Background In 2001, the European League Against Rheumatism developed and disseminated the first guidelines for musculoskeletal (MS) ultrasound (US) in rheumatology. Fifteen years later, the dramatic expansion of new data on MSUS in the literature coupled with technological developments in US imaging has necessitated an update of these guidelines. Objectives To update the existing MSUS guidelines in rheumatology as well as to extend their scope to other anatomic structures relevant for rheumatology. Methods The project consisted of the following steps: (1) a systematic literature review of MSUS evaluable structures; (2) a Delphi survey among rheumatologist and radiologist experts in MSUS to select MS and non-MS anatomic structures evaluable by US that are relevant to rheumatology, to select abnormalities evaluable by US and to prioritise these pathologies for rheumatology and (3) a nominal group technique to achieve consensus on the US scanning procedures and to produce an electronic illustrated manual (ie, App of these procedures). Results Structures from nine MS and non-MS areas (ie, shoulder, elbow, wrist and hand, hip, knee, ankle and foot, peripheral nerves, salivary glands and vessels) were selected for MSUS in rheumatic and musculoskeletal diseases (RMD) and their detailed scanning procedures (ie, patient position, probe placement, scanning method and bony/other landmarks) were used to produce the App. In addition, US evaluable abnormalities present in RMD for each anatomic structure and their relevance for rheumatology were agreed on by the MSUS experts. Conclusions This task force has produced a consensus-based comprehensive and practical framework on standardised procedures for MSUS imaging in rheumatology.

/pdf/the-2017-eular-standardised-procedures-for-ultrasound-3faw4ulmd5.pdf

The 2017 EULAR standardised procedures for ultrasound imaging in rheumatology

Objective

To determine the accuracy of salivary gland ultrasonography (SGUS) for diagnosing primary Sjogren's syndrome (SS) and to suggest modifications of the American–European Consensus Group (AECG) classification criteria.



Methods

We conducted a cross-sectional study in a prospective cohort of patients with suspected primary SS that was established between 2006 and 2011. The echostructure of the bilateral parotid and submandibular glands was graded from 0 to 4, and the gland size was measured; blood flow to the parotid gland was assessed using Doppler waveform analysis. The reference standard was a clinical diagnosis of primary SS as determined by a group of experts blinded to the results of SGUS. Receiver operating characteristic (ROC) curve analysis was performed to compare the diagnostic value of the 0–4-point echostructure grade for each of the 4 major salivary glands, the sum of the grades for the 4 glands, and the highest grade among the 4 glands.



Results

Of the 158 patients in the study, 78 had a diagnosis of primary SS according to the experts, including 61 patients (78.2%) who met the AECG criteria. Doppler waveform analysis and gland size measurement showed poor diagnostic performance. The results of ROC curve analysis showed that the highest grade among the 4 glands provided the best diagnostic value. The optimal grade cutoff was 2 (62.8% sensitivity and 95.0% specificity). A weighted score was constructed using scores for the 5 variables selected by logistic regression analysis, as follows: (salivary flow × 1.5) + (Schirmer's test × 1.5) + (salivary gland biopsy × 3) + (SSA/SSB × 4.5) + (SGUS × 2). According to ROC curve analysis, a score of ≥5 of 12.5 had 85.7% sensitivity and 94.9% specificity, compared with 77.9% sensitivity and 98.7% specificity for the AECG criteria. The addition of SGUS to the AECG criteria increased sensitivity to 87.0% but did not change specificity.



Conclusion

Modifications of the AECG criteria, including the addition of a SGUS score, notably improved diagnostic performance.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.37698

Contribution of salivary gland ultrasonography to the diagnosis of Sjögren's syndrome: toward new diagnostic criteria?

Objectives. To reveal typical ultrasonographic (US) changes in major salivary glands associated with Sjogren's syndrome (SS) and to determine the diagnostic value of a novel US scoring system. Methods. In 218 consecutive patients with suspected SS, US of both parotid and submandibular salivary glands was performed besides the regular diagnostic procedure following the American-European Consensus Group criteria of 2002. Five US parameters were assessed: echogenicity, inhomogeneity, number of hypoechogenic areas, the hyperechogenic reflections and clearness of the borders of the salivary gland. The grades of these five parameters for all four salivary glands were summed. The final US score ranged from 0 to 48. Results. SS was established in 68 patients. The remaining 150 subjects, in whom SS was not confirmed, constituted our control group. All five US parameters were significantly associated with SS. The patients with SS had significantly higher US scores than those not diagnosed with SS (P< 0.01). Setting the cut-off US score at 17 resulted in the best ratio of specificity (98.7%) to sensitivity (58.8%). Conclusions. Well-defined US changes in the major salivary glands summarized in our novel scoring system were typical of SS patients. Advanced structural changes found on US imaging almost invariably represent SS salivary gland involvement.

Ultrasonographic changes of major salivary glands in primary Sjögren's syndrome. Diagnostic value of a novel scoring system

Objectives. To evaluate ultrasonography (US) of salivary glands in primary Sjogren's syndrome (SS) and to compare US with parotid magnetic resonance (MR) imaging and MR sialography. Methods. US examination of parotid, submandibular and sublingual glands was performed on 27 patients with primary SS, 27 healthy controls and 27 symptomatic controls without SS. The results were compared with parotid MR imaging and MR sialography and the clinical features of the patients. Results. Salivary gland abnormalities, parenchymal inhomogeneity or adipose degeneration, were visualized in 21 (78%) SS patients, in one healthy control and in two symptomatic controls by US. Eighteen (67%) patients had changes in the parotid and submandibular glands and 8 (30%) changes in the sublingual glands. In the comparison, MR sialography was found to be the most sensitive method (96%), followed by MR imaging (81%) and US (78%), in detecting glandular changes. The specificity of US was 94%. The US and MR results were related to anti-Ro/SSA positivity but not to saliva secretion. The focus scores were related only to parotid MR imaging findings. Conclusions. US, MR imaging and MR sialography with modern technology have reached such a good accuracy in visualizing glandular structural changes that they are promising alternatives to the conventional invasive examinations in the diagnostics of SS.

/pdf/ultrasonography-of-salivary-glands-in-primary-sjogren-s-8wm6w27t0c.pdf

Ultrasonography of salivary glands in primary Sjögren's syndrome. A comparison with magnetic resonance imaging and magnetic resonance sialography of parotid glands

The idiopathic inflammatory myopathies (IIMs) of childhood are a rare group of disorders characterized by chronic skeletal muscle inflammation of unknown cause, with onset at less than 18 years of age. The first pediatric case of dermatomyositis was reported by Potain in 1875, 240 and the early history of adult and juvenile dermatomyositis was thoroughly reviewed by Steiner in 1903. 295 Research in this century has concentrated on the epidemiology, etiology, pathogenesis, and clinical characteristics of juvenile dermatomyositis (JDM), the most common of the juvenile IIMs. This article highlights other aspects of the juvenile IIMs: first, the classification of juvenile myositis and the placement of JDM into a broader context of illnesses that share idiopathic chronic muscle inflammation and often meet the same clinical diagnostic criteria 26 and second, the clinical management of these disorders.

Classification and treatment of the juvenile idiopathic inflammatory myopathies

A series of different ultrasonographic abnormalities detected by salivary gland echography (SGE) were investigated for their discriminant power for Sjogren's syndrome (SS) in 53 patients with either primary SS (n = 27) or secondary SS (n = 26), as well as in 90 controls. Among the controls, 26 suffered from dry mouth and/or recurrent or persistent swelling of at least one parotid or submandibular gland due to other selected disorders, while 64 were healthy, asymptomatic subjects. Mild, evident or gross inhomogeneous parenchymal patterns were the only variables selected by stepwise discriminant analysis, when comparing patients to controls. However, a mild submandibular inhomogeneity did not prove useful for such a discrimination. Based on these data, a simplified evaluation and standardised quantification of salivary involvement, as detected by SGE, is proposed using an echographic score (range 0 to 6) which assigns points to the different degrees of glandular inhomogeneity. Score values above 0 showed a sensitivity of 88.8% in primary SS and of 53.8% in secondary SS, as well as a specificity of 84.6% and of 92.2% with respect to either symptomatic or healthy controls. The lower sensitivity of SGE for patients with secondary SS presumably was a result of their milder salivary involvement.

Salivary gland echography in primary and secondary Sjögren's syndrome.

Thirty-seven non-elderly primary Sjogren's syndrome (I SS) patients, and 27 controls complaining of xerostomia and/or recurrent or persistent swelling of at least one parotid or submandibular gland due to other selected disorders, were studied. A new scintigraphic score (scsc) is proposed for a standardised semiquantitative evaluation of salivary involvement by qualitative sequential salivary scintigraphy (SSS), and is compared with two other well-known methods generally used for this purpose. The scsc proved to be much more suitable for such an evaluation: the other criteria did not allow us either to classify or to score a great number of cases. Furthermore, the scsc allowed us to obtain better SSS sensitivity (89.2%) and specificity (96.3%) values for I.SS (33 of the 37 I SS patients and 26 of the 27 controls were correctly classified by discriminant analysis, for scsc values greater than 8). This was made possible by the different "weights" which we assigned to the various glandular and oral parameters in the scsc determination.

Sequential salivary scintigraphy in Sjögren's syndrome: proposal for a new method of evaluation.

Short-term cyclophosphamide pulse therapy was administered to five patients with idiopathic inflammatory myopathies (IIM) which were refractory to corticosteroids and, in two cases, to other aggressive therapies. Both disease remission and a reduction in corticosteroid dosage were obtained in four patients. Three of them have been in remission over since, taking only minimal doses of steroids during the subsequent two years. In the fourth patient a less intensive, maintenance pulse therapy could not be continued due to unacceptable side effects. On the basis of these results and of data from the literature, herein reviewed, cyclophosphamide pulse therapy may be recommended for adult patients with IIM who do not respond to or who do not tolerate conventional corticosteroid treatment, as well as when a steroid-sparing effect is desired.

Treatment of idiopathic inflammatory myopathies with cyclophosphamide pulses: clinical experience and a review of the literature.

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Papers

Salivary gland echography in primary and secondary Sjögren's syndrome.

Sequential salivary scintigraphy in Sjögren's syndrome: proposal for a new method of evaluation.

Treatment of idiopathic inflammatory myopathies with cyclophosphamide pulses: clinical experience and a review of the literature.