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Rheumatology International Jul 2023Central nervous system (CNS) involvement can occur in primary Sjögren's syndrome (pSS) due to co-existing neuromyelitis optica spectrum disorder (NMOSD) which has a...
Central nervous system (CNS) involvement can occur in primary Sjögren's syndrome (pSS) due to co-existing neuromyelitis optica spectrum disorder (NMOSD) which has a highly relapsing course requiring indefinite immunosuppression, and if not diagnosed early, damage accrual occurs over time leading to permanent disability and morbidity. In this review, we describe and outline the clinical course and outcomes of anti-aquaporin 4 (AQP4) antibody seropositive NMOSD with pSS overlap cases. To investigate the co-existence of AQP4 + NMOSD with pSS, we conducted a review of individual patient data from case reports and case series found in major databases. The study extracted clinico-demographic features, imaging and laboratory profiles, treatment approaches, and outcomes of these patients. Inclusion criteria for the review required patients to have positivity for anti-AQP4 or NMO-IgG autoantibodies in the blood and/or cerebrospinal fluid (CSF) and exhibit at least one manifestation of both pSS and NMOSD. In this overlap between AQP4 + NMOSD and pSS, 44 patients were included of whom 41 (93.2%) were females. The mean age of pSS onset was 44.8 ± 18.4 years and NMOSD onset was 43.2 ± 19.8 years. In 20 (45.5%) patients, NMOSD preceded pSS onset, 13 (29.5%) NMOSD occurred after pSS onset, and 11 (25%) patients had a simultaneous presentation. 31 (70.5%) patients experienced acute transverse myelitis, 21 (47.7%) optic neuritis, 14 (31.8%) cerebral syndrome, 10 (22.7%) acute brainstem syndrome, 5 (11.4%) area postrema syndrome, and 2 (4.5%) diencephalic clinical syndromes. For the treatment of acute phase, 40 (90.9%) patients received intravenous methylprednisolone, 15 (34.1%) received plasma exchange, and 10 (22.7%) received intravenous immunoglobulin; and for the induction/maintenance therapy, 16 (36.4%) patients received cyclophosphamide, 6 (13.6%) received rituximab, 16 (36.4%) received azathioprine, and 10 (22.7%) received mycophenolate mofetil. Disease course was monophasic in 2 (4.5%) and relapsing in 27 (61.4%) patients. At median (IQR) follow-up duration of 2.4 (6) years, 39 (88.6%) patients showed improvement, 3 (6.8%) showed stabilization and 2 (4.5%) showed worsening of their NMOSD manifestations. In this overlap syndrome of AQP4 + NMOSD and pSS, patients have a neurologically disabling disorder that can mimic neurological manifestations of pSS, frequently occurs prior to the onset of pSS, has a relapsing course, responds well to immunosuppressants, and necessitates indefinite treatment. Collaborative multicentre studies are needed to clarify the natural history and outcomes of this rare overlap syndrome.
PubMed: 37500817
DOI: 10.1007/s00296-023-05397-0 -
The American Journal of Case Reports Jul 2023BACKGROUND Severe hypokalemia, which often causes life-threatening malignant arrhythmias, is usually first diagnosed in the Emergency Department (ED). It is important to...
BACKGROUND Severe hypokalemia, which often causes life-threatening malignant arrhythmias, is usually first diagnosed in the Emergency Department (ED). It is important to note that hypokalemia is often closely and complexly related to renal tubular acidosis (RTA) associated with autoimmune diseases such as Sjögren's syndrome (SS), especially in females with acute myopathy or acute liver injury (ALI). Severe hypokalemia can directly cause muscle injury, which can lead to hyper-creatine kinaseemia (HCK) and ALI, while SS can also directly cause hypokalemia, HCK, and even ALI and renal tubular/interstitial injury. Therefore, by reporting a rare case of SS-associated RTA (SS-RTA), we systematically reviewed the relationship between SS-RTA and severe hypokalemia, which may be beneficial to increase attention on this topic. CASE REPORT A 35-year-old female patient who presented to the ED primarily for limb weakness symptoms was initially diagnosed with severe hypokalemia, acute myopathy, and ALI. She was eventually diagnosed with primary SS (pSS) and SS-RTA, although she did not present with the typical dry mouth, dry eyes, and other clinical manifestations of SS. CONCLUSIONS Severe hypokalemia is a serious life-threatening emergency, and although the differential diagnosis is very broad, we should be aware of RTA associated with autoimmune diseases such as SS in female patients, especially when combined with clinical manifestations such as acute myopathy and ALI that cannot be explained by other causes. Simultaneously, we hope to be able to guide emergency physicians encountering similar patients to complete the diagnostic and therapeutic process.
Topics: Humans; Female; Adult; Acidosis, Renal Tubular; Sjogren's Syndrome; Hypokalemia; Muscular Diseases; Autoimmune Diseases; Creatine
PubMed: 37481699
DOI: 10.12659/AJCR.940268 -
BMC Oral Health Jul 2023Several systemic conditions can result in distinct degrees of salivary gland damage and consequent hypofunction. The development of successful management schemes is... (Review)
Review
BACKGROUND
Several systemic conditions can result in distinct degrees of salivary gland damage and consequent hypofunction. The development of successful management schemes is highly challenging due to the complexity of saliva. This study aimed to systematically map the literature on the physical stimulation of salivary glands for hyposalivation management and the response of individuals according to different systemic conditions causing salivary impairment.
METHODS
A systematic search in the literature was performed. Two reviewers independently selected clinical trials, randomized or not, that used physical stimulation to treat hyposalivation caused by systemic conditions. Studies evaluating healthy subjects without hyposalivation were included as controls. Single-arm clinical studies or case series were also included for protocol mapping (PRISMA extension for scoping reviews).
RESULTS
Out of 24 included studies, 10 evaluated healthy subjects, from which 9 tested transcutaneous electrical nerve stimulation (TENS) and 1 tested acupuncture and electroacupuncture. Fourteen studies evaluated individuals with hyposalivation: 6 applied TENS, 6 applied low-level laser therapy (LLLT), and 2 applied acupuncture, carried out in post-chemotherapy, medication use, postmenopausal women, hemodialysis patients, smokers, diabetics, Sjögren's syndrome (SS). All showed increased salivation after treatment, except for two LLLT studies in individuals with SS.
CONCLUSIONS
Among the different patient groups, individuals with Sjögren's syndrome (SS) exhibited the poorest responses, while those with medication-induced hyposalivation demonstrated the most favorable treatment outcomes, independently of the management strategy for saliva stimulation. It means that physical stimulation of salivary glands holds promise as an alternative for managing hyposalivation in cases of reversible gland damage. However, to make informed decisions in current practice, it is necessary to conduct new well-designed randomized clinical trials with appropriate methodologies.
Topics: Humans; Female; Sjogren's Syndrome; Xerostomia; Saliva; Healthy Volunteers; Physical Stimulation
PubMed: 37480103
DOI: 10.1186/s12903-023-03192-8 -
Temporomandibular disorders in immune-mediated rheumatic diseases of the adult: A systematic review.Seminars in Arthritis and Rheumatism Aug 2023To systematically review the literature concerning temporomandibular disorders (TMDs) in immune-mediated rheumatic diseases (IMRDs) of the adult. The temporomandibular... (Review)
Review
OBJECTIVE
To systematically review the literature concerning temporomandibular disorders (TMDs) in immune-mediated rheumatic diseases (IMRDs) of the adult. The temporomandibular joint (TMJ) outcomes used in clinical studies, the prevalence of TMDs in IMRDs and the risk factors for their development were qualitatively synthetized.
METHODS
A literature search on PubMed Central, Embase and Cochrane Library databases was performed for studies including TMJ outcomes in IMRDs patients compared with healthy controls, other rheumatic diseases or in the assessed IMRDs patients after follow-up and treatment. Among the IMRDs of the adult, original articles investigating TMJ involvement in inflammatory polyarthritides and/or autoimmune connective tissue diseases were considered. The quality of the studies was scored using the Newcastle-Ottawa scale (NOS).
RESULTS
Of the 3259 screened abstracts, 56 papers were included in the systematic review. Most of the papers (77%) investigated TMDs in rheumatoid arthritis (RA) with a prevalence of signs and symptoms varying from 8% to 70%. The risk factors for TMDs development in RA were female sex, younger age, anti-citrulline peptide autoantibodies (ACPA) positivity, higher disease activity, cervical spine involvement, cardiovascular and neuropsychiatric comorbidities. Ten papers (18%) evaluated TMDs in spondylarthritides (SpA) reporting a prevalence of symptoms and signs in 12%-80% of patients with higher TMDs prevalence in patients with radiographic spine involvement, skin psoriasis and HLADRB1×01 positivity. Among autoimmune connective tissue diseases (CTDs), systemic sclerosis (SSc) displayed the highest evidence of TMDs patient-reported outcomes (PROs) and clinical findings (20-93%), followed by systemic lupus erythematosus (SLE) in 18-85%, primary Sjogren's syndrome (24-54%) and idiopathic inflammatory myopathies (4-26%). In SSc and SLE, TMDs were more frequent in patients with higher disease activity and duration, correlating with the extent of skin fibrosis in SSc and with renal involvement in SLE.
CONCLUSION
TMDs in IMRDs display a significant relevance in the rheumatological clinical practice even if often misdiagnosed. This burden is epidemiologically important in terms of PROs and clinical findings which correlate with disease activity in RA, SpA, SSc and SLE. The early recognition and multidisciplinary management of TMDs is warranted and should be aimed at hindering the TMJ structural damage maximizing the quality of life of patients.
Topics: Humans; Adult; Female; Male; Quality of Life; Rheumatic Diseases; Autoimmune Diseases; Arthritis, Rheumatoid; Lupus Erythematosus, Systemic; Scleroderma, Systemic; Temporomandibular Joint Disorders
PubMed: 37167773
DOI: 10.1016/j.semarthrit.2023.152215 -
Zeitschrift Fur Rheumatologie Feb 2024This study aimed to update the prevalence estimates of inflammatory rheumatic diseases (IRD) in Germany.
OBJECTIVE
This study aimed to update the prevalence estimates of inflammatory rheumatic diseases (IRD) in Germany.
METHODS
A systematic literature search in PubMed and Web of Science (last search 08 November 2022) identified original articles (regional and nationwide surveys and claims data analyses for arthritides, connective tissue diseases, and vasculitides) on prevalences for the period 2014-2022. Data sources, collection period, case definition, and risk of bias are reported. Prevalences were estimated from available national data, with consideration of international data.
RESULTS
Screening by two authors yielded 263 hits, of which 18 claims data analyses and 2 surveys met the inclusion criteria. Prevalences ranged from 0.42 to 1.85% (rheumatoid arthritis), 0.32-0.5% (ankylosing spondylitis), 0.11-0.32% (psoriatic arthritis), 0.037-0.14% (systemic lupus erythematosus), 0.07-0.77% (Sjögren's disease/sicca syndrome), 0.14-0.15% (polymyalgia rheumatica, ≥ 40 years), 0.04-0.05% (giant cell arteritis, ≥ 50 years), and 0.015-0.026% (ANCA-associated vasculitis). The risk of bias was moderate in 13 and high in 7 studies. Based on the results, we estimate the prevalence of IRD in Germany to be 2.2-3.0%, which corresponds to approximately 1.5-2.1 million affected individuals. The prevalence of juvenile idiopathic arthritis was reported to be around 0.10% (0.07-0.10%) of 0-18-year-olds, corresponding to about 14,000 children and adolescents in Germany.
CONCLUSION
This systematic review shows an increase in the prevalence of IRD in Germany, which is almost exclusively based on claims data analyses. In the absence of multistage population studies, the available data are, overall, uncertain sources for prevalence estimates, with a moderate to high risk of bias.
Topics: Child; Adolescent; Humans; Prevalence; Arthritis, Rheumatoid; Spondylitis, Ankylosing; Polymyalgia Rheumatica; Sjogren's Syndrome; Lupus Erythematosus, Systemic; Rheumatic Fever; Giant Cell Arteritis; Rheumatic Diseases
PubMed: 36749363
DOI: 10.1007/s00393-022-01302-5 -
The Laryngoscope Aug 2023A number of patient-reported outcome measures (PROMs) assess quality of life and symptom severity in patients with salivary gland dysfunction, but many vary in the... (Review)
Review
OBJECTIVE
A number of patient-reported outcome measures (PROMs) assess quality of life and symptom severity in patients with salivary gland dysfunction, but many vary in the extent of validation and domain types addressed. We identified PROMs validated to measure salivary gland function and analyzed key properties.
DATA SOURCES
PubMed, Web of Science, Embase, PsycInfo, and CINAHL.
REVIEW METHODS
A systematic review was performed using Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Validated instruments with >1 item to assess salivary gland-related symptoms were included. PROMs were evaluated for developmental methodology, structure, validity, and reliability using Consensus-based Standards for the Selection of Health Measurement Instruments (COSMIN) criteria.
RESULTS
A total of 2059 abstracts were retrieved, and 133 full-text articles were reviewed. Sixteen PROMs assessing xerostomia (n = 7), sialadenitis (n = 4), Sjogren's syndrome (n = 2), Parkinson's-associated sialorrhea (n = 2), and oral systemic sclerosis (n = 1) were identified. Most (n = 15) were developed de novo, one was adapted from a pre-existing questionnaire. Eleven PROMs demonstrated "very good" analysis of internal consistency per COSMIN criteria, and 10 included test-retest data. Regarding content validity, four PROMs were developed with both patient and physician input, but none were rated as "adequate." All included comparisons against other questionnaires (n = 7), salivary flow rate (n = 9), and/or healthy controls (n = 3). The most rigorously developed PROM, the Xerostomia Inventory, was rated adequate in 6 out of 7 domains.
CONCLUSIONS
Several PROMs evaluate salivary function. The abilties of these PROMs to meet design and validation standards were variable, with notable limitations in content validity for all tools. New and updated PROMs assessing obstructive and inflammatory salivary symptoms should utilize patient and provider input.
LEVEL OF EVIDENCE
N/A Laryngoscope, 133:1796-1805, 2023.
Topics: Humans; Quality of Life; Reproducibility of Results; Patient Reported Outcome Measures; Surveys and Questionnaires; Consensus
PubMed: 36606658
DOI: 10.1002/lary.30550 -
Zeitschrift Fur Rheumatologie Nov 2023To update the estimated prevalence of inflammatory rheumatic diseases (IRD) in Germany.
OBJECTIVE
To update the estimated prevalence of inflammatory rheumatic diseases (IRD) in Germany.
METHODS
A systematic literature search in PubMed and Web of Science (last search 8 November 2022) identified original articles (regional and nationwide surveys and routine data analyses for arthritides, connective tissue diseases, and vasculitides) on the prevalence for the period 2014-2022. Data sources, collection period, case definition, and risk of bias are reported. The prevalences were estimated from available national data, with consideration of international data.
RESULTS
Screening by 2 authors yielded 263 hits, of which 18 routine data analyses and 2 surveys met the inclusion criteria. Prevalence data ranged from 0.42% to 1.85% (rheumatoid arthritis), 0.32-0.5% (ankylosing spondylitis), 0.11-0.32% (psoriatic arthritis), 0.037-0.14% (systemic lupus erythematosus), 0.07-0.77% (Sjoegren's disease/sicca syndrome), 0.14-0.15% (polymyalgia rheumatica, ≥ 40 years), 0.04-0.05% (giant cell arteritis, ≥ 50 years), and 0.015-0.026% (ANCA-associated vasculitis). The risk of bias was moderate in 13 and high in 7 studies. Based on the results, we estimate the prevalence of IRD in Germany to be 2.2-3.0%, which corresponds to approximately 1.5-2.1 million affected individuals. Prevalence data of juvenile idiopathic arthritis was reported to be around 0.10% (0.07-0.10%) of patients 0-18 years old, corresponding to about 14,000 children and adolescents in Germany.
CONCLUSION
This systematic review shows an increase in the prevalence of IRD in Germany, which is almost exclusively based on routine data analyses. In the absence of multistage population studies, the available data are overall uncertain sources for prevalence estimates at moderate to high risk of bias.
Topics: Child; Adolescent; Humans; Infant, Newborn; Infant; Child, Preschool; Prevalence; Arthritis, Rheumatoid; Spondylitis, Ankylosing; Arthritis, Juvenile; Sjogren's Syndrome; Rheumatic Fever; Giant Cell Arteritis; Rheumatic Diseases
PubMed: 36592211
DOI: 10.1007/s00393-022-01305-2 -
Modern Rheumatology Jul 2023To systemically investigate the prevalence and risk factors of monoclonal gammopathy (MG) in patients with autoimmune inflammatory rheumatic disease (AIIRD). (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To systemically investigate the prevalence and risk factors of monoclonal gammopathy (MG) in patients with autoimmune inflammatory rheumatic disease (AIIRD).
METHODS
A literature search was conducted using databases of PubMed, EMBASE, and Web of Science for relevant studies from inception to 31 July 2021. The pooled prevalence, odds ratio (OR), weighted mean difference (WMD), and 95% confidence interval (CI) were calculated with Stata 16.0 using a random or fixed effects model.
RESULTS
In 17 included studies involving 6667 AIIRD patients, the pooled prevalence of MG in AIIRD patients was 7% (95%CI: 0.06-0.09). Compared to general populations, patients with Sjögren's syndrome (SS) possessed the highest risk for MG (OR 4.51; 95%CI: 3.39-5.74), followed by systemic lupus erythematosus (OR 3.99; 95%CI: 2.84-5.14), ankylosing spondylitis (OR 2.04; 95%CI: 1.11-2.97), and rheumatoid arthritis (OR 2.00; 95%CI: 1.79-2.22). Older age (WMD = 5.17 years; 95%CI: 0.68-9.66), higher erythrocyte sedimentation rate (WMD = 14.04 mm/H; 95%CI: 7.77-20.30), higher serum gammaglobulins level (WMD = 1.92 mg/dl, 95%CI: 0.51-3.32) were associated with a greater risk of MG in AIIRD patients.
CONCLUSIONS
MG prevalence was higher in AIIRD patients, especially in SS patients. Older age, higher erythrocyte sedimentation rate, and hypergammaglobulins were risk factors for MG in AIIRD patients.
Topics: Humans; Prevalence; Sjogren's Syndrome; Risk Factors; Arthritis, Rheumatoid; Paraproteinemias
PubMed: 35786736
DOI: 10.1093/mr/roac066 -
Acta Neurologica Belgica Oct 2023
PubMed: 34709595
DOI: 10.1007/s13760-021-01823-x