Standardization of interstitial lung disease assessment by ultrasound: results from a Delphi process and web-reliability exercise by the OMERACT ultrasound working group.

OBJECTIVES: Over the last years ultrasound has shown to be an important tool for evaluating lung involvement, including interstitial lung disease (ILD) a potentially severe systemic involvement in many rheumatic and musculoskeletal diseases (RMD). Despite the potential sensitivity of the technique the actual use is hampered by the lack of consensual definitions of elementary lesions to be assessed and of the scanning protocol to apply. Within the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group we aimed at developing consensus-based definitions for ultrasound detected ILD findings in RMDs and assessing their reliability in dynamic images. METHODS: Based on the results from a systematic literature review, several findings were identified for defining the presence of ILD by ultrasound (i.e., Am-lines, B-lines, pleural cysts and pleural line irregularity). Therefore, a Delphi survey was conducted among 23 experts in sonography to agree on which findings should be included and on their definitions. Subsequently, a web-reliability exercise was performed to test the reliability of the agreed definitions on video-clips, by using kappa statistics. RESULTS: After three rounds of Delphi an agreement >75 % was obtained to include and define B-lines and pleural line irregularity as elementary lesions to assess. The reliability in the web-based exercise, consisting of 80 video-clips (30 for pleural line irregularity, 50 for B-lines), showed moderate inter-reader reliability for both B-lines (kappa = 0.51) and pleural line irregularity (kappa = 0.58), while intra-reader reliability was good for both B-lines (kappa = 0.72) and pleural line irregularity (kappa = 0.75). CONCLUSION: Consensus-based ultrasound definitions for B-lines and pleural line irregularity were obtained, with moderate to good reliability to detect these lesions using video-clips. The next step will be testing the reliability in patients with ILD linked to RMDs and to propose a consensual and standardized protocol to scan such patients.

Catastrophic Antiphospholipid Syndrome as a Complication of COVID-19 Infection.

COVID-19 infection has been found to precipitate hypercoagulability and transiently increase antiphospholipid antibodies. However, it is yet to be determined how likely these transient changes contribute to thrombotic events and antiphospholipid syndrome. We present a case in which antiphospholipid antibodies were detected in the presence of significant thromboses. The patient was subsequently treated for suspected catastrophic antiphospholipid syndrome following COVID-19 infection.

Screening for acquired latent tuberculosis in rheumatoid arthritis patients on tumor necrosis factor inhibition therapy in Southern California.

BACKGROUND: There are no studies assessing the development of latent tuberculosis infection (LTBI) in patients on tumor necrosis factor inhibitors (TNFα-I) in high TB prevalence areas of the USA. Our objective was to assess the rate of LTBI development in rheumatoid arthritis (RA) patients on TNFα-I therapies in San Bernardino and Riverside Counties of California, high TB prevalence areas in the US. METHODS: Data were extracted from the electronic health record for 217 adult RA patients across three health centers from January 2010 to January 2017 who have had at least 1 year of TNFα-I use and negative initial QuantiFERON Gold status. Demographics, TNFα-I type, duration of use, TB risk factors, QuantiFERON results, rates of re-screening, TB test seroconversion, and its association with drug use and other factors were assessed. RESULTS: Of the 217 patients, 115 (53%) received baseline and annual screening for LTBI. LTBI was diagnosed in 9.4% (10) of patients. Four patients were on infliximab, three on golimumab, two on adalimumab, and one on etanercept. Hispanic patients tended to have a greater than 200% increase in odds of seroconversion compared to non-Hispanic patients. Infliximab and golimumab were associated with a 92% and 400% increase in odds of seroconversion, respectively. CONCLUSION: The LTBI developed in 9.4% of the patients. This is higher than what is reported for previous US studies. Screening for LTBI in the US should take into consideration TB prevalence, ethnicity, drug type, and duration of use. For our local population and similar populations, annual screening should be practiced. Key Points • Although patients on TNFα inhibitor (TNFα-I) therapy are at high risk of latent tuberculosis infection (LTBI), few studies report the rate of LTBI in patients living in high prevalence areas of the US. • The rate of LTBI was 9.4% in patients on TNFα-I therapy in Southern California. The risk of seroconversion was higher in patients of Hispanic ethnicity and also higher for those on infliximab and golimumab compared to those on other TNFα-I therapies. • Screening guidelines for LTBI screening on TNFα-I should consider local TB prevalence, drugs used, duration of use and ethnicity for cost efficient, and optimal healthcare.

Atypical manifestations of sarcoidosis in a Hispanic male.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can present with nonspecific features, often resulting in delayed diagnosis. The diagnosis requires the demonstration of non-caseating granulomas on biopsy. While the prevalence of sarcoidosis in the USA is rare, the disease is rarer yet in Hispanics. It is for this reason that we report herein the case of a Hispanic gentleman with a unique clinical manifestations of sarcoidosis. With what began as a two-month history of joint pain and skin rash, this 55-year-old man was hospitalized with multiple joint pain, weight loss, fatigue and a pruritic rash with leonine facies in the setting of anemia, leukopenia, hypercalcemia, elevated serum creatinine, and urine Bence-Jones proteinuria. CT imaging of the chest was nonspecific, but skin biopsy revealed non-caseating granulomatous disease. After completing an infectious and malignancy evaluation, the patient was diagnosed with sarcoidosis, which was treated successfully with low-dose steroid therapy.

Lumbar Spinal Stenosis in Older Adults.

Lumbar spinal stenosis (LSS) is a frequent cause of low back pain among adults, caused by a narrowing impinging on the spinal cord or nerve roots. Several conditions cause LSS, including disc herniation, spondylolisthesis, tumor, fractures, and other degenerative changes. Back pain is frequently experienced. MRI is the radiologic modality of choice. Radiographic evidence of LSS may not correlate well with symptoms. An increase in utilization of surgery has been noted. However, surgery has no significant benefit over more conservative options. An appropriate risk/benefit discussion between the patient and an interdisciplinary medical team is optimal.

Sarcoidosis and Systemic Sclerosis: Strange Bedfellows.

Coexistence of systemic sclerosis and sarcoidosis is rare. Both have predominant lung manifestations, each with distinctive features on computed tomography (CT) of the chest. We present herein a 52-year-old male with limited systemic sclerosis manifested primarily by sclerodactyly and subsequently by shortness of breath. A series of CT scans of the chest were reviewed. Initial CT chest one year prior to sclerodactyly onset revealed bilateral hilar and right paratracheal, prevascular, and subcarinal adenopathy. Five-year follow-up demonstrated thin-walled cysts, mediastinal lymphadenopathy, and nonspecific nodules. Due to progression of dyspnea, follow-up CT chest after one year again demonstrated multiple cysts with peripheral nodularity and subpleural nodules, but no longer with hilar or mediastinal adenopathy. Diagnostic open lung biopsy was significant for noncaseating granulomas suggestive of sarcoidosis. This is the first known case of a patient with systemic sclerosis diagnosed with sarcoidosis through lung biopsy without radiographic evidence of hilar or mediastinal lymphadenopathy at the time of biopsy. A review of cases of concomitant sarcoidosis and systemic sclerosis is discussed, including the pathophysiology of each disease with shared pathways leading to the development of both conditions in one patient.

Financial conflicts of interest and their association with outcome and quality of fibromyalgia drug therapy randomized controlled trials.

AIMS: To evaluate the association of financial conflicts of interest (FCOI) with the characteristics, outcome and reported methodological quality of fibromyalgia drug therapy randomized controlled trials (FM-RCTs). METHODS: A cross-sectional study of original, parallel-group, drug therapy FM-RCTs published between 1997 and 2011 from Medline and Cochrane Central Register of Controlled Trials was conducted. Two reviewers independently assessed each RCT for funding source, authors' FCOI(s), study characteristics, reporting of methodological measures important for internal validity and outcome (positive [statistically significant result favoring experimental drug for the primary outcome] or non-positive). RESULTS: Forty-seven RCTs were eligible with funding source as: 26 (55.3%) industry; eight (17%) non-profit source(s); five (10.6%) mixed; and eight (17%) unspecified. Industry-funded RCTs were more likely to be multicenter and enroll greater number of patients. Reporting of key methodological measures was suboptimal; however, industry and non-profit funded RCTs did not differ in their reporting. Thirty (63.8%) RCTs had ≥ one author who disclosed an FCOI (receipt of research grant [21, 44.7%], industry sponsor employee [20, 42.6%], receipt of consultancy fee/honorarium [16, 34%] and stock ownership [11, 23.4%]). Although industry funding and certain authors' FCOIs (employment and receipt of consultancy fee/honorarium) were univariately associated with positive outcome, such association was not observed after adjusting for study sample size. CONCLUSIONS: The majority of FM-RCTs were industry-sponsored, and had at least one author with an FCOI. Reporting of key methodological measures was suboptimal. After adjusting for study sample size, no association of industry funding or author's FCOI with study outcome was seen.

The interplay between diet, urate transporters and the risk for gout and hyperuricemia: current and future directions.

Diet plays a significant role in the development of gout and hyperuricemia. Gout and hyperuricemia have likewise been associated with the development of cardiovascular disease and metabolic syndrome. Epidemiological studies have shown that certain foods influence levels of serum uric acid and the risk for development of gout.This article reviews the influence of dietary factors on serum uric acid levels and risk of gout, as well as the role of urate transporters in the development of hyperuricemia and gout.Various epidemiological studies have shown the effects of certain foods on the risk of developing gout and hyperuricemia. Low-fat dairy products, purine-rich vegetables, whole grains, nuts and legumes, and less sugary fruits, coffee and vitamin C supplements decrease the risk, whereas intake of red meat, fructose-containing beverages and alcohol increase the risk of gout. There is also an increased although basic understanding of the effects of vitamin C, alcohol and fructose on urate transporters. Certain foods can lead to a decreased or increased risk of development of gout and hyperuricemia. Advances have established the interplay of certain foods on urate transporters and renal handling of urate. More studies, especially prospective ones, are needed to increase our understanding of the roles of foods and urate transporters and other molecular mechanisms on the risk of developing gout and hyperuricemia.

Soft tissue infections.

Soft tissue infections are common and potentially fatal conditions. Infections are a major cause of morbidity and mortality in patients who have rheumatic disease. Patients who have rheumatic diseases may be at increased risk for soft tissue infections because of various factors, including inherent immunologic defects, genetics, and use of immunomodulatory therapy, including biologic agents. Timely diagnosis and management with the institution of antibiotics with or without surgical intervention is imperative for effective resolution of infection. This article provides a review of recent literature on the presentation and clinical course of infectious tenosynovitis, septic bursitis, pyomyositis, and necrotizing fasciitis, especially in relation to patients who have rheumatic disease.

Sarcoidosis and the rheumatologist.

PURPOSE OF REVIEW: The purpose of this review is to discuss challenges in the diagnosis and treatment of sarcoidosis by rheumatologists. RECENT FINDINGS: Sarcoidosis is a heterogeneous multisystem granulomatous disease. Rheumatologists are faced with multiple challenges in the management of this disease. Features that can have similarities to many rheumatic diseases are being increasingly reported. There are many reports of sarcoidosis coexisting with or mimicking rheumatic diseases such as systemic lupus erythematosus, rheumatoid arthritis, and ankylosing spondylitis. Musculoskeletal features of sarcoidosis can also mimic infection and malignancy. Biomarkers for the diagnosis and monitoring of treatment of response are lacking. Tumor necrosis factor (TNF) inhibition therapy is a viable alternative for immunodulation for various manifestations. However, increased vigilance is needed, as there are also emerging reports of drug-induced sarcoidosis in association with the use of anti-TNFalpha agents and other medications. This article reviews these diagnostic and treatment challenges that rheumatologists face. SUMMARY: Many questions remain to be answered. More studies looking at the reliability of certain serological and radiological biomarkers are needed. Issues concerning the safety of the use of biological response modifiers in inducing sarcoidosis need further study.

Sarcoid arthritis: a review of clinical features, pathology and therapy.

The rheumatic manifestations of sarcoidosis include inflammatory arthritis, periarticular soft tissue swelling, tenosynovitis, dactylitis, bone involvement and myopathy. Two types of arthritis that differ in clinical course and prognosis are recognized. Acute sarcoid arthritis is self-limiting and resolves without permanent sequelae. Chronic sarcoid arthritis although less common can progress to cause joint deformities. There are proliferative and inflammatory changes in the synovium and non-caseating granulomas are seen in half of patients. The pathogenesis of sarcoid arthritis is not well understood, however genetic and environmental factors are important. Drug therapy of sarcoid arthritis with nonsteroidal anti-inflammatory agents, corticosteroids, colchicine, antimalarials and/or immunosuppressive medications is based mainly on open label uncontrolled studies. This review focuses on the current knowledge on the various features of sarcoid arthritis including clinical presentation, course, imaging, and pathology. Recent developments in the usage of anti-tumor necrosis factor therapy for sarcoidosis will be reviewed.