Treatment of Systemic Sclerosis-associated Interstitial Lung Disease: Evidence-based Recommendations. An Official American Thoracic Society Clinical Practice Guideline.

Background: Interstitial lung disease (ILD) is a significant cause of morbidity and mortality in patients with systemic sclerosis (SSc). To date, clinical practice guidelines regarding treatment for patients with SSc-ILD are primarily consensus based. Methods: An international expert guideline committee composed of 24 individuals with expertise in rheumatology, SSc, pulmonology, ILD, or methodology, and with personal experience with SSc-ILD, discussed systematic reviews of the published evidence assessed using the Grading of Recommendations, Assessment, Development, and Evaluation approach. Predetermined conflict-of-interest management strategies were applied, and recommendations were made for or against specific treatment interventions exclusively by the nonconflicted panelists. The confidence in effect estimates, importance of outcomes studied, balance of desirable and undesirable consequences of treatment, cost, feasibility, acceptability of the intervention, and implications for health equity were all considered in making the recommendations. This was in accordance with the American Thoracic Society guideline development process, which is in compliance with the Institute of Medicine standards for trustworthy guidelines. Results: For treatment of patients with SSc-ILD, the committee: 1) recommends the use of mycophenolate; 2) recommends further research into the safety and efficacy of (a) pirfenidone and (b) the combination of pirfenidone plus mycophenolate; and 3) suggests the use of (a) cyclophosphamide, (b) rituximab, (c) tocilizumab, (d) nintedanib, and (e) the combination of nintedanib plus mycophenolate. Conclusions: The recommendations herein provide an evidence-based clinical practice guideline for the treatment of patients with SSc-ILD and are intended to serve as the basis for informed and shared decision making by clinicians and patients.

Large, ulcerated infantile hemangioma of the chest wall complicated by life-threatening hemorrhage: Case report and literature review.

While ulceration is one of the most common infantile hemangioma (IH) complications, severe bleeding is a rare consequence, with a paucity of patients reported. We report a 5-month-old girl with a very large, mixed, partial segmental IH of the upper chest wall who, despite medical intervention, developed severe ulceration and multiple episodes of life-threatening bleeding that ultimately led to hemorrhagic shock. Experience in our patient and a review of six previous reports shows that severe bleeding is a risk when ulceration extends directly into an arterial feeding vessel that is often visible clinically. Other potential predictors for severe bleeding include large to very large IH size with extension of the tumor into underlying structures, segmental or partial segmental patterning, mixed and bulky morphology, and white discoloration as a sign of impending or worsening ulceration.

Antisynthetase syndrome-related interstitial lung disease (ASyS-ILD): longitudinal imaging findings.

OBJECTIVES: Interstitial lung disease (ILD) impacts mortality in antisynthetase syndrome (ASyS). Computed tomographic (CT) patterns and evolution in ASyS ILD are not well described. We report longitudinal CT patterns in ASyS-ILD and their impact on survival. METHODS: This is a monocentric retrospective study of 47 patients with ASyS-ILD. Longitudinal CT patterns and fibrosis severity (severity of radiographic features indicating fibrosis) were analyzed by two radiologists in consensus. The association between imaging features and survival was examined using univariate Cox regression analysis. RESULTS: In total, 211 CT scans were analyzed with an average of 4 ± 2 CT scans/patient with a median follow-up of 79 months in 47 patients. Non-fibrotic patterns were present initially in 63.8% (n = 30) of patients, while fibrotic patterns occurred in 36.2% (n = 17). The initial non-fibrotic patterns/abnormalities resolved in 23.3% (n = 7), evolved in 6.7% (n = 2), persisted in 13.3% (n = 4), and progressed in 56.7% (n = 17), while initial fibrotic patterns persisted in 82.4% (n = 14) and progressed in 17.6% (n = 3). Radiographic progression of ILD (progression in CT pattern or increased fibrosis severity) occurred in 53.2% (n = 25) of patients. Advanced age and radiographic progression were associated with decreased survival (all p < 0.05). The presence of ground-glass opacities (GGO) and predominant lower lung distribution of abnormalities on initial CTs were associated with increased survival (all p < 0.05). CONCLUSION: Progression occurred in 56.7% of ASyS-ILD patients presenting with non-fibrotic patterns. Fibrotic patterns tended to persist. Age and radiographic progression were associated with reduced survival while the initial presence of GGO and predominant lower lobe distribution were associated with increased survival. KEY POINTS: • In ASyS-ILD, initial non-fibrotic patterns such as OP, cNSIP, or OP-cNSIP tended to progress to fNSIP. • Fibrotic patterns such as fNSIP or UIP in ASyS-ILD tended to persist without pattern changes. • GGO and lower lung predominance on initial CT were associated with better survival while advanced baseline age and radiographic ILD progression during follow-up were associated with decreased survival.

Sporadic inclusion body myositis and primary Sjogren's syndrome: an overlooked diagnosis.

Sporadic inclusion body myositis (sIBM) has been reported to occur in association with autoimmune diseases and in particular, primary Sjogren's syndrome (pSS). This brief report describes patients identified with a positive SSA antibody and diagnosis of sIBM at a large academic medical center over a 13.5-year period. A cohort identification tool was used to identify patients with positive SSA antibody and a diagnosis of sIBM between January 1, 2006 and June 1, 2019. All cases of sIBM had diagnostic confirmation by a neuromuscular specialist. Demographics, clinical features, autoantibodies, MRI and EMG findings, and muscle biopsy features were reviewed for each identified case. Eight patients were found to carry the diagnosis of pSS and sIBM. Two additional sIBM patients were SSA antibody positive without other pSS features. The mean time from initial symptom onset of muscle weakness to diagnosis was 5.4 years (range 1-15 years). All patients had alternative diagnoses offered for their myopathic symptoms prior to sIBM identification. The NT5c1A antibody was positive in 7 of 8 patients tested. No patient had a durable response to immunosuppressive therapy. The diagnosis of sIBM went unrecognized for over 5 years in our cohort of SSA antibody-positive patients with myopathy. The patients in this cohort were treated with a variety of immunosuppressive agents prior to diagnosis without benefit. Recognizing the clinical features of sIBM in patients with pSS is crucial in instituting appropriate therapy and avoiding unnecessary immunosuppression. Key Points • Sporadic inclusion body myositis (sIBM) can be associated with Sjogren's syndrome. • In this case series, prevalence of the NT5c1A antibody was higher among patients with associated Sjogren's syndrome compared to the cited prevalence of the NT5c1A antibody in patients with isolated sIBM. • It is crucial to consider sIBM in patients with Sjogren's syndrome presenting with motor weakness in order to avoid unnecessary immunosuppression and institute appropriate therapy.

A multidisciplinary approach to VA ECMO cannulation in children.

PURPOSE: Extracorporeal membrane oxygenation (ECMO) supports gas exchange and circulation in critically ill patients. This study describes a multidisciplinary approach to ECMO cannulation using the expertise of pediatric surgery (PS) and interventional radiology (IR). MATERIAL AND METHODS: Pediatric patients (<18 years) undergoing percutaneous cannulation for peripheral veno-arterial (VA) ECMO by PS and IR from April 2017 to May 2018 were included. Cardiac patients and children cannulated by PS alone were excluded. RESULTS: Five patients were included in the series. Median age was 16 [12.5-17] years and 3 were female. Median ECMO arterial and venous catheter sizes were 19 [17-22] Fr and 25 [25-28] Fr, respectively. Both catheters were placed in the common femoral vessels. A 6Fr antegrade distal perfusion cannula (DPC) was also placed in the superficial femoral artery by IR at the time of cannulation. The median time from admission to procedure start was 10 [7-50] hours and the children were on ECMO for a median length of 3.2 [2.3-4.8] days. There were two episodes of bleeding. No patients had loss of limb circulation. CONCLUSION: A multidisciplinary approach to peripheral VA ECMO cannulation is feasible and safe. Maintenance of limb perfusion by percutaneous placement and removal of DPC may be an advantage of this collaborative approach. LEVEL OF EVIDENCE: IV. TYPE OF RESEARCH: Case series.

Use of Anakinra in Hospitalized Patients with Crystal-associated Arthritis.

OBJECTIVE: In this retrospective observational study, we assess the efficacy and safety of the interleukin 1 receptor antagonist anakinra in medically complex, hospitalized patients with acute gout and calcium pyrophosphate crystal arthritis. METHODS: Adult inpatients treated with anakinra from 2014 to 2017 were identified for inclusion. Charts were reviewed for demographics, comorbidities, laboratory data, pain scores, joint involvement, prior treatment, dosing and response to anakinra, concurrent infections, and surgical interventions. Response to anakinra treatment was determined from review of provider documentation, as well as recorded pain scores on a numeric scale. RESULTS: We identified 100 individuals accounting for 115 episodes of arthritis. This population was 82% male, with an average age of 60 years. Comorbidities included renal disease (45%) and history of organ transplantation (14%). Twenty-nine episodes of arthritis occurred in the perioperative setting. Concurrent infection was present in 34 episodes. Eighty-six episodes of arthritis had partial or complete response to anakinra within 4 days of treatment initiation; 66 episodes had partial or complete response within 1 day of anakinra administration. Anakinra was well tolerated. CONCLUSION: To our knowledge, this is the largest observational study of anakinra use in the inpatient setting for the acute treatment of crystal-associated arthritis. We observed a rapid response to anakinra, with 75% of episodes significantly improving or completely resolving within 4 days of the first dose. Our data also support the use of this biologic agent in individuals with infections, as well as perioperative individuals and immunosuppressed transplant recipients.

Perioperative medication management for the patient with rheumatoid arthritis.

The treatment of rheumatoid arthritis has improved dramatically in recent years with the advent of the latest generation of disease-modifying antirheumatic drugs. Despite these advances, in some patients inflammation is not diminished sufficiently to prevent irreversible musculoskeletal damage, thus requiring surgical intervention to reduce pain and improve function. In these cases, the orthopaedic surgeon frequently encounters patients on a drug regimen consisting of nonsteroidal anti-inflammatory drugs, glucocorticoids, methotrexate, and biologic agents (disease-modifying antirheumatic drugs). Consultation with a rheumatologist is recommended, but the surgeon also should be aware of these medications that could potentially affect surgical outcome. Prudent perioperative management of these drugs is required to optimize surgical outcome. A balance must be struck between minimizing potential surgical complications and maintaining disease control to facilitate postoperative rehabilitation of patients with rheumatoid arthritis.

Impact of educational intervention on confidence and competence in the performance of a simple surgical task.

BACKGROUND: Many complex new procedures involve a learning curve, and patients treated by individuals who are new to a procedure may have more complications than those treated by a practitioner who has performed the intervention more frequently. Still, at some point on the learning curve, each individual must decide that he or she is qualified to perform a procedure, presumably on the basis of his or her level of confidence, background, education, and skill. To evaluate the interrelationship of these factors, we designed a study in which we assessed the performance of a simulated knee joint injection. METHODS: Ninety-three practitioners attending a continuing medical education session on knee injection were randomized to receive skills instruction through the use of a printed manual, a video, or hands-on instruction; each performed one injection before and after instruction. The participants completed pre-instruction and post-instruction questionnaires gauging confidence and also provided self-assessments of their performances of injections before and after instruction. Self-assessments were compared with objective performance standards measured by custom-designed knee models with electronic sensors that detected correct needle placement. RESULTS: Before instruction, the participants' confidence was significantly but inversely related to competent performance (r = -0.253, p = 0.02); that is, greater confidence correlated with poorer performance. Both men and physician-practitioners displayed higher pre-instruction confidence (p < 0.01), which was not correlated with better performance. After instruction, performance improved significantly in all three training groups (p < 0.001), with no significant differences in efficacy detected among the three groups (p = 0.99). After instruction, confidence correlated with objective competence in all groups (r = 0.24, p = 0.04); however, this correlation was weaker than the correlation between the participants' confidence and their self-assessment of performance (r = 0.72, p = 0.001). CONCLUSIONS: Even low-intensity forms of instruction improve individuals' confidence, competence, and self-assessment of their skill in performing the fairly straightforward psychomotor task of simulated knee injection. However, men and physicians disproportionately overestimated their skills both before and after training, a finding that worsened as confidence increased. The inverse relationship between confidence and competence that we observed before the educational intervention as well as the demographic differences that we noted should raise questions about how complex new procedures should be introduced and when self-trained practitioners should begin to perform them.

Musculoskeletal workload versus musculoskeletal clinical confidence among primary care physicians in rural practice.

We studied the prevalence of musculoskeletal conditions in a rural Oregon primary care practice and the self-assessed musculoskeletal knowledge and skills of primary physicians practicing in the area. We recorded how many musculoskeletal complaints were evaluated and treated in a primary care physician's office between April 1 and April 30, 2002. In addition, to all primary care physicians in the surrounding county, we distributed a self-administered questionnaire assessing physician confidence regarding common musculoskeletal conditions and procedures. Prevalence of musculoskeletal conditions for the month was 17.5% (48/274 office visits). Questionnaire results indicated that local physicians felt relatively uncomfortable with common musculoskeletal conditions (mean confidence scores on a 10-point Likert scale: 4.2, musculoskeletal conditions; 9.0, nonmusculoskeletal medical conditions). These findings suggest a disparity between rural primary care physicians' self-assessed musculoskeletal knowledge and skill and the levels they require for their practice.

Ordering and interpreting rheumatologic laboratory tests.

Many mechanical and systemic conditions can cause joint pain and synovitis. When rheumatologic illness is suspected, the initial evaluation begins with an accurate history, physical examination, and selective use of confirmatory testing, which can help avoid common pitfalls inherent in serologic evaluation. Tests for erythrocyte sedimentation rate, C-reactive protein level, rheumatoid factor, antinuclear antibodies, anticardiolipin antibodies and lupus anticoagulant, HLA-B27, uric acid level, and Lyme disease, either alone or in combination, may support certain diagnoses. Using these tests nonselectively may yield false-positive results, causing unnecessary concern and expense. However, using these tests effectively may reduce the number of unneeded invasive procedures.

Quantitative and morphologic change associated with breast cancer-related lymphedema. Comparison of 3.0T MRI to external measures.

UNLABELLED: Abstract Introduction: Lymphedema is a chronic disease of increasing importance to cancer survivors. A tape measurement tool used for lymphedema relies on indirect volume calculations based on external circumference, which may not reflect the true extent of abnormal fluid accumulation accurately. Fluid-sensitive MRI sequences may be able to delineate the severity of this condition more precisely and thus also monitor response to therapy. METHODS AND RESULTS: Eight patients being followed by physical therapy for clinically diagnosed breast cancer-related lymphedema were recruited to participate in this study. External measurements and upper extremity MRI were performed on all subjects. Arm circumference, arm volume, and lymphedema volumes were calculated for each method. MR imaging detected lymphedema in all study subjects. Correlation was found between external circumferential measurements and with the 3.0T MRI (r=0.9368). There was poor correlation between lymphedema volumes calculated from clinical measurements and MR imaging (r=0.5539). CONCLUSIONS: External measurements were not found to be an accurate measure of lymphedema volume associated with breast cancer lymphedema. MRI is a reliable means to obtain upper extremity circumferential and volume measurements. MRI is able to evaluate morphologic change associated with breast cancer-related lymphedema. Lymphedema research requires integrated use of tools to further describe the disease process over time, quantitate the distribution of tissue changes, and improve the sensitivity and specificity of the measurements.

Lipid-associated rheumatologic syndromes.

Rheumatologic manifestations of hyperlipidemia and lipid-associated arthritis are rarely seen in the rheumatologist's office. On the other hand, a rheumatologist may be the clinician who identifies and initiates proper therapy for disorders related to hyperlipidemia when the musculoskeletal manifestations of these syndromes are recognized. In this article both the joint and tendon manifestations are reviewed, including the lesser known lipid liquid crystal form of arthritis. The relationship between gout and hyperuricemia is briefly discussed, as are the autoimmune manifestations of lipid-lowering therapy.

Treatment of acute gouty arthritis in complex hospitalized patients with anakinra.

OBJECTIVE: To report our experience with the efficacy and safety of anakinra for acute gouty arthritis in medically complex hospitalized patients. METHODS: We reviewed the hospital charts of 26 patients treated with anakinra for crystal-induced arthritis since 2007. Demographics, comorbid conditions, reason for anakinra use, response to treatment, and any adverse outcomes were recorded. RESULTS: Twenty-six patients received 40 courses of anakinra therapy. In 67% of patients, pain improved significantly within 24 hours, and complete resolution of signs and symptoms of gout occurred by day 5 in 72.5% of patients. Seven patients received multiple courses with no decrement in response with repeated treatments. Anakinra was well tolerated and no adverse outcomes were attributed to the medication. Only 1 patient appeared to be refractory to this form of interleukin-1 inhibition. CONCLUSION: Anakinra is an effective and safe alternative treatment for acute gouty arthritis in medically complex hospitalized patients who fail or cannot undergo more conventional therapy.

A survey of arthrocentesis and soft-tissue injection procedures performed in primary care practice: effect of resident training and using data to shape curriculum.

OBJECTIVE: The purpose of this study is (1) to survey graduates of our internal medicine program for use of musculoskeletal (MSK) procedures in primary care practice and assess the influence of participating in a first-year resident arthrocentesis and soft-tissue injection training course on their MSK procedure comfort/utilization; and (2) to use the results to modify our MSK procedure curriculum. METHODS: A questionnaire designed to assess numbers of, comfort with, and effect of resident training on MSK procedures in the preceding year was sent to 2002-2006 graduates of the internal medicine training program in outpatient primary care (OPC). Graduates practicing hospital medicine (HM) also received the questionnaire and served as a comparison group. RESULTS: There were 52 responses from this group of 84 graduates (64% response rate). OPC graduates (N = 32) were more comfortable doing procedures than those practicing HM exclusively (N = 20), and performed significantly more procedures in the preceding year (32.9 procedures per OPC/year vs 2.2 for HM). The most common procedures performed were knee joint, subacromial bursa, and trochanteric bursa, comprising > 75% of all procedures performed. A structured resident course in MSK procedures had a significant effect on the OPC physicians. Course participants (N = 17) performed almost twice the number of procedures/year as the nonparticipants (N = 15), were more comfortable with the procedures, and were significantly less likely to refer procedures to other clinicians. Written comments by respondents suggest additional MSK procedure training during and after residency is needed. CONCLUSION: Our results suggest a structured resident course in MSK procedures has a longterm influence. A progressively more focused approach to training is needed.

Evaluation of the activity of an academic rheumatology consult service over 10 years: using data to shape curriculum.

OBJECTIVE: We reviewed rheumatology consults over the last 10 years at a major academic medical center and used these data to revise our fellowship curriculum. METHODS: The medical records of all patient consults from 1994 to 2003 at a university hospital were reviewed with regard to reason for consult, demographic data, and final rheumatologic diagnosis. For comparison we reviewed one year of data from our veterans hospital rheumatology consult service during this same period. RESULTS: A total of 1409 patients were seen on the university hospital consult service between 1994 and 2003. The 5 top reasons for consultation in descending order were: vasculitis, lupus, gout, rheumatoid arthritis, and soft-tissue rheumatic conditions. Specific diagnoses within each category are presented. The number of consults increased significantly over the 10 year period when compared to total hospital admissions. A total of 163 inpatient consults were seen at our veterans hospital in 2001. Crystal arthritis and noninflammatory regional musculoskeletal conditions were the top 2 reasons for consult requests. Many of these consults came from the primary care clinic and required a procedure or simple treatment plan. CONCLUSION: The rheumatology consultation service at our university hospital has become busier over the last 10 years. Since many of the patients had complex problems, we have modified our curriculum approach in response to the information. The veterans hospital data suggest that part of our educational efforts might be directed toward the services requesting rheumatology consultation.