The Prevalence of MRI-Defined Sacroiliitis and Classification of Spondyloarthritis in Patients with Acute Anterior Uveitis: A Longitudinal Single-Centre Cohort Study

Background: Acute anterior uveitis (AAU) is a relatively common extra-musculoskeletal manifestation of axial spondyloarthritis (axSpA); however, data on the prevalence of active sacroiliitis in patients with AAU are limited. Methods: 102 patients with AAU and 39 healthy subjects (HS) underwent clinical assessment and sacroiliac joint MRI. Patients with absence of active sacroiliitis were reassessed after two years. International Spondyloarthritis Society (ASAS) classification criteria for axSpA (regardless of patient’s age) and expert opinion for definitive diagnosis of axSpA were applied. Results: Although chronic back pain was equally present in both groups, bone marrow edema (BME) in SIJ and BME highly suggestive of axSpA was found in 52 (51%) and in 33 (32%) patients with AAU compared with 11 (28%) and none in HS, respectively. Out of all AAU patients, 41 (40%) patients fulfilled the ASAS classification criteria for axSpA, and 29 (28%) patients were considered highly suggestive of axSpA based on clinical features. Two out of the 55 sacroiliitis-negative patients developed active sacroiliitis at the two-year follow-up. Conclusions: One-third of patients with AAU had active inflammation on SIJ MRI and clinical diagnosis of axSpA. Therefore, patients with AAU, especially those with chronic back pain, should be referred to a rheumatologist, and the examination should be repeated if a new feature of SpA appears.

Pitfalls of the Semi-Quantitative Analyzing 99mTc-Pyrophosphate Planar Images for Diagnosing Transthyretin Cardiac Amyloidosis: A Possible Solution

Background: Two different approaches, 1-h heart-to-contralateral (H/CL) ratio and 3-h visual grading scale relative to ribs (VGSr), have been established to interpret 99mTc-PYP planar images for the detection of amyloid transthyretin cardiac amyloidosis (ATTR-CA). Since they are prone to pitfalls, this pilot study aimed to explore the diagnostic practicality of the 3-h visual grading scale relative to the upper segment of sternum (VGSs) approach for interpreting 99mTc-PYP planar images. Methods: A total of 42 patients were enrolled in this retrospective study. SPECT/CT approach and planar approaches including H/CL ratio, VGSr, and VGSs were utilized to interpret the 99mTc-PYP images obtained at both 1 and 3 h. The classification criteria of the latest expert consensus recommendations were considered as the gold standard. The concordance between the interpretation of each approach and the gold standard was investigated. Results: In addition to 1- and 3-h SPECT/CT approaches, the interpretation of planar images using the 3-h VGSs approach was also applicable, which turns identical to the gold standard (κ = 1.000; p < 0.001). Conclusions: For the interpretation of 99mTc-PYP planar images, the 3-h VGSs approach should be the optimal method, particularly in the case without available or feasible tomography imaging. Only one imaging session (planar and SPECT/CT) at 3 h would be sufficient for the detection of ATTR-CA, and favorable for patient satisfaction.

Study on Effective Response Plans for Disaster Sites with Multiple Casualties

This study draws implications through a case analysis of disaster sites with multiple casualties. Based on this, through a survey targeting firefighters working at disaster sites, this study aimed to present an effective response improvement plan at disaster sites with multiple casualties. First, it is necessary to strengthen the operational capability of the emergency medical center of the first responders. Second, an easy and convenient triage pocket book should be produced and distributed to strengthen the triage capability of all firefighters, including paramedics. Third, it is necessary to introduce the multiple casualty e-triage system for casualty management. Fourth, the communication system for prompt on-site dispatch at public health centers and DMAT should be improved. Lastly, it is necessary to re-establish the severity classification criteria for cardiac arrest patients at disaster sites.

Cutaneous manifestations of systemic lupus erythematosus: experience from a tertiary care hospital of Bangladesh

Background: Systemic lupus erythematosus (SLE) is a chronic, multisystem disorder that can affect any organ of the body. Approximately 80 percent of patients develop skin disease at some point in their disease course. The association with SLE varies among the subtypes of cutaneous lupus erythematosus (LE). Better understanding of cutaneous manifestations can help in more effective management. This study aimed to evaluate the pattern of cutaneous manifestations of SLE and to find out association with organ involvement. Methods: This cross-sectional observational study was conducted in the Green Life Medical College Hospital from January 2019 to December 2020. Sixty four lupus patients who fulfilled the SLICC 2012 classification criteria and having new onset or preexisting skin complaints were enrolled. Mixed connective tissue disease and other overlap syndromes were excluded. All patients were evaluated by a dermatologist and diagnosis was done as per modified Gilliam Classification criteria. Results: Out of 64 patients, 56 were female and 8 were male. Female and male ratio was 7:1. Mean age was 28.4±9.6 years. Among the cutaneous manifestations, LE specific was 38 (59.4%), LE non-specific was 41 (64.1%). Among LE specific, 66% were acute (ACLE), 42% were sub-acute (SCLE) and 37% patients were chronic (CCLE). Among ACLE, 72% had malar rash and 84% had photosensitivity. Among SCLE, most common was papulosqumous (68%). DLE (86%) was the most common CCLE. Among LE non-specific, 85% had non-scarring alopecia, 52% had vascular abnormalities. Most common organ involvement was musculoskeletal (84%), then renal (56%). DLE had negative association with renal involvement [OR (-0.04)]. No other cutaneous manifestations showed any significant association with any other organ involvement. Conclusion: Cutaneous manifestations are important feature in SLE. LE non-specific was more common than LE specific manifestations in this study. Better understanding can help in efficient diagnosis and management. BIRDEM Med J 2022; 12(1): 57-61

Interstitial pneumonia with autoimmune features: challenges and controversies

The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial pneumonia with autoimmune features (IPAF) research entity to capture such patients in a standardised manner, with the intention of nurturing clinical research. This initiative resulted in the publication of several series of IPAF patients, with significant variation between cohorts in clinical characteristics, outcome and the application of IPAF criteria in patient selection. From this increasing body of published work, it has become apparent that revision of IPAF criteria is now required in order to justify the eventual designation of IPAF as a standalone diagnostic term, as opposed to a provisional entity put forward as a basis for clinical research. This review covers the current state of IPAF, conclusions that can and cannot be drawn from the IPAF evidence base, and ongoing uncertainties that require further expert group consideration.

Recommendations for the Diagnosis and Treatment of Multiple Sclerosis Relapses

Minimizing the risk of relapse is essential in multiple sclerosis (MS). As none of the treatments currently available are capable of completely preventing relapses, treatment of these episodes remains a cornerstone of MS care. The objective of this manuscript is to reduce uncertainty and improve quality of care of this neurological process. This article addresses definitions of key concepts, recommendations for clinical examination, classification criteria, magnetic resonance imaging, biomarkers, and specific therapeutic counsels including special populations such as pregnant and breastfeeding women, and children. An algorithm for treating MS relapses is also provided.

Clinical Characteristics and Therapeutic Response of Immunoglobulin G4-Related Disease: a Retrospective Study of 127 Chinese Patients

Background & Aims: Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. The aim of the present study was to characterize the clinical features and therapeutic response of patients with IgG4-RD and identify risk factors for disease relapse.Methods: We collected baseline data of eligible patients with IgG4-RD and analyzed clinical feature by interview and review of medical records. The patients who received medical therapy with at least 3 months follow-up were used to characterize the therapeutic response and identify risk factors for relapse. Result: Totally 127 IgG4-RD patients, including 92 males and 35 females, were included in the present study. The median age at diagnosis was 63.0 years, ranging from 23 to 86. The pancreas, bile duct and lymph nodes were the most frequently involved organs. The serum IgG4 level was elevated in 94.5% of the patients and was correlated with the number of organs involved. Patients classified as head and neck limited group were more likely to be female. Patients classified as pancreato-hepatobiliary group had highest aminotransferase, alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (GGT), bilirubin level (all p＜0.05). Mikulicz syndrome and systemic involvement group had the highest IgG-RI score, serum globulin, IgG and IgG4 level. Among 92 patients who received medical therapy with at least 3 months follow-up,76 received glucocorticoids (GC) alone or in combination with immunosuppressive agents and 16 patients did not take GC. 74 out of the 76 patients (97.3%) achieved remission, with 59 of them remained in remission and 15 of them relapsed. Wheras16 patients did not take GC, among them, 6 patients achieved remission with one patient relapsed. On multivariate analysis, higher initial score of ACR/EULAR IgG4-RD Classification Criteria, GC monotherapy and GC withdrawal were independent predictors for relapse.Conclusion: Different phenotypes of IgG4-RD showed different demographic and serological features. The risk factors of relapse were GC withdrawal, GC monotherapy and higher score of ACR/EULAR IgG4-RD Classification Criteria.