Citations

Background: Rheumatoid arthritis is a relatively common rheumatic disease that can present with inflammatory arthritis and subcutaneous nodules. Multicentric reticulohistiocytosis and fibroblastic rheumatism are rarer entities that also present with these features. Methods: Two cases, one of each of fibroblastic rheumatism and multicentric reticulohistiocytosis, are described highlighting characteristic clinical, radiographic, and histologic findings. A narrative review of the literature on these rarer conditions, compared with rheumatoid arthritis, is provided with a focus on articular and cutaneous findings, available information on disease presentations, and key contrasting features that can aid in diagnosis. Results: Radiographic erosion distribution and joint space narrowing, clinical nodule distribution and characteristics, and nodule histology can differ between these diseases. Conclusions: Multicentric reticulohistiocytosis and fibroblastic rheumatism should be considered in the evaluation of seronegative rheumatoid arthritis, especially in cases that do not respond predictably to standard therapies, and cutaneous nodule biopsy can aid in differentiating these three conditions.

Takayasu arteritis is a chronic, large vessel vasculitis that typically affects the aorta and its main branches. We present a case of a 33-year-old male patient who was diagnosed with Takayasu arteritis after being admitted for acute cardiogenic shock requiring inotropes. Initial transthoracic echocardiography demonstrated severe biventricular dysfunction. Computed tomography imaging of his chest showed pulmonary infarcts. Patient subsequently developed sudden lower extremity weakness and bowel incontinence, and urgent magnetic resonance imaging of his spine demonstrated thoracolumbar vascular myelopathy. Transesophageal echocardiography revealed diffuse symmetrical thickening of the walls of the aorta; findings suggestive of aortitis. Patient was initiated on intravenous corticosteroid and systemic anticoagulation. Subsequent computed tomography angiography demonstrated irregular luminal contour of the aortic arch, and complete occlusion of the left subclavian artery, abdominal aorta, superior mesenteric artery, and left renal artery. He underwent an emergent aortoiliac thrombectomy. He was transitioned to oral prednisone, tocilizumab, and azathioprine with excellent recovery.

ObjectiveNot much is known regarding musculoskeletal ultrasound (MSUS) practices of rheumatologists in the United States. We sought to determine the current use of MSUS among past participants of the Ultrasound School of North American Rheumatologists (USSONAR) training program and, by extension, MSUS‐practicing rheumatologists and to understand barriers to its MSUS use.MethodsAn online survey was sent to 374 participants in the eight‐month USSONAR blended course (Fundamentals in Musculoskeletal Ultrasound and Train the Trainer) between 2009 and 2020. Each respondent had a unique identifier linked to their total number of submitted practice scans and examination scores during training.ResultsThe survey response rate was 28.1% (105 of 374), comprising 82% adult and 18% pediatric rheumatologists. Of the respondents, 71% were MSUS certified: 86.7% performed and/or interpreted diagnostic MSUS, 81.0% performed/interpreted procedural MSUS, 59.8% billed for at least 50% of diagnostic studies, and 78.8% billed for at least 50% of procedural studies. The top reasons for not doing diagnostic and procedural ultrasonography were lack of administrative support and limited time, respectively. For 25% of diagnostic ultrasonography and 12.9% of procedural ultrasonography, billing was done <50% of the time. Of the respondents, 78.0% reported that USSONAR training made them better rheumatologists.ConclusionMost USSONAR‐trained rheumatologists are certified, practicing both diagnostic and procedural MSUS and billing for most of their work. However, a substantial number of studies are not being billed due to time constraints, limited administrative support, and legal liability. Participants agreed that USSONAR training made them better rheumatologists.

Hand impairment is a frequently reported complaint in systemic sclerosis (SSc) patients and a leading cause of disability and diminished quality of life. Managing hand pain can be particularly challenging due to the coexistence of non-inflammatory arthralgias, inflammatory arthritis, acro-osteolysis, tenosynovitis, joint contractures, tendon friction rubs, nerve entrapment, Raynaud’s phenomenon (RP), digital ulcers (DU), sclerodactyly, calcinosis, and chronic pain. While physical examination and radiographs are the first line methods for evaluating hand pain, they are limited in scope and miss many underlying etiologies of hand impairment. We propose a joint ultrasound (US) hand protocol to differentiate between various articular, periarticular, ischemic, skin, and nerve pathologies and to assist in targeted treatment strategies.

Objectives To analyse the amount, reporting and handling of missing data, approach to intention-to-treat (ITT) principle application and sensitivity analysis utilisation in randomised clinical trials (RCTs) of rheumatoid arthritis (RA). To assess the trend in such reporting 10 years apart (2006 and 2016). Methods Parallel group drug therapy RA RCTs with a clinical primary endpoint. Results 176 studies enrolling a median of 160 (IQR 62–339) patients were eligible. In terms of actual analysis: 81 (46%) RCTs conducted ITT, 42 (23.9%) conducted modified ITT while 53 (30.1%) conducted non-ITT analysis. Only 58 of 97 (59.8%) RCTs reporting an ITT analysis actually performed it. The median (IQR) numbers of participants completing the trial and included in analysis for primary outcome were 86% (74%–91%) and 100% (97.1%–100%), respectively. 53 (32.7%) and 65 (40.1%) RCTs had >20% and 10%–20% missing primary outcome data, respectively. Missing data handling was unreported by 58 of 171 (33.9%) RCTs. When reported, vast majority used simple imputation methods. No significant trend towards improved reporting was seen between 2006 and 2016. Sensitivity analysis numerically improved from 2006 to 2016 (14.7% vs 21.4%). Conclusions There is significant discrepancy in the reported and the actual performed analysis in RA drug therapy RCTs. Nearly one-third of RCTs had >20% missing data. The reporting and methods of missing data handling remain inadequate with high usage of non-preferred simple imputation methods. Sensitivity analysis utilisation was low. No trend towards better missing data reporting and handling was seen.

Introduction: Granulomatosis with polyangiitis (GPA) is a vasculitic process that can cause neurological dysfunction in addition to characteristic sinus and pulmonary manifestations. This case report highlights the spectrum of nervous system manifestations and includes rarely reported autonomic and pituitary involvement. Case Report: A 62-year-old woman presented with orthostatic intolerance, tachycardia, dry mouth, and temperature sensitivity; subsequent autonomic reflex study demonstrated widespread postganglionic sympathetic sudomotor, cardiovagal, and cardiovascular adrenergic impairment reflective of autonomic neuropathy and overall autonomic failure. Additional progressive symptoms included dysarthria, dysphagia, bilateral hearing loss, voice hoarseness, and right-sided facial numbness with multiple cranial neuropathies identified on neurological examination. The diagnosis of central diabetes insipidus was also confirmed. Pachymeningitis was present on brain magnetic resonance imaging. Pathologic review of the dural biopsy specimen revealed necrotizing granulomatous vasculitis consistent with GPA. She was treated with intravenous methylprednisolone and rituximab. Over the next 2 months, she had near-complete resolution of her symptoms with normalization on repeat autonomic testing. Conclusions: This is a unique GPA case presenting with autonomic failure and pituitary dysfunction with conclusive objective findings of autonomic dysfunction. Autonomic dysfunction and other disease manifestations were responsive to immunosuppressive therapy.

AbstractAimTo identify the appropriate methods of synovial fluid (SF) specimen storage, manipulation and handling for crystal associated arthritides (CAA) diagnosis.MethodA systematic literature review was conducted using 5 medical databases to identify diagnostic studies assessing SF specimen handling for calcium pyrophosphate (CPP) and monosodium urate (MSU) crystals identification. All included studies were rated for quality using the Quality Assessment of Diagnostic Accuracy Studies 2.ResultsFifteen studies, including 2 non‐English language manuscripts, were included. Eight studies examined both types of crystals, while 3 studies examined CPP and 4 studies examined MSU crystals only. Overall, MSU crystals were more stable over time compared to CPP crystals. MSU stability was generally independent of time, preservative and temperature. CPP crystals deteriorated with time and were more stable if refrigerated. Ethylenediaminetetraacetic acid (EDTA) was a suitable preservative. Re‐examining an initially negative SF sample at 24 hours facilitated detection of additional cases. Very few studies had an overall low risk of bias and applicability.ConclusionMonosodium urate crystals remain stable over time independent of storage time, temperature and preservative. CPP crystals are mostly stable for 24‐48 hours but can deteriorate with time. Overall, SF crystal examination should ideally be done within 24‐48 hours. They may be stored at room temperature without any preservative. Otherwise, refrigeration (4°C/39°F) and EDTA preservation is reasonable. Stored SF re‐examination, at 24 hours, helps identify a small number of additional MSU and CPP cases. Centrifugation techniques allow better and easier crystal identification, particularly CPP. Most studies were of unclear or low quality.