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Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Sjögren syndrome is an autoimmune condition which affects the salivary glands and also has pulmonary associations. Whilst Sjögren syndrome is one of the only connective tissue diseases associated with lymphocytic interstitial pneumonitis (LIP), and the features described in the main stem could be in keeping with LIP. This condition is rare. The most common associated interstitial lung fibrosis is NSIP. This is demonstrated as predominantly ground glass change with reticular opacities and immediate subpleural sparing with traction dilatation of the small airways.
Sjögren Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Sjögren syndrome is an autoimmune disorder characterized by diffuse infiltration of autoreactive lymphocytes into exocrine glands (salivary and lacrimal glands) and other tissues, leading to glandular dysfunction. Based on the presence of particular clinical symptoms, Sjögren syndrome is distinguished into primary and secondary forms. Primary Sjögren syndrome (pSS) only displays xerophthalmia/keratoconjunctiva sicca (dry eyes) and xerostomia (dry mouth), which are collectively referred to as sicca symptoms. However, patients with pSS are prone to non-Hodgkin B-cell lymphoma and possibly other tumors (e.g., breast cancer, lung adenocarcinoma, leukemia) compared to the general population. Secondary Sjögren syndrome (sSS) shows sicca symptoms (i.e., keratoconjunctiva sicca [dry eyes] and xerostomia [dry mouth]) as well as extraglandular manifestations (i.e., autoimmune diseases such as rheumatoid arthritis, lupus erythematosus, and scleroderma) [1].
Sjögren syndrome and mixed connective tissue disease
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Currently, there is no one treatment that can take care of all of the manifestations of Sjögren syndrome or that can modify its natural course. Treatment is directed toward the affected system. Treatment strategies for different cutaneous manifestations are summarized in Table 43.1 [4,5]. Drawing from the experience from other rheumatic diseases, hydroxychloroquine is often used for fatigue, muscle, and joint pains. For more severe internal organ involvement, systemic corticosteroids and steroid-sparing agents such as methotrexate, azathioprine, and cyclophosphamide are used [26]. TNF-α blockers such as infliximab and etanercept have also been tried for the treatment of sicca symptoms with variable results [27,28].
Lipid layer thickness decrease due to meibomian gland dysfunction leads to tear film instability and reflex tear secretion
Published in Annals of Medicine, 2022
Wung Jae Kim, Ye Jin Ahn, Min Ho Kim, Hyun Seung Kim, Man Soo Kim, Eun Chul Kim
All participants were divided into four groups: the normal group comprised subjects who met the following criteria: (1) OSDI less than 12, (2) no tear film abnormality (Schirmer test value ≥5mm and TBUT ≥5 s), and (3) no abnormalities of the lid margins and meibum. The ADDE group included subjects who fulfilled the following criteria: (1) presence of dry eye symptoms (OSDI ≥12), (2) abnormal tear production as determined by the Schirmer test (<5 mm after 5 min) or abnormal tear film stability as determined by TBUT (<5 s), and (3) presence of conjunctival and corneal epithelial damage as evidenced by a fluorescein staining score ≥3, based on the SICCA ocular staining score. Patients with Sjögren’s syndrome were excluded from the study. The MGD group included subjects who fulfilled the following criteria: (1) presence of dry eye symptoms (OSDI ≥12), (2) at least 1 lid margin abnormality, and (3) poor meibum secretion (MGD staging grades 1–5). The ADDE and the MGD group (mixed group) were composed of candidates who met the entry criteria for both the ADDE and MGD groups.
Autoimmune disorders associated with common variable immunodeficiency: prediction, diagnosis, and treatment
Published in Expert Review of Clinical Immunology, 2022
Niloufar Yazdanpanah, Nima Rezaei
Rheumatologic manifestations have been reported in CVID patients through different studies. Chronic inflammatory arthritis is the most common manifestation, which is reported both in adult and pediatric CVID patients. Rheumatoid arthritis (RA) is a chronic multi-system disease that occurs in 1–10% of CVID patients [191]. In a systematic review and meta-analysis study conducted in 2020, the pooled prevalence of rheumatologic manifestations among 3335 CVID patients was 6.4% (95% CI: 3.8–9.0, I2 = 88.3%) [197]. Among the studied rheumatologic disorders, chronic inflammatory arthritis (presenting as RA, juvenile idiopathic arthritis (JIA), or juvenile RA) was the most prevalent with a pooled prevalence of 3.1% (95% CI: 2.1–4.1, I2 = 48.9%), reported in 14 studies with 3149 patients. Sjögren’s syndrome was reported in four studies with 853 patients, which resulted in a pooled prevalence of 1.2% (95% CI: 0.0–2.5, I2 = 71.5%). The pooled prevalence for vasculitis and SLE were 0.8% (5 studies, 1536 patients) and 0.7% (10 studies, 1955 patients), respectively [197]. Controversial reports exist about SLE [191,198]. Relatively reduced activity of SLE is reported in patients who developed CVID in five years after SLE diagnosis; nevertheless, SLE exacerbation was also reported in some patients after CVID diagnosis [191,198].
Ocular Pathophysiology of Sjögren’s Syndrome
Published in Ocular Immunology and Inflammation, 2021
Zujaja Tauqeer, Vatinee Y. Bunya, Ilaria Macchi, Mina Massaro-Giordano
This constellation of findings and its systemic associations have been described in the scientific literature since the late nineteenth and early twentieth centuries, when case reports emerged from Europe describing patients with dry eyes suffering from other dysfunctions of the endocrine system, including rheumatism or gout, as well as lymphocytic aberrations.1 In a series of cases published in the 1930s, the Swedish physician Henrik Sjögren described the cases of 19 female patients of menopausal age suffering from arthritic symptoms who presented with complaints of scratchy eyes with foreign body sensation, burning, and photophobia, and whose conjunctiva stained with rose Bengal, a condition which he termed keratoconjunctivitis sicca and which has since come to bear his own name.2,3 He also presented histological evidence of inflammatory infiltration of the conjunctiva, lacrimal glands, and salivary glands in these patients. Nearly 100 years later there are no one universally accepted criteria for the diagnosis of Sjögren’s syndrome, which can have dry eyes or dry mouth, as classically expected, or less well-known extra-glandular manifestations.4