Sjögren's syndrome (SS) is a chronic heterogeneous disease that mainly affects exocrine glands, leading to sicca syndromes such as xerostomia. Despite the second highest prevalence rate among systemic autoimmune diseases, its pathophysiology remains largely unknown. Here we report that SKG mice, a cardinal model of Th17 cell-mediated arthritis, also develop a secondary form of SS-like disorder upon systemic exposure to purified curdlan, a type of β-glucan. The reduced production of saliva was not caused by focal immune cell infiltrates but was associated with IgG deposits in salivary glands. Sera from curdlan-injected SKG mice contained elevated titers of IgG (predominantly IgG1), autoantibody to the muscarinic type 3 receptor (M3R) and inhibited carbachol-induced Ca2+ signaling in salivary acinar cells. These results suggest that the Th17 cells that are elicited in SKG mice promote the production of salivary gland-specific autoantibodies including anti-M3R IgG; the antibodies are then deposited on acinar cells and inhibit M3R-mediated signaling required for salivation, finally leading to hypofunction of the salivary glands. This type II hypersensitivity reaction may explain the origin of secondary SS occurring without focal leukocyte infiltrates.
Acinar Cells ; Animals ; Antibodies ; Arthritis ; Autoantibodies ; Autoimmune Diseases ; Exocrine Glands ; Hypersensitivity ; Immunoglobulin G ; Leukocytes ; Mice ; Prevalence ; Saliva ; Salivary Glands ; Salivation ; Sjogren's Syndrome ; Th17 Cells ; Xerostomia

Recent findings indicate that Type 2 taste receptors (T2Rs) are expressed outside the gustatory system, including in the gastrointestinal tracts and the exocrine glands, such as the submandibular (SM), parotid (P), lacrimal (L) glands and pancreas (PC). Specifically, T2Rs are found in some of the gastrointestinal endocrine cells, and these cells secreted peptide hormones in response to stimulation by bitter-tasting compounds. The results show that T2Rs may have significant physiological roles besides bitter taste reception. The functions of the T2Rs in the exocrine glands remain poorly understood. An expression levels analysis of T2Rs will help to determine those functions in the exocrine glands. The expression levels of the T2Rs in the exocrine glands were discovered via the qPCR. C57BL/6J mice of 42~60-day-old were used. Messenger RNAs were extracted from S, P, L and PC. Cloned DNAs were synthesized by reverse transcription. Quantitative PCRs were performed using the SYBR Green method. The expression levels of the T2Rs were calculated as relative expression levels to that of the GAPDH. The statistical significance among the observed exocrine glands was tested using the variance analysis (ANOVA test). Tas2r108, out of murine 35 T2Rs, was the most highly expressed in every observed exocrine gland. This finding was similar to previous results from tongue papillae, but the expression levels were lower than those of the tongue papillae. Tas2r137 of SM, P, L and PC were expressed a little lower than that of tongue papillae. The T2Rs in the exocrine glands may play slightly different roles from those in the tongue. We suggest that physiological studies such as a patch clamp and functional Ca²⁺ imaging of acinar cells are necessary for understanding the Tas2r108 functions.
Acinar Cells ; Animals ; Clone Cells ; DNA ; Enteroendocrine Cells ; Exocrine Glands ; Gastrointestinal Tract ; Methods ; Mice ; Pancreas ; Peptide Hormones ; Polymerase Chain Reaction ; Reverse Transcription ; RNA, Messenger ; Tongue

Primary Sjögren's syndrome (pSS) is characterized by lymphocytic infiltration of the exocrine glands resulting in decreased saliva and tear production. It uncommonly involves the kidneys in various forms, including tubulointerstitial nephritis, renal tubular acidosis, Fanconi syndrome, and rarely glomerulonephritis. Its clinical symptoms include muscle weakness, periodic paralysis, and bone pain due to metabolic acidosis and electrolyte imbalance. Herein, we describe the cases of two women with pSS whose presenting symptoms involve the kidneys. They had hypokalemia and normal anion gap metabolic acidosis due to distal renal tubular acidosis and positive anti-SS-A and anti-SS-B autoantibodies. Since one of them experienced femoral fracture due to osteomalacia secondary to renal tubular acidosis, an earlier diagnosis of pSS is important in preventing serious complications.
Acid-Base Equilibrium ; Acidosis ; Acidosis, Renal Tubular* ; Autoantibodies ; Diagnosis ; Exocrine Glands ; Fanconi Syndrome ; Female ; Femoral Fractures ; Glomerulonephritis ; Humans ; Hypokalemia ; Kidney ; Muscle Weakness ; Nephritis, Interstitial ; Osteomalacia ; Paralysis ; Saliva ; Tears

Sjogren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocyte-mediated destruction of exocrine glands, which produces classical symptoms of dry eyes and dry mouth. Aside from the clinical manifestations associated with exocrine glands, extraglandular features of SS include a major long-term concern for development of lymphoma. The lifetime risk of non-Hodgkin's lymphoma (NHL) in an SS patient is approximately 5% to 10%, 20 times higher than that of the normal population. This case report describes a rare occurrence of NHL in the eyelid and lung of an adolescent female with SS, whose disease activity had been monitored closely. This is the first reported case in Korea.
Adolescent* ; Conjunctiva ; Exocrine Glands ; Eyelids ; Female ; Humans ; Korea ; Lung* ; Lymphoma ; Lymphoma, Non-Hodgkin* ; Mouth ; Sjogren's Syndrome*

Sjögren Syndrome (SS) is one of the most frequent systemic autoimmune disorders, mainly involving the eye and mouth due to inflammation of lacrimal and salivary glands. Exocrine glands affected with a typical focal lymphocytic infiltration potentially lead to dry eyes and dry mouth. In addition to the known pathogenic mechanism of SS through autoimmunity, corneal neuropathy, as a peripheral neuropathy which is a relatively frequent extraglandular systemic manifestation of SS, recently draws attention as a possible pathogenic mechanism of ocular symptoms and dry eye induction. The diagnostic criteria of SS changed recently, proposed by the American College of Rheumatology/Sjögren's International Collaborative Clinical Alliance (ACR/SICCA) in 2012, and the ocular surface staining score is the only required test for ocular manifestation of SS. However, other diagnostic methods evaluating tear film status, though excluded from the new criteria, are still important for the staging and treatment planning, including direct observation of tear film, tear film break up time, Schirmer test, and measurement of the tear film levels of inflammatory mediators. Eye-specific symptoms and signs and ocular treatment options for SS including tear substitutes, secretogogue, topical anti-inflammatory therapy with corticosteroids and cyclosporine, punctal occlusion, autologous serum, and mucolytic therapy were summarized and discussed in this review article.
Adrenal Cortex Hormones ; Autoimmunity ; Cyclosporine ; Dry Eye Syndromes ; Exocrine Glands ; Inflammation ; Mouth ; Peripheral Nervous System Diseases ; Salivary Glands ; Tears

Renal tubular acidosis (RTA) is a syndrome characterized by hyperchloremic metabolic acidosis and an inability to excrete highly acid urine, in which the impaired acid excretion is disproportional to the reduction in the glomerular filtration rate. Distal renal tubular acidosis (dRTA) is frequently associated with immune-mediated disease, including Sjogren's syndrome. Sjogren's syndrome is a systemic autoimmune disease that mainly affects exocrine glands, such as the lacrimal and salivary glands, resulting in xerophthalmia and xerostomia. Extraglandular manifestations are frequent and may include renal involvement. Recently, we experienced two cases of renal tubular acidosis in patients with Sjogren's syndrome. The first patient had lower extremity weakness and hypokalemia and the second had nephrocalcinosis. We discuss the frequency and pathogenesis of dRTA in Sjogren's syndrome.
Acidosis ; Acidosis, Renal Tubular* ; Autoimmune Diseases ; Exocrine Glands ; Glomerular Filtration Rate ; Humans ; Hypokalemia ; Lower Extremity ; Nephrocalcinosis ; Salivary Glands ; Sjogren's Syndrome* ; Xerophthalmia ; Xerostomia

Sjogren syndrome (SS) is a chronic inflammatory autoimmune disorder involving the exocrine glands, which often presents with salivary and tear gland dysfunction leading to dry mouth and eyes (sicca symptoms). This disease occurs alone as primary SS, or in the background of connective tissue diseases as secondary SS. Neurological involvement is seen in 20-25% of SS cases. Cerebral involvement is generally heterogeneous both in terms of localization (focal or diffuse) and progression (acute, progressive or reversible) and may resemble the clinical and radiological findings of multiple sclerosis (MS). Here we present the case of a patient with primary SS who experienced acute progressive diffuse MS.
Connective Tissue Diseases ; Exocrine Glands ; Humans ; Mouth ; Multiple Sclerosis* ; Sjogren's Syndrome* ; Tears

Sjogren's syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in a dry mouth and eyes. The disease can present either alone or in association with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and inflammatory myopathy. In addition to symptoms caused by exocrinopathy, about half of patients present with extraglandular (systemic) manifestations including arthritis, Raynaud's phenomenon, lymphadenopathy, vasculitis, peripheral neuropathy, and interstitial nephritis. Patients often suffer from severe fatigue, myalgia, and dryness, which lead to a poor quality of life. Physicians should be aware of the increased risk of lymphoma development in patients with Sjogren's syndrome. Currently, there are no drugs available that are able to improve the natural course of disease, making symptom relief the primary goal of therapy. Currently, pilocarpine is the only drug clinically proven for the treatment of dry eyes and mouth in patients with Sjogren's syndrome.
Arthritis ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Exocrine Glands ; Fatigue ; Humans ; Lupus Erythematosus, Systemic ; Lymphatic Diseases ; Lymphoma ; Mouth ; Myalgia ; Myositis ; Nephritis, Interstitial ; Peripheral Nervous System Diseases ; Pilocarpine ; Quality of Life ; Scleroderma, Systemic ; Sjogren's Syndrome* ; Vasculitis

Multiple calcification in the major salivary glands is very rare. Sjogren's syndrome is characterized by tissue damage due to chronic lymphocyte infiltration of exocrine glands, and the involvement of the major salivary glands is followed by typical symptoms such as multiple formation of sialolith, blockage of salivary duct, and edema of the parenchyme. When multiple calcification is found in the parenchyme of parotid gland on the computed tomography imaging, Sjogren syndrome should be considered, where the primary solution is conservative treatment and preventing recurrent inflammation.
Edema ; Exocrine Glands ; Inflammation ; Lymphocytes ; Parotid Gland* ; Salivary Ducts ; Salivary Gland Calculi* ; Salivary Glands ; Sjogren's Syndrome*

Forest musk deer (Moschus berezovskii), a rare wild medicinal animal, is listed under the category of the state key protected wildlife list of China. Musk, secreted by the musk glands, is with high economic and medicinal value and used as precious traditional medicine in China. In order to meet the needs of musk in Chinese traditional medicine, forest musk deer farming was conducted in 1950s, but the research progress on musk secretion mechanism was slow. Therefore, by reviewing the histological and anatomical structure of forest musk deer musk gland, the relationship between sex hormones and the musk secretion process, and the molecular mechanism of the musk secretion, the existing problems in investigating the musk secretion mechanism were analyzed and the development trends in this field were also discussed, in order to provide a reference for further studies on the musk secretion mechanism and improve musk production of forest musk deer.
Animals ; Deer ; metabolism ; Exocrine Glands ; anatomy & histology ; chemistry ; secretion ; Fatty Acids, Monounsaturated ; chemistry ; metabolism ; Male