Recommendations from the Brazilian society of rheumatology for the diagnosis of Sjögren's syndrome (Part I): glandular manifestations (systematic review)

Abstract Background: Primary Sjögren's syndrome (pSS) is a systemic immune-mediated disease whose main characteristic is exocrine gland inflammation and, subsequent reduction in tear and saliva production. A delayed diagnosis is common due to the nonspecific clinical manifestations of disease. The aim of the present study was to develop recommendations for the diagnosis of glandular manifestations of pSS based on evidence and expert opinion. Main body of the abstract: We conducted a systematic literature review to retrieve the best evidence available on the accuracy of diagnostic tests for pSS. We also held two in-person meetings with experts (rheumatologists, pathologists, ophthalmologists and dentists) to establish their level of agreement using the Delphi method. Ultimately, we generated 18 recommendations that aim to facilitate the diagnosis of the glandular manifestations of pSS. Conclusion: The diagnosis of glandular manifestations of pSS is complex and multidisciplinary. It requires specific knowledge in the field of ophthalmology, immunology, pathology and imaging, making it compulsory for the rheumatologist to work with professionals from these different areas in order to improve accuracy and early diagnosis. Glandular dysfunction tests, ANA, RF, Anti-Ro, protein electrophoresis, urinalysis, blood count, C-Reactive protein, complement, testing for syphilis and viruses (HCV, HIV) and SGUS should be investigated when dryness or systemic manifestation are present. Minor salivary gland biopsy is recommended for all anti-Ro negative or incomplete criteria cases.

Ultrasound Elastography – Implications in the Head and Neck Region.

Ultrasound elastography (USE) is a method to assess the mechanical properties of tissue, by applying stress and detecting tissue displacement using ultrasound.1 It is based on the principle that malignant tissues are harder than the nonmalignant tissues. While histopathology is a gold standard, USE is a noninvasive method for differentiating benign and malignant lesions in multiple organs. Ultrasonography is used widely as diagnostic means because it is cost-effective, easy to use, and provides information of high resolution. However, no single ultrasound criterion has sufficient diagnostic accuracy and has limited sensitivity or specificity. The aim of this review is to provide an overview on the uses of USE in the head and neck region.

Electrolyte Imbalances and Nephrocalcinosis in Acute Phosphate Poisoning on Chronic Type 1 Renal Tubular Acidosis due to Sjogren's Syndrome

Although renal calcium crystal deposits (nephrocalcinosis) may occur in acute phosphate poisoning as well as type 1 renal tubular acidosis (RTA), hyperphosphatemic hypocalcemia is common in the former while normocalcemic hypokalemia is typical in the latter. Here, as a unique coexistence of these two seperated clinical entities, we report a 30-yr-old woman presenting with carpal spasm related to hypocalcemia (ionized calcium of 1.90 mM/L) due to acute phosphate poisoning after oral sodium phosphate bowel preparation, which resolved rapidly after calcium gluconate intravenously. Subsequently, type 1 RTA due to Sjogren's syndrome was unveiled by sustained hypokalemia (3.3 to 3.4 mEq/L), persistent alkaline urine pH (> 6.0) despite metabolic acidosis, and medullary nephrocalcinosis. Through this case report, the differential points of nephrocalcinosis and electrolyte imbalances between them are discussed, and focused more on diagnostic tests and managements of type 1 RTA.

Uso combinado de la sialografía y el ultrasonido en las enfermedades de las glándulas salivares mayores / Combined use of sialography and ultrasound in diseases of major salivary glands

Se realizó un estudio descriptivo ­ retrospectivo de seis años de trabajo del departamento de Imagenología del Hospital Universitario Camilo Cienfuegos de Sancti Spíritus desde julio de 1998 hasta junio del 2004, para correlacionar los resultados de la sialografía y la ultrasonografía con el diagnóstico anatomopatológico de las lesiones que afectan las glándulas salivares mayores. Se conformó una serie de 70 pacientes con los requisitos para el estudio, 45 masculinos y 25 femeninos, con un rango de edades entre 15 y 82 años. La muestra la constituyeron 36 masas intraglandulares, 22 procesos inflamatorios crónicos no obstructivos, 10 procesos inflamatorios crónicos obstructivos y 2 abscesos simples, los que se clasificaron mediante la observación y análisis de las imágenes en 5 patrones sialográficos y 9 ultrasonográficos que permitieron agrupar las afecciones obtenidas en la muestra. En los resultados obtenidos se encontró asociación de los patrones sialográficos II y ultrasonográfico F en el 50% de los tumores, de los patrones sialográficos III y ultrasonográfico B en alrededor del 70% de los procesos inflamatorios crónicos no obstructivos y de los patrones sialográficos V y ultrasonográfico I en el 80% de las sialolitiasis, por lo que el estudio orienta que ambas técnicas se complementan.(Au)

Ultrasonographic evaluation of salivary gland diseases.

Ultrasonography is an imaging modality in which the high frequency sound waves are sent into the tissues and the acoustic impedence of the tissues are received, amplified and imaged. The echoes of the tissues vary according to their densities. Ultrasonography is applied in the field of medicine, in the evaluation of soft tissues, to record their movements and to assess their vascularity. In the oro-facial region, ultrasonography is employed in the evaluation of salivary glands, cervical lymph nodes, in soft tissues and post-operative swellings. Ten interesting cases of salivary gland diseases are presented here, each case showing a characteristic sonographic feature and providing a useful diagnostic clue in the identification of the disease.

Quantitative assessment of salivary gland function by radioisotopic scanning in a randomized trial of pilocarpine for prevention of radiation induced xerostomia

Radioisotope scanning is the best method for objective assessment of salivary gland function. Thus, it was used in a randomized trial of concomitant pilocarpine for assessment of radiation-induced xerostomia, in addition to subjective evaluation by an approved questionnaire and objective standard xerostomia grading. Patients randomized in placebo-controlled trial of pilocarpine concurrent with irradiation for prevention of radiation-induced xerostomia were evaluated by salivary gland scintigraphy immediately before and 6 months after the end of head and neck radiotherapy. Salivary gland function was measured by ejection fraction [EF] of Technetium-99m pertechnetate. The mean values for pre and post-radiotherapy scans were calculated and compared. Also post-radiotherapy scan findings in the two groups of pilocarpine and placebo were compared using the student's t-test. In addition, comparison was made between the scan results and the subjective findings and objective gradings. Twenty patients underwent the pre-radiotherapy salivary scintigraphy, and also 20 post-radiotherapy scans were performed. Mean parotid EF was 60.85% in the pre-radiotherapy and 9.08% in the post-radiotherapy scans [P<0.01]. The means for submandibular glands in the pre and post-radiotherapy scans were 41% and 11.2%, respectively [P<0.01]. Also the mean EF was 14.5% in the pilocarpine group and 3.65 in the placebo group for parotid glands [P=0.07] and 18.3% and 4.1% respectively for submandibular glands [P<0.05]. The salivary scans confirmed the subjective and objective xerostomia findings. Salivary gland scintigraphy is a valuable method for evaluation of xerostomia after head and neck radiotherapy, quantitatively demonstrating the protective effect of pilocarpine compared to placebo on salivary glands

Salivary gland ultrasonography as a screening method for sjogren's syndrome

To evaluate the usefulness and reliability of high resolution ultrasonography [US] as a safe and non-invasive method for screening the salivary glands to diagnose Sjogren's syndrome [SS] in patients with rheumatoid arthritis and other autoimmune connective tissue disorders attending the Rheumatology Clinic, Al-Amiri Hospital, Kuwait. Forty-five patients with rheumatoid arthritis and 20 patients with other connective tissue disorders, 10 patients with primary SS, and 25 healthy normal individuals, were included in the study US examination of the salivary glands was performed using a linear real time 7.5 MHz probe. Sialography incisional labial salivary gland biopsy excisional parotid gland biopsy as well as specific immunological tests, were performed in many patients. Normal salivary glands were distinctly homogeneous, while salivary glands involved with SS showed a heterogenous echotextural pattern caused by the presence of diffusely scattered focal hypoechogenic areas. These areas represented diffusely scattered aggregates of focal lympho-epithelial infiltrations, considered to be the specific histologic markers of SS, as well as sialectatic sacculations. Depending on the degree of echotextural heterogenity of the salivary glands, SS was classified as either definite or early The prevalence of SS among patients with rheumatoid arthritis was sonographically definite in 60% and early in 20%, while the prevalence among patients with other connective tissue disorders was definite in 10% and early in 25%!. US of the salivary glands is a useful and reliable screening method for detecting and monitoring the progression of salivary gland involvement in Sjogren's syndrome

Evaluation of radiotherapy effect on major salivary glands in head and neck cancer patients by ultrasonography

In 15 patients, the acute parotid inflammatory and degenerative changes appearing shortly after irradiation were sonographically expressed as decreased echogenicity and haziness of the gland contour. In 13 patients, examined 3 years after irradiation, reduction in the gland size as well as diffuse coarse parenchymal pattern with linear areas of echogenicity were demonstrable. This reflects the underlying fibrosis and parenchymal atrophy. Sonography may therefore help in evaluating acute and chronic radiation-induced salivary gland damage

Role of ultrasonography in the diagnosis of salivary gland lesion

Because of the close relation of salivary glands to the surface, sonography considered to be the most method of choice that can assess the different lesions of the salivary glands. This work is based on the sonogram performed on 25 patients, 3 patients with tumors, 5 patients with sialolithiasis, 12 patients with inflammatory diseases of the parotid and submandibular glands, 3 patients with autoimmune diseases and 2 cases were normal