Rheum Dis Clin North Am [journal]
- Sjögren Syndrome in the Twenty-First Century. [Editorial]
- Rheum Dis Clin North Am 2016 Aug; 42(3):xiii-xiv.
- Sjogren's Disease. [Editorial]
- Rheum Dis Clin North Am 2016 Aug; 42(3):xi-xii.
- New Treatment Guidelines for Sjögren's Disease. [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):531-51.
Sjögren's disease is associated with a high burden of illness, diminished quality of life, and increased health care costs. The Sjögren's Syndrome Foundation developed the first US clinical practice guidelines for management of the oral, ocular, and rheumatologic or systemic manifestations. Guideline recommendations were reviewed by a consensus expert panel using a modified Delphi process. This initiative should improve the quality and consistency of care for Sjögren's disease in the United States, guide insurance reimbursement, and define areas for future study. Guidelines will be periodically reviewed and revised as new information becomes available.
- Sjögren Syndrome: Why Do Clinical Trials Fail? [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):519-30.
Sjögren syndrome (SS) comprises glandular and extraglandular manifestations. Double-blind prospective trials of traditional disease-modifying antirheumatic drugs and biologics have failed because they have not improved benign symptoms, the major cause of lowered quality of life. Rituximab has proven effective in SS patients with associated mixed cryoglobulinemia, parotid gland swelling, lymphocytic interstitial pneumonitis, thrombocytopenia, and other manifestations. There were few of these SS patients in the trials required for FDA approval. Most patients had benign symptoms and did not show benefit, leading to failure of the study. This article examines the reasons for these failures and proposes future directions.
- Major Salivary Gland Ultrasonography in the Diagnosis of Sjögren's Syndrome: A Place in the Diagnostic Criteria? [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):501-17.
Major salivary gland (SG) ultrasonography (US) represents a noninvasive, nonirradiating imaging modality for evaluation of the major SGs in the diagnosis and follow-up of primary and secondary Sjögren syndrome. Structural changes can be visualized as hyperechogenic and hypoechogenic areas, inhomogeneity, and altered echogenicity in general. The reliability of SG-US is poorly investigated, and the definition of US abnormalities varies in previously published studies. Recent studies have shown correlations between SG-US findings and focus score in the minor SGs; however further studies are needed to validate a US criterion in updated classification/diagnostic criteria.
- Parotid Gland Biopsy, the Alternative Way to Diagnose Sjögren Syndrome. [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):485-99.
Salivary gland biopsy is a technique broadly applied for the diagnosis of Sjögren syndrome (SS), lymphoma in SS, and connective tissue disorders (sarcoidosis, amyloidosis). In SS characteristic histology findings are found, including lymphocytic infiltration surrounding the excretory ducts in combination with destruction of acinar tissue. In this article the main techniques are described for taking labial and parotid salivary gland biopsies with respect to their advantages, postoperative complications, and usefulness for diagnostic procedures, monitoring disease progression, and evaluation of treatment.
- Salivary Gland Pathology in Sjögren's Syndrome. [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):473-83.
Primary Sjögren syndrome (pSS) can be considered a systemic autoimmune disease with a strong organ bias. The involvement of the exocrine glands is prevalent and drives the pathognomonic manifestations of dryness that define the sicca syndrome. The salivary glands also represent the hub of pSS pathology. Elements belonging to both innate and acquired immune responses have been described at this site that contribute to disease establishment and progression. The interaction between those elements and their relative contributions to the clinical manifestations and lymphoma progression largely remain to be addressed.
- Polyautoimmunity in Sjögren Syndrome. [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):457-72.
Polyautoimmunity is defined as the presence of more than one well-defined autoimmune disease (AD) in a single patient. Polyautoimmunity is a frequent condition in Sjögren syndrome (SS) and follows a grouping pattern. The most frequent ADs observed in SS are autoimmune thyroid disease, rheumatoid arthritis, and systemic lupus erythematosus. Main factors associated with polyautoimmunity in SS are tobacco smoking and some genetic variants. The study of polyautoimmunity provides important clues for elucidating the common mechanisms of autoimmne diseases (ie, the autoimmune tautology).
- The Proteomics of Saliva in Sjögren's Syndrome. [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):449-56.
One of the main characteristics of primary Sjögren's syndrome (pSS) is chronic dysfunction and destruction of the salivary and lacrimal glands; their secretory biofluids should reflect the glandular biological status. Saliva is a heterogeneous biofluid comprised of biomolecules and omics constituents that are altered in response to various diseases. Scientific effort has evaluated saliva proteome to diagnose, monitor, and prognosticate pSS. This article reviews the recent advances in salivary proteomics in the context of pSS, highlighting the most significant and promising findings. Determining saliva as a credible means of early disease detection could lead to translational advantages and significant clinical opportunities for pSS.
- Genetics in Sjögren Syndrome. [Journal Article, Review]
- Rheum Dis Clin North Am 2016 Aug; 42(3):435-47.
The genes associated with Sjögren syndrome (SS) can be assigned to the NF-kB pathway, the IFN signaling pathway, lymphocyte signaling, and antigen presentation. The frequencies of risk variants show they are common with modest genetic effects. The strongest genetic association outside the human leukocyte antigen region is in IRF5, a gene relevant in the IFN signaling pathway and for B cell differentiation. Although no association has been found with the NF-kB gene itself, associations in TNFAIP3 and TNIP1 (both genome-wide significant), VCAM1 and IRAK1BP (both suggestive), point to genetic explanations for dysregulation of the NF-kB pathway in SS.