DermKnowledgeBASE: Sicca Syndrome

Sicca Syndrome

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The text is the summary of recent articles on Sicca Syndrome at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.


According to immunoglobulin composition, cryoglobulinemia is classified into three types [1]. Here, we report our experience over an 18-month period with 15 patients evaluated in the rheumatology department for rheumatic irAEs [2]. All cases required glucocorticoids, and three required a biological agent [3]. VV reports grants and personal fees from Bristol-Myers Squibb, grants and personal fees from Genentech, grants and personal fees from Merck, grants and personal fees from Astra Zeneca, personal fees from Celgene, grants and personal fees from Genoptix, personal fees from Foundation medicine, outside the submitted work [4]. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, Pulmonary, renal, hematological and neurological involvement [5].

In addition, many extrahepatic manifestations including rheumatologic disorders have been reported in up to two-third of HCV infected patients [6]. The mechanism of these drugs, non-specifically activating T Cells, also leads to immune-mediated damage of tissue or immune-related adverse events (IRAE) [7]. Anti-HCV Antibodies and viral ribonucleic acid, HCV RNA, can be found in the cryoprecipitates, together with the rheumatoid factor [8]. Moreover, at the immunohistochemical level in patients with pSS, minor salivary glands showed a peculiar pattern characterized by immunostaining for Tβ10 in acinar Cells in the absence of any reactivity for Tβ4 [9]. In this respect, a multidisciplinary network of experts, the International Study Group of Extrahepatic Manifestations Related to Hepatitis C Virus Infection (ISG-EHCV), was organized with the intention to formulate diagnostic guidelines for the work-up of possible HCV-EHDs [10].

The proposed multidisciplinary expert statement represents the first attempt to draw comprehensive diagnostic guidelines for HCV-infected individuals encompassing the entire spectrum of HCV-related disorders, namely typical hepatic manifestations along with less common, often unpredictable HCV-EHDs [11]. Twenty-one women with pSS and 11 with nS-SS (investigated by xerophtalmia and xerostomia tests, Biopsy of minor salivary glands, gynecological history, and gynecologic symptoms score) underwent vulvar biopsies, evaluated for histopathologic and immunohistochemicalchanges, and compared with those obtained from 26 patients with lichen sclerosus [12]. No differences were found in gynecologic symptoms, as well as in clinical and demographical characteristics between patients with mild and those with moderate vulvar inflammatory score [13]. Women with pSS and nS-SS show a high and similar prevalence of vulvar Inflammatory Infiltrate [14]. The diagnosis of pSS is complex and requires a stepwise approach to evaluate symptoms of ocular and Oral Dryness, objective measures of lacrimal and salivary Gland dysfunction, and evidence of autoimmunity with Ro/La autoantibodies and labial salivary Gland Biopsy [15].

All samples were immunohistochemically evaluated for the presence and Distribution of specific leukocyte subsets using appropriate markers and for the expression of certain immunoregulatory molecules by salivary Gland epithelial Cells [16]. Nine of 13 patients developed an inflammatory arthritis, 4 with synovitis confirmed on imaging (3 Ultrasound, 1 MRI) and 4 with inflammatory synovial fluid [17]. Serositis, joint and neurological involvement were more frequently diagnosed in the South Asian patients [18]. Neurological manifestations are rarely seen in SS although they are debilitating [19]. Peripheral neuropathies namely sensory axonal Neuropathy and Painful small fiber Neuropathy are the most frequent neurological manifestations [20].

The severity of clinical manifestations is variable and sometimes life-threatening [21]. The main presenting Symptom is Pain in the Extremities, whereas at a more advanced stage, the manifestations include Hypertrophic cardiomyopathy, cardiac dysrhythmia, proteinuria, Chronic Kidney dysfunction, stroke, and hearing loss [22]. Musculoskeletal manifestations may constitute the presenting symptoms [23]. Together with the family history, the presence of musculoskeletal manifestations can lead to the correct diagnosis by prompting alpha-galactosidase assays in males and genetic testing in females [24]. fabry disease is often responsible for musculoskeletal manifestations, of which the most common are Pain in the Extremities and osteoporosis [25].

These manifestations can be inaugural and lead to diagnostic wanderings [26]. Published by Oxford University Press on behalf of the British Society for Rheumatology [27].

References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 ,

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