Early referral of patients with suspected polymyalgia rheumatica - A systematic review.

INTRODUCTION: Prompt diagnosis and treatment of polymyalgia rheumatica (PMR) is crucial to prevent long-term complications and improve patient outcomes. However, there is currently no standardized approach to referral of suspected PMR patients to rheumatologists, leading to inconsistent management practices. The objective of this systematic review was to clarify the existing evidence regarding the following aspects of early management strategies in patients with suspected PMR: diagnostic strategies, GCA screening, glucocorticoid initiation prior to referral, value of shared care and value of fast track clinic. METHODS: Two authors performed a systematic literature search, data extraction and risk of bias assessment independently. The literature search was conducted in Embase, MEDLINE (PubMed) and Cochrane. Studies were included if they contained cohorts of suspected PMR patients and evaluated the efficacy of different diagnostic strategies for PMR, screening for giant cell arteritis (GCA), starting glucocorticoids before referral to secondary care, shared care, or fast-track clinics. RESULTS: From 2,437 records excluding duplicates, 14 studies met the inclusion criteria. Among these, 10 studies investigated the diagnostic accuracy of various diagnostic strategies with the majority evaluating different clinical approaches, but none of them showed consistently high performance. However, 4 studies on shared care and fast-track clinics showed promising results, including reduced hospitalization rates, lower starting doses of glucocorticoids, and faster PMR diagnosis. CONCLUSION: This review emphasizes the sparse evidence of early management and referral strategies for patients with suspected PMR. Additionally, screening and diagnostic strategies for differentiating PMR from other diseases, including concurrent GCA, require clarification. Fast-track clinics may have potential to aid patients with PMR in the future, but studies will be needed to determine the appropriate pre-referral work-up.

Association of ultrasound-confirmed axillary artery vasculitis and clinical outcomes in giant cell arteritis.

OBJECTIVES: The aim of this observational study was to compare clinical outcomes including glucocorticoid treatment and relapses between giant cell arteritis (GCA) patients with (axGCA) and without axillary artery involvement (non-axGCA). METHODS: Axillary artery ultrasound was performed in 101 GCA patients at multiple time points. Patients with signs of vasculitis of the axillary arteries at baseline were compared to patients without signs of axillary artery involvement. Cumulative GC doses and relapse rates were calculated as well as survival curves to compare the time until GC discontinuation and occurrence of the first clinical relapse. A linear mixed model was used to assess the effect of a clinical relapse on the intima media thickness (IMT) in axGCA patients. RESULTS: Sixty-seven patients were classified as axGCA, 34 as non-axGCA patients. Compared with non-axGCA, axGCA patients yielded a higher (albeit not significant) median time until GC discontinuation (42 months (95% CI: 33-84) vs 30 months (95% CI: 21-42), p=0.060) and median cumulative GC dose (6801mg (range 1748-34169) vs 5633mg (range: 2553-19967), p=0.051). Time until the first relapse (axGCA: 12 months (95% CI: 8-42) vs non-axGCA: 13.5 months (95% CI: 6-27), p=0522) and relapse rates (2 (range: 0-16) vs 1 (range: 0-13), p=0.67) were similar in both groups. Relapses resulted in an increase of the IMT by 0.18mm (95% CI: 0.07-0.30, p=0.003). CONCLUSION: Patients with axGCA have a trend towards longer treatment duration and higher GC requirements as compared to non-axGCA patients. A relapse leads to an increase of the IMT by 0.18mm.

A study on echocardiographic findings in hospitalized patients with connective tissue diseases.

OBJECTIVE: To determine the prevalence of echocardiographic findings and their change over time in patients with connective tissue diseases (CTDs) and to analyse which findings were associated with escalation of immunosuppressive therapy. METHOD: We conducted a retrospective cohort study of consecutive hospitalized patients from a tertiary rheumatology referral centre who received transthoracic echocardiography between 1 January 2006 and 31 December 2015. We tested for associations between echocardiographic findings and treatment escalation via Fisher's exact test; p < 0.05 was considered significant. Escalation of therapy was defined by dosage of glucocorticoids and type of disease-modifying anti-rheumatic drug. The clinical relevance of echocardiographic findings concerning change in immunosuppressive therapy was recorded. RESULTS: In total, 1004 patients were included (865 females), with a total of 1660 echocardiographic examinations. The most frequent findings were mitral, tricuspid, and aortic valve regurgitation (found in 36.7%, 25.4%, and 17.7% of all patients), aortic valve sclerosis (20.1%), left ventricular dysfunction (21.5%), and left atrial dilatation (19.2%). Only pericardial effusions were more frequent in cases with treatment escalation (10.9% of cases with escalated therapy vs 6.9% of cases without, p = 0.007). In 314 patients who received follow-up examinations, echocardiographic findings were found to change between examinations. Only 73 of all 1660 examinations were discussed in depth considering the treatment strategy in the hospital discharge letter. CONCLUSION: Patients with CTDs exhibited a wide, dynamically changing spectrum of echocardiographic abnormalities. Most findings neither reflected disease activity nor appeared to influence the therapeutic regimen.

[Imaging diagnostics in large vessel vasculitis]. / Bildgebende Diagnostik bei Großgefäßvaskulitiden.

Imaging procedures have become an important diagnostic tool in vasculitis. In large vessel vasculitides, such as giant-cell arteritis (GCA) and Takayasu arteritis, ultrasound, magnetic resonance imaging (MRI), computed tomography (CT) and 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can depict specific abnormalities of the arterial wall. A clinically suspected diagnosis can be confirmed by imaging if performed by a trained specialist using appropriate equipment, without histological investigations. Ultrasound, MRI and CT show a homogeneous, concentric thickening of the arterial wall and PET can detect increased glucose metabolism of the arterial wall. Ultrasound is the method of choice, especially in predominantly cranial GCA. Imaging should be performed before or within the first few days of glucocorticoid treatment as the PET findings of all arteries as well as ultrasound and MRI findings in temporal arteries normalize quickly with treatment. A planned imaging examination must not delay initiation of glucocorticoid treatment.

Settings and artefacts relevant for Doppler ultrasound in large vessel vasculitis.

Ultrasound is used increasingly for diagnosing large vessel vasculitis (LVV). The application of Doppler in LVV is very different from in arthritic conditions. This paper aims to explain the most important Doppler parameters, including spectral Doppler, and how the settings differ from those used in arthritic conditions and provide recommendations for optimal adjustments. This is addressed through relevant Doppler physics, focusing, for example, on the Doppler shift equation and how angle correction ensures correctly displayed blood velocity. Recommendations for optimal settings are given, focusing especially on pulse repetition frequency (PRF), gain and Doppler frequency and how they impact on detection of flow. Doppler artefacts are inherent and may be affected by the adjustment of settings. The most important artefacts to be aware of, and to be able to eliminate or minimize, are random noise and blooming, aliasing and motion artefacts. Random noise and blooming artefacts can be eliminated by lowering the Doppler gain. Aliasing and motion artefacts occur when the PRF is set too low, and correct adjustment of the PRF is crucial. Some artefacts, like mirror and reverberation artefacts, cannot be eliminated and should therefore be recognised when they occur. The commonly encountered artefacts, their importance for image interpretation and how to adjust Doppler setting in order to eliminate or minimize them are explained thoroughly with imaging examples in this review.

[Ultrasound diagnostics in Sjögren's syndrome]. / Ultraschalldiagnostik beim Sjögren-Syndrom.

Ultrasound of the salivary glands is a specific examination for detecting pathology of salivary glands in the diagnosis of Sjögren's syndrome. It is easy to learn, rapidly performed, non-invasive and inexpensive. Other imaging techniques, such as sialography and scintigraphy, are currently only rarely performed. For the examination, linear ultrasound probes with frequencies between 7 and 12 MHz are recommended. Such probes are already widely available to the rheumatologist performing musculoskeletal ultrasound. The parotid and submandibular glands are bilaterally scanned both in longitudinal and transverse planes as a standard.Normal salivary glands have uniformly hyperechoic and homogeneous tissue. They can be clearly delineated from the surrounding muscles and soft tissue and appear similar to the thyroid gland. The salivary glands are typically hypoechoic and inhomogeneous in Sjögren's syndrome. Focal or diffuse hypoechoic or anechoic foci are found in the glands. The submandibular glands may become atrophic (sagittal diameter <8 mm). Particularly in disease flares, the parotid glands may become enlarged (sagittal diameter >20 mm). The sensitivity for the diagnosis is 60 to 90% and the specificity is over 90%.Doppler sonography does not further improve the diagnostic accuracy. Sonography has thus become an important tool in the diagnosis of Sjögren's syndrome.

[Rare manifestations of axial skeleton gouty arthropathy]. / Seltene Manifestationen der Arthritis urica am Achsenskelett.

Clinical manifestations of gouty arthropathy are usually acute inflammatory arthritis, bursitis and accumulation of urate crystals in the form of tophi. Manifestations on the axial skeleton are also known and have been described but occur infrequently and for this reason play a subordinate role in the awareness of rheumatologists. With dual energy computed tomography (CT) gout tophi can be detected even in unusual regions or regions that are difficult to access for puncturing. We describe two rare cases of gouty arthropathy of the axial skeleton in a 76-year-old male patient with spinal involvement and in a 53-year-old female patient with involvement of the sacroiliac joint. In both cases the diagnosis was achieved with dual energy CT.

Arthritis of the Knee Joint in Rheumatoid Arthritis - Evaluation of Treatment Response by Ultrasound in Daily Clinical Practice.

BACKGROUND: Rheumatoid arthritis (RA) commonly involves the knee joint in up to 30% of patients. Musculoskeletal ultrasound enables the skilled clinician to easily assess disease activity. OBJECTIVE: To evaluate the sensitivity to change of the sonography score of large joints in Rheumatology (SOLAR) for different treatments of knee arthritis in RA. METHOD: Joints were assessed by ultrasound at 4 visits. Laboratory, immunological and clinical parameters were recorded. RESULTS: 225 RA patients were analyzed. The DAS 28 in the subgroup receiving systemic steroids was significantly higher (p < 0.001) than in patients treated with intraarticular glucocorticosteroids (GCs) at T0, comparing the values from T0 to T3 the same appeared (p=0.003). Concerning the acute GC treatment regimens, the gray scale ultrasound (GSUS) sum score was found to be significantly higher in patients receiving intraarticular GCs versus no GCs (p=0,035), as well as in patients receiving systemic versus intraarticular GCs (p=0.001). Regarding the differences from T0 and T3, similar to the baseline analysis, a high GSUS sum score was significantly associated with intraarticular GCs, a low to no GC administration (p=0.035), while a high GSUS sum score was significantly linked to intraarticular GCs, rather than systemic GCs (p=0.008). CONCLUSION: SOLAR score is sensitive to change in knee arthritis. Intraarticular GC administration is performed in patients with high GSUS scores. Systemic administration of GC is linked to high disease activity (DAS28) rather than GSUS or power Doppler ultrasound (PDUS) results.

[Spinal epidural lipomatosis as a rare side effect in steroid-dependent Jo-1 antibody syndrome]. / Spinale epidurale Lipomatose als seltene Nebenwirkung bei steroidabhängigem Jo-1-Antikörper-Syndrom.

Spinal epidural lipomatosis (SEL) of the thoracic and lumbar spine is a rare entity, which leads to compression of the spinal canal. The exact pathogenesis is still unknown. It most commonly occurs in patients with long-term exogenous or endogenous glucocorticoid excess or morbid obesity but there are also idiopathic forms. The symptoms depend on the severity of the SEL and can manifest as clinically asymptomatic, non-specific back pain, radiculopathy up to spinal cord compression. The diagnosis is usually achieved by magnetic resonance imaging (MRI) of the affected spinal segments. The treatment varies between discontinuation of glucocorticoids, weight reduction up to multisegmental decompressive laminectomy. The following case report presents the findings of SEL in a patient with steroid-dependent Jo-1 antibody syndrome and provides a current literature review on this rare disease.

[Polymyalgia rheumatica in daily routine practice]. / Polymyalgia rheumatica in der täglichen Praxis.

DEFINITION AND EPIDEMIOLOGY: Polymyalgia rheumatica (PMR) is a very painful inflammatory disease which regularly affects the shoulder region but in 70% of cases the pelvic girdle region is also affected. The disease occurs in people over the age of 50 years and reaches a peak at 72 years old. Women are affected twice as often as men. The prevalence is estimated to be 0.3-0.7% in the Caucasian population over 50 years old. DIAGNOSTICS AND CLASSIFICATION: Misdiagnosis of PMR is common. The differential diagnosis primarily includes impingement syndrome, osteoarthritis of the shoulders, calcifying tendinitis of the rotator cuff, bursitis, omarthritis or inflammatory rheumatic diseases, such as rheumatoid arthritis. Taking a structured medical history and performing a thorough clinical examination are crucial. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels are usually highly elevated and should be investigated particularly in patients who present with new onset bilateral shoulder pain and pronounced general impairment of movement. Imaging shows characteristic inflammatory changes around the shoulders and hip joints. The new European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) classification criteria of PMR including ultrasound imaging are superior to previous classification and diagnostic criteria in terms of positive and negative predictive values. THERAPY: Glucocorticoids are still the mainstay of treatment. Recommended daily prednisolone starting doses are between 15 mg and 25 mg with a weekly dose reduction until 10 mg/day and then further dose reductions of 1 mg per month. Methotrexate can aid reducing prednisolone doses in patients who fail to reach doses below the Cushing threshold quickly enough, which can have major side effects.

Comparison of discrimination and prognostic value of two US Doppler scoring systems in rheumatoid arthritis patients: a prospective cohort study.

OBJECTIVES: The aim of this paper is to investigate sensitivity to change (SRM), predictive validity and discriminative ability of a quantitative (QS) and a semi-quantitative (SQS) Doppler ultrasound scoring systems in patients with rheumatoid arthritis (RA) treated with anti-TNF-α therapy. METHODS: RA patients with wrist joint affection treated with TNF-α inhibitor were followed for one year. The wrist was examined with Doppler before initiating therapy and after one year. DAS28 was determined at both visits. One person trained in the SQS system and one in the QS system evaluated the anonymised images. The SRM, predictive validity and discriminative ability for both systems were calculated using DAS28 as the measure of disease improvement. RESULTS: Fourty-six patients with RA (80% females) were included. The mean Doppler activity at baseline was QS:24.4% (SD=17.7%) and SQS:2.0 (SD=0.6). A decrease in Doppler activity was seen for both systems after anti-TNF-α therapy. Sensitivity to change was seen, SRM=-0.52 (95%CI; -0.83 to -0.21; QS) and -0.24 (-0.53 to -0.05; SQS). Predictive value was poor (QS rs=-0.24; SQS rs=-0.05). Construct validity was; QS: rs=0.29, SQS: rs=0.23. CONCLUSIONS: Both systems were to some extent sensitive to change. Predictive validity and discriminate capacity of both systems showed only a weak association to DAS 28 in the study population. The QS was a little superior to the SQS. The results do not necessarily reflect Doppler evaluation as being ineffective, but may be caused by DAS28 not being a perfect marker of inflammation.

Defining enthesitis in spondyloarthritis by ultrasound: results of a Delphi process and of a reliability reading exercise.

OBJECTIVE: To standardize ultrasound (US) in enthesitis. METHODS: An initial Delphi exercise was undertaken to define US-detected enthesitis and its core components. These definitions were subsequently tested on static images taken from spondyloarthritis patients in order to evaluate their reliability. RESULTS: Excellent agreement (>80%) was obtained for including hypoechogenicity, increased thickness of the tendon insertion, calcifications, enthesophytes, erosions, and Doppler activity as core elementary lesions of US-detected enthesitis. US definitions were subsequently obtained for each elementary component. On static images, the intraobserver reliability showed a high degree of variability for the detection of elementary lesions, with kappa coefficients ranging from 0.13-1. The interobserver kappa values were variable, with the lowest kappa coefficient for enthesophytes (0.24) and the highest coefficient for Doppler activity at the enthesis (0.63). CONCLUSION: This is the first consensus-based US definition of enthesitis and its elementary components and the first step performed to ensure a higher degree of homogeneity and comparability of results between studies and in daily clinical work.

EFSUMB minimum training requirements for rheumatologists performing musculoskeletal ultrasound.

In order to optimize and standardize musculoskeletal ultrasonography education for rheumatologists, there is a need for competency assessments addressing the required training and practical and theoretical skills. This paper describes how these competency assessments for rheumatologists were developed and what they contain.

[Ultrasound in rheumatology. What's new?]. / Sonographie in der Rheumatologie. Was gibt es Neues?

Ultrasound has become an established imaging modality in rheumatology and is now indispensible in the clinical routine. In the last few years the technology has remarkably improved and new areas of application have been introduced. This review provides an overview of the innovations that are relevant in rheumatologic practice.

[Polymyalgia rheumatica]. / Polymyalgia rheumatica.

Polymyalgia rheumatica is characterized by bilateral pain in the shoulder and pelvic girdles, malaise, morning stiffness and weight loss. The erythrocyte sedimentation rate (ESR) and the C-reactive protein (CRP) level are elevated. Imaging using ultrasound, magnetic resonance imaging (MRI) or positron emission tomography (PET) displays inflammatory changes in the painful anatomical areas. Nearly all patients are older than 50 years and the average age is 70-75 years. Polymyalgia rheumatica occurs 2-3 times more often in women than in men and some patients exhibit temporal arteritis or giant cell arteritis. Symptoms rapidly decrease with glucocorticoid treatment and the dose can normally be reduced during the course of the disease. Methotrexate may be given only if high glucocorticoid doses do not sufficiently control symptoms. On average treatment can be stopped after 2 years.

[Giant-cell arteritis: update: diagnosis and therapy]. / Riesenzellarteritis : Update: Diagnose und Therapie.

Giant-cell arteritis (GCA) is the most common form of vasculitis in patients over 50 years old. Loss of vision is the most common severe complication but involvement of extracranial arteries including the aorta is more frequent than previously assumed. The role of dendritic and T-cells indicates the presence of an antibody but a clear association with underlying infections has not yet been demonstrated. Even if the inflammation parameters in blood are mostly increased in the diagnosis of GCA, specific laboratory tests for the diagnosis of GCA are not available. Of the imaging procedures ultrasonography, magnetic resonance imaging and (18)F-fluorodeoxyglucose positron emission tomography can be useful to localize and estimate the extent of vascular involvement. Glucocorticoids are still the standard therapy of GCA, whereby the cumulative dose can possibly be reduced by additional methotrexate. In addition acetylsalicylic acid is recommended as prophylaxis against cardiovascular and cerebrovascular events as well as prophylaxis and therapy of accompanying osteoporosis.