “fibromyalgia acupuncture”. We used only full-text articles available online and related to the objective of this review. The last ACR’s criteria [2010] have included the evaluation of other symptoms of fibromyalgia besides pain. New diagnostic and therapeutic technologies have also enabled the publishing of new trials, scientifically demonstrating the effectiveness of acupuncture in patients with the fibromyalgia syndrome. It is concluded that acupuncture is currently considered a scientifically recommended treatment for fibromyalgia.

Characteristics of dry eye in patients with pre-existing Sjögren's syndrome according to the revised 2016 ACR-EULAR classification criteria.

To compare the characteristics of dry eye (DE) patients who did and did not satisfy the 2016 American College of Rheumatology (ACR)-European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (SS) among patients with pre-existing SS diagnosed according to the 2012 ACR criteriaThis cross-sectional study evaluated 91 patients with pre-existing SS and 55 with non-SS DE. Patients with SS were divided into 2 groups according to whether they met the revised 2016 ACR-EULAR classification criteria for primary SS. Group 1 (n = 71) was comprised of patients who satisfied the revised 2016 criteria and group 2 (n = 20) was comprised of patients who did not satisfy the newly revised criteria. Group 3 consisted of 55 patients with non-SS DE. The ocular surface disease index (OSDI) score, tear break-up time (TBUT), Schirmer score, tear clearance rate (TCR), and corneal and conjunctival staining scores were evaluated and compared between the groups. Laboratory profiles, including antinuclear antibodies, rheumatoid factor levels, erythrocyte sedimentation rate, and C-reactive protein levels, and focus scores were analyzed.TBUT, Schirmer, and corneal/conjunctival staining scores were significantly worse in both groups of patients with SS (groups 1 and 2) than in those with non-SS DE (group 3). However, there were no significant differences between groups 1 and 2 in laboratory findings as well as in ocular surface findings, including OSDI, TBUT, Schirmer score, TCR, and corneal/conjunctival staining scores. The focus score, which shows the level of lymphocytic infiltration in the salivary glands, was higher in group 1 than in group.Of the patients with pre-existing SS who were diagnosed according to the 2012 ACR classification, patients who did not satisfy the 2016 ACR-EULAR classification criteria for primary SS showed similar ocular surface parameters and laboratory findings to patients who did meet the revised classification, except for focus score. There is no need to change the direction of treatment of DE in patients with pre-existing SS who did not meet the revised 2016 ACR-EULAR criteria.

Psychiatric Symptoms in Systemic Lupus Erythematosus: Diagnosis and Treatment

According to the American College of Rheumatology classification, lupus erythematosus has five psychiatric manifestations, including cognitive dysfunction, mood disorder, anxiety disorder, psychosis, and acute confusional state, which are frequently accompanied by other symptoms. Cognitive dysfunction is the most common psychiatric manifestation in lupus patients with a prevalence rate ranging from 20% to 80%. The expression of psychiatric manifestations has been considered to be associated with disease activity, side effects of medications, and/or psychosocial stresses from the chronicity of lupus, but this has not been fully understood. Appropriate management of psychiatric symptoms is essential as it affects treatment adherence and quality of life. This review aimed to facilitate understanding of psychiatric manifestations of lupus through literature review on the prevalence, clinical features, diagnosis, and treatments of each psychiatric symptom.

Brief Report: How Do Patients With Newly Diagnosed Systemic Lupus Erythematosus Present? A Multicenter Cohort of Early Systemic Lupus Erythematosus to Inform the Development of New Classification Criteria.

Systemic lupus erythematosus (SLE) presents with nonspecific signs and symptoms that are also found in other conditions. This study aimed to evaluate manifestations at disease onset and to compare early SLE manifestations to those of diseases mimicking SLE. Academic lupus centers in Asia, Europe, North America, and South America collected baseline data on patients who were referred to them during the previous 3 years for possible SLE and who had a symptom duration of <1 year. Clinical and serologic manifestations were compared between patients diagnosed as having SLE and those diagnosed as having SLE-mimicking conditions. Diagnostic performance of the 1997 American College of Rheumatology (ACR) SLE classification criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria was tested. Data were collected on 389 patients with early SLE and 227 patients with SLE-mimicking conditions. Unexplained fever was more common in early SLE than in SLE-mimicking conditions (34.5% versus 13.7%, respectively; P < 0.001). Features less common in early SLE included Raynaud's phenomenon (22.1% versus 48.5%; P < 0.001), sicca symptoms (4.4% versus 34.4%; P < 0.001), dysphagia (0.3% versus 6.2%; P < 0.001), and fatigue (28.3% versus 37.0%; P = 0.024). Anti-double-stranded DNA, anti-β2 -glycoprotein I antibodies, positive Coombs' test results, autoimmune hemolytic anemia, hypocomplementemia, and leukopenia were more common in early SLE than in SLE-mimicking conditions. Symptoms detailed in the ACR and SLICC classification criteria were significantly more frequent among those with early SLE. Fewer patients with early SLE were not identified as having early SLE with use of the SLICC criteria compared to the ACR criteria (16.5% versus 33.9%), but the ACR criteria demonstrated higher specificity than the SLICC criteria (91.6% versus 82.4%). In this multicenter cohort, clinical manifestations that could help to distinguish early SLE from SLE-mimicking conditions were identified. These findings may aid in earlier SLE diagnosis and provide information for ongoing initiatives to revise SLE classification criteria.

Performance of the 2016 ACR-EULAR classification criteria for primary Sjogren's syndrome in a Korean cohort.

This study compared the performance of the newly proposed 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) criteria to the 2002 American-European Consensus Group (AECG) and 2012 ACR classification criteria for primary Sjogren's syndrome (pSS) in well-characterized Korean patients. Patients with pSS from 12 university-affiliated hospitals in Korea were enrolled from October 2013 to January 2017. Clinical and laboratory data were reviewed. For the validation set, patients who underwent evaluation tests to rule out pSS at Seoul St. Mary's hospital from November 2016 to December 2017 were analyzed. Baseline registry data were available in 458 patients, and 328 patients had sufficient data to determine the fulfillment of each criteria set. All three sets of criteria were met by 307 patients (93.6%). The newly proposed 2016 ACR/EULAR criteria were met by 325 patients (99.1%). The 2002 AECG and 2012 ACR criteria were met by 325 (99.1%) and 310 patients (94.5%), respectively. In a validation cohort consisting of 161 patients with pSS-related symptoms/signs, the sensitivity and specificity of the 2016 ACR/EULAR criteria were 100% [95% confidence interval (CI), 96.11-100.00] and 81.8% [95% CI, 76.15-94.26], respectively. Agreement between the 2016 criteria and 2012 or 2002 criteria was high (Cohen's kappa 0.736 and 0.769, respectively). The newly proposed 2016 ACR/EULAR criteria were met by most patients diagnosed with pSS according to previous criteria and showed higher sensitivity and lower specificity compared with both previous criteria sets.

About a subcutaneous calcification

We report a case of 62-year-old Tunisian woman with a 10-year history of a CREST syndrome (systemic sclerosis meeting the criteria of the CREST syndrome of the 1980 American College of Rheumatology classification for raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia). Her daughter is treated in neurology for myasthenia gravis. Our patient presents a 5-month history of increasing inflammatory right thumb pain concomitant with the discovery of a subcutaneous hard mass (A). Radiographs of her thumb showed a sub-cutaneous calcification (as another part of the CREST syndrome criteria) (B). X-rays of hands found an acro-osteolysis (C). In the CREST syndrome, the calcific deposits can be subclinical. But, when symptomatic, it becomes painful, tender and an inflammatory reaction can occur facing the calcinosis.

Autoimmune Hepatitis: SLE Overlap Syndrome - An Uncommon Presentation

Autoimmune Hepatitis (AIH)/Lupoid hepatitis and Lupus Hepatitis are difficult to be differentiated due to overlapping clinical findings. Lupus hepatitis is a very rare entity, especially as an initial presentation for systemic lupus erythematous (SLE). A 24 year old African American female presented to our clinic for evaluation of abnormal liver enzymes. While investigating her etiology, patient presented to the emergency department with shortness of breath and left sided chest pain. Her chain pain was exacerbated with deep breathing and coughing. She was diagnosed with massive pericardial effusion and pericarditis. Complete laboratory findings were significant for hemolytic anemia, pancytopenia with lymphopenia, and presence of anti-Ds DNA meeting more than four criteria of American College of Rheumatology classification for SLE. She also presented with Alkaline Phosphatase to AST ratio of 3.2, gamma globulin of 5.5, positive ANA with ratio of 1:640, and negative viral hepatitis markers. Her scoring according to international autoimmune hepatitis group was 28 (definite diagnosis of AIH). Patient denied having skin lesion, photosensitivity or oral ulcers. A-SMA was negative. She was started on indomethacin and prednisone. Her symptoms improved and remained stable on follow up clinic visit. Hepatitis in patients with SLE although rare, has been reported. Differentiating Hepatitis due to SLE or as an independant autoimmune condition overlapping with SLE is required for accurate treatment. Patients who fulfil ACR Criteria for SLE and International Autoimmune Hepatitis group scoring for AIH can be considered as having AIH-SLE overlapping syndrome.

Validation of the Systemic Lupus International Collaborating Clinics classification criteria in a cohort of patients with full house glomerular deposits

In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) presented a new classification for systemic lupus erythematosus (SLE). In this classification, biopsy-confirmed lupus nephritis with positive antinuclear or anti-double-stranded DNA antibodies became a stand-alone criterion. Because of the unknown diagnostic performance among patients from nephrology clinics, we aimed to test the validity of the SLICC classification, compared with the American College of Rheumatology classification, in a cohort of patients whose renal biopsies would raise the clinicopathologic suspicion of lupus nephritis. All patients with a renal biopsy showing full house glomerular deposits and clinical follow-up in our center were included and reevaluated, after which clinicians and a pathologist reached a consensus on the reference-standard clinical diagnosis of SLE. The diagnostic performance and net reclassification improvement were assessed in 149 patients, 117 of whom had clinical SLE. Compared with the American College of Rheumatology classification, the SLICC classification had better sensitivity (100 vs. 94%); although, this was at the expense of specificity (91 vs. 100%; net reclassification improvement-0.03). Excluding the stand-alone renal criterion, the specificity of the SLICC classification reached 100%, with a significant net reclassification improvement of 0.06 compared with the American College of Rheumatology classification. The SLICC classification performed well in terms of diagnostic sensitivity among patients with full house glomerular deposits; whereas, the stand-alone renal criterion had no additional value and compromised the specificity. Thus, presumed patients with lupus nephritis in nephrology clinics reflect a distinct SLE disease spectrum warranting caution when applying SLE classification criteria.

Ultrasonography of major salivary glands compared with parotid and labial gland biopsy and classification criteria in patients with clinically suspected primary Sjögren’s syndrome

ObjectiveTo assess the validity of ultrasound of major salivary glands (sUS) compared with parotid and labial gland biopsies, sialometry, anti-SSA/Ro antibody status and classification criteria in patients clinically suspected with...

Comparison of performance of the 2016 ACR-EULAR classification criteria for primary Sjögren's syndrome with other sets of criteria in Japanese patients

ObjectivesTo compare the performance of the new 2016 American College of Rheumatology (ACR)-European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (SS) with 1999 revised Japanese Ministry of...