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7 Sjgren's Disease/Syndrome
Pages 209-234

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From page 209...
... However, because most of the literature referred to in this chapter was published using the Sjögren's syndrome terminology, this chapter will refer to the disease as Sjögren's syndrome. The primary disease pathogenesis includes exocrine gland inflammation and eventual dysfunction resulting in decreased tear and saliva production.
From page 210...
... , as the immune system targets the salivary glands, which produce saliva, and the lacrimal glands, which produce tears. Those symptoms (dry mouth and dry eyes)
From page 211...
... Such patients experience, on a daily basis, significant vision fluctuation with blinking, blurred vision, eye fatigue, and difficulty reading, which in turn can have a negative impact on employment and workplace productivity. Additionally, SS can cause corneal melt and perforation, uveitis, scleritis, retinal vasculitis, and optic neuritis (Akpek et al., 2019)
From page 212...
... Table 7-1 describes each systemic manifestation and notes its estimated prevalence, clinical manifestations, and method of diagnosis. The prevalences of systemic manifestations are estimated based on data from cohort studies and may range widely.
From page 213...
... livedo reticularis; lichen planus; vitiligo; granuloma annulare; vasculitis Lungs 10–20 Dry cough; nasal dryness; dyspnea; interstitial lung disease Radiography, CT, pulmonary function Kidneys < 10 Distal renal tubular acidosis; nephrogenic diabetes insipidus; proximal Systematic renal tests, acid tubular acidosis; hypokalemia loading test, biopsy Muscles 44 Myalgia; muscle weakness; myositis Biopsy Peripheral nervous 10–19 Axonal sensorimotor polyneuropathy; painful, burning dysesthesias in EMG, small fiber nerve system the distal extremities; sensory ataxic neuropathy; mononeuritis multiplex; biopsy, tilt table test, cranial neuropathies; radiculoneuropathy; autonomic neuropathy quantitative sudomotor axon reflex test Central nervous 20–25 Motor or sensory deficits; seizures or cerebellar syndromes; psychiatric EMG, MRI, CSF system abnormalities; dementia and spinal cord involvement; subacute aseptic investigation, psychiatric meningitis; chorea; optic neuritis; cognitive dysfunction analysis Hematologic 20 Normochromic, normocytic anemia; thrombocytopenia; mild leukopenia; Biochemical tests, bone lymphopenia marrow Biologic 36–62 Hypergammaglobulinemia; hypogammaglobulinemia; hypocomplementia; Serology and biological cryoglobulinemia tests, bone marrow NOTE: CSF = cerebrospinal fluid; CT = computed tomography; EMG = electromyography; MRI = magnetic resonance imaging. SOURCE: Both et al., 2017, with permission.
From page 214...
... . Clinical Diagnosis and Professionally Accepted Classification Criteria The diagnosis of SS involves a combination of taking a medical history, a physical examination, the measurement of tear production, blood tests, and a salivary gland assessment which may include the measurement of saliva production, a gland biopsy, and salivary gland imaging (Romão et al., 2018)
From page 215...
... . The salivary glands can be evaluated using sialometry.
From page 216...
... . The AECG consensus criteria for SS include subjective evidence of disease, objective measures of a decrease in salivary and lacrimal gland function, salivary gland biopsy, and Sjögren-specific auto­antibodies (Table 7-2)
From page 217...
... focal lymphocytic sialoadenitis, biopsy exhibiting evaluated by an expert histopathologist, with a focus score focal lymphocytic ≥ 1, defined as number of lymphocytic foci (which are adjacent sialadenitis with a to normal-appearing mucous acini and contain more than 50 focus score lymphocytes) per 4 mm2 of glandular tissue ≥ 1 focus/4 mm2 5 Salivary gland involvement: objective evidence of salivary gland involvement defined by a positive result for at least one of the following diagnostic tests: Unstimulated whole salivary flow (≤ 1.5 mL in 15 min)
From page 218...
... In particular, patients with dry eyes may experience significant vision fluctuation with blinking, blurred vision, eye fatigue, and difficulty reading on a daily basis, which has a negative impact on employment and workplace productivity (Akpek et al., 2019)
From page 219...
... . There are several known risks factors for lymphoma among patients with SS, including persistent salivary gland enlargement, lymphadenopathy, cryoglobulinemia, higher focus score at diagnosis, and nephritis (Sandhya et al., 2017)
From page 220...
... Unfortunately, irreparable glandular damage often has occurred at the time of sicca development, and therefore return of glandular function is not anticipated. Treatment is aimed at slowing or preventing further glandular damage, alleviating symptoms of dryness, and considering immune modulation in the case of extra-glandular disease (Ramos-Casals et al., 2020)
From page 221...
... . Glucocorticoids are commonly used for episodes of salivary gland swelling as well as for more severe manifestations including severe cutaneous, pulmonary, renal, musculoskeletal, or neurological involvement.
From page 222...
... 222 TABLE 7-3  Drugs Indicated for Treatment of Active Systemic Symptoms of Sjögren's Syndrome Treatment Disease Metho- Mycophenolate Azathio- Lefluno- Cyclopho- Belimu- EculizuInvolvement trexate IVIG mofetil (cellcept) prine mide sphamide Rituximab mab mab*
From page 223...
... Patients require regular monitoring to assess the progression of symptoms and the development of systemic manifestations, additional autoimmune disease, and transformation to lymphoma. There is usually no remission of most symptoms.
From page 224...
... They have been incorporated both into clinical research and into clinical TABLE 7-4  Muscular Domain in the EULAR Sjögrens Syndrome Disease Activity Index Disease Activity Level Description None = 0 Absence of currently active muscular involvement Low = 6 Mild active myositis shown by abnormal EMG, MRI, or biopsy with no weakness, and creatine kinase Moderate = 12 Moderately active myositis proven by abnormal EMG, MRI, or biopsy with weakness (maximal deficit of 4/5) , or elevated creatine kinase High = 18 Highly active myositis shown by abnormal EMG, MRI, or biopsy with weakness (deficit ≤ 3/5)
From page 225...
... . Select Treatments Currently in Clinical Trials Drug approvals for treating SS have lagged behind approvals for other immune disorders such as rheumatoid arthritis.
From page 226...
... Recently, a number of novel drugs to target B cells in SS have shown promise in clinical trials, including belimumab, ianalumab, and iscalimab (Narain et al., 2020; van den Hoogen and van Laar, 2020)
From page 227...
... . Another study in a population of ­working-age Dutch SS patients found that approximately 47 percent received work disability, which was related to male sex, high educational level, a higher number of systemic manifestations, and the use of artificial saliva or hydroxychloroquine (Meijer et al., 2009)
From page 228...
... Parotid swelling often precedes regular oral and ocular symptoms, although serological finding may be absent. Salivary gland ultrasonography shows features typical of primary SS/juvenile SS which can add useful information in diagnosing.
From page 229...
... The disease course of SS is slowly progressive and unlikely to improve, with the exception of extra-glandular involvement. Patients will accrue advancing gland damage which leads to varying levels of decreased exocrine gland function, and they can also accumulate additional autoimmune diagnoses; for example, SS patients are at an increased risk of non-Hodgkin's lymphoma.
From page 230...
... Juvenile SS is a rare and poorly defined disease with a mean age at diagnosis of 10 years. As with adults, the hallmarks of the disease involve exocrine gland inflammation with an eventual loss of function along with the potential for extra-glandular disease.
From page 231...
... 2017. Treatment guidelines for rheumatologic manifestations of Sjögren's syndrome: Use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain.
From page 232...
... 2017. Comparison of 2002 AECG and 2016 ACR/EULAR clas sification criteria and added value of salivary gland ultrasonography in a patient cohort with suspected primary Sjögren's syndrome.
From page 233...
... 2018. Sjögren's syndrome: State of the art on clinical practice guidelines.
From page 234...
... 2008. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: A pooled analysis within the InterLymph Consortium.


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