scholarly journals SUN-304 Hypophisitis in Patients with and Without Autoimmune Rheumatological Disease

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Marlon Vladimir Vázquez Aguirre ◽  
Juan Pablo Godoy-Alonso ◽  
Germán González-de la Cruz ◽  
Andrea Rocha-Haro ◽  
Karla Krystel Ordaz-Candelario ◽  
...  

Abstract Background: Hypophysitis is an inflammatory process of the pituitary gland with different origins. Infundibulum may be also involved, called infundibulum-hypophysitis. Sometimes, enlargement of pituitary gland causes mass effect and anterior or posterior hormonal dysfunction, including hypopituitarism, and diabetes insipidus (DI), respectively. Hypophysitis is a rare autoimmune disease, however, the number of cases have been recently increasing due to higher detection related with more magnetic resonance imaging (MRI) studies. In addition, hypophysitis may be related with autoimmune rheumatologic disease (ARD) such as generalized lupus erythematosus (GLE), granulomatosis with polyangeitis (GPA), IgG4-related disease, and rheumatoid arthritis (RA). Aim: to compare the clinical and biochemical findings among patients with hypophysitis grouped depending on the presence of coexistent ARD activity. Methodology: it is a comparative and cross-sectional study. We registered all data from cases followed-up in Neuroendocrinology and Rheumatology units, from January 1987 to July 2019. Results: 24 patients showed confirmed diagnosis of hypophysitis. Majority of them (n=17, 71%) do not have coexistent diagnosis of ARD. However, 7 cases (29%) presented pituitary involvement with coexistent activity of GPA (n=4, 17%), GLE (n=1, 4%), RA (n=1, 4%) and IgG4-related disease (n=1, 4%). Female gender predominate in hypophysitis cases with and without ARD (p=0.9). Interestingly, age of hypophysitis diagnosis was significantly younger in cases without (38±14) vs. with (49±5) ARD (p=0.01). MRI results showed similar and typical findings related with hypophysitis independently of the presence of ARD. Hypopituitarism was present in the majority of cases, however, none of cases with hypophysitis and ARD showed hypogonadism (p=0.02). DI was present in 15 patients (63%), three of them with ARD (all with GPA). All cases received only medical therapy (i.e., glucocorticoids, rituximab, or azathioprine). Surgery or radiotherapy was not necessary in any case. Conclusions: Almost a 30% of cases with hypophysitis may have coexistent ARD. Pituitary function should be evaluated in cases with previous ARD diagnosis. However, hypogonadism was not present in cases with ARD and hypophysitis. Patients with ARD presented hypophysitis at older age. The remaining clinical and radiological data were similar between groups. References: 1) Clinical Diabetes and Endocrinology. December 2016;2(1). 2) Clin Rheumatol. 2019 Aug 24. doi: 10.1007/s10067-019-04735-7

2020 ◽  
Author(s):  
Eduardo Martín-Nares ◽  
Vanessa Saavedra-González ◽  
Reynerio Fagundo-Sierra ◽  
Blanca Estela Santinelli-Núñez ◽  
Teresa Romero-Maceda ◽  
...  

Abstract Introduction: The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD.Methods: Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren’s syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry.Results: sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) patients, respectively. There were no differences in sFLC among phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher k sFLC and λ sFLC. The AUC for k sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for k sFLC and k/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels.Conclusions: sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.


2020 ◽  
Vol 8 (1) ◽  
Author(s):  
Jorge Ivan Gamez-Nava ◽  
Valeria Diaz-Rizo ◽  
Edsaul Emilio Perez-Guerrero ◽  
Jose Francisco Muñoz-Valle ◽  
Ana Miriam Saldaña-Cruz ◽  
...  

Abstract Background To date, the association of serum macrophage migration inhibitory factor (MIF) and serum adipokines with lupus nephritis is controversial. Objective To assess the utility of serum MIF, leptin, adiponectin and resistin levels as markers of proteinuria and renal dysfunction in lupus nephritis. Methods Cross-sectional study including 196 systemic lupus erythematosus (SLE) patients and 52 healthy controls (HCs). Disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Renal SLE involvement was investigated by renal-SLEDAI. MIF, adiponectin, leptin and resistin levels were quantified by ELISA. We assessed the correlations of quantitative variables by Spearman correlation (rs). Multivariable linear regression adjusted the variables associated with the severity of proteinuria. Results SLE patients had higher MIF (p = 0.02) and adiponectin (p < 0.001) than HCs. Patients with renal SLE involvement (n = 43) had higher adiponectin (19.0 vs 13.3 μg/mL, p = 0.002) and resistin (10.7 vs 8.9 ng/mL, p = 0.01) than patients with non-renal SLE (n = 153). Proteinuria correlated with high adiponectin (rs = 0.19, p < 0.009) and resistin (rs = 0.26, p < 0.001). MIF (rs = 0.27, p = 0.04). Resistin correlated with increased creatinine (rs = 0.18, p = 0.02). High renal-SLEDAI correlated with adiponectin (rs = 0.21, p = 0.004). Multiple linear regression showed that elevated adiponectin (p = 0.02), younger age (p = 0.04) and low MIF (p = 0.02) were associated with the severity of proteinuria. Low MIF and high adiponectin levels interacted to explain the association with the severity of proteinuria (R2 = 0.41). Conclusions High adiponectin combined with low MIF concentrations int+eract to explain the severity of proteinuria in renal SLE. These findings highlight the relevance of adiponectin, resistin and MIF as markers of LN.


Author(s):  
I. Sudoł-Szopińska ◽  
G. A. Santoro ◽  
M. Kołodziejczak ◽  
A. Wiaczek ◽  
U. Grossi

AbstractAnal fistula (AF) is a common referral to colorectal surgeons. Management remains challenging and sometimes controversial. Magnetic resonance imaging (MRI) is commonly performed in initial workup for AF. However, reports often lack key information for guiding treatment strategies. It has been shown that with structured radiology reports, there is less missing information. We present a structured MRI template report including 8 key descriptors of anal fistulas, whose effectiveness and acceptability are being assessed in a cross-sectional study (NCT04541238).


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1509.1-1510
Author(s):  
T. Klein ◽  
S. Tiosano ◽  
A. Chohen ◽  
H. Amital

Background:Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammatory lesions affecting many organ systems in the body. Familial Mediterranean fever (FMF) is an autosomal recessive disease of chronic autoimmune inflammation characterized by frequently relapsing self-limiting fever and inflammation that may be localized in peritoneum, pleura, joint or skin.1Previous studies have described the similarity of clinical symptoms of FMF among SLE patients. However, the literature on this topic is inconsistent and based mostly on case reports.2-4Objectives:To examine the proportions of coexistence of FMF among SLE patients compared to the general population. We hypothesized that the proportion of FMF among SLE patients is higher than the general population.Methods:This cross-sectional study used the Clalit Health Services database, the largest Health Maintenance Organization in Israel, serving 4,400,000 members. SLE patients were compared to age- and sex-matched controls. Chi- was used for univariate analysis.Results:The study included4886 SLEpatients and 24430 age- and sex-matched controls. The SLE group had a significantly higher proportion of FMF patients compared to non-SLE controls (0.68% and 0.21% respectively; p < 0.001).Table 1. All study populationTable 1.SLE patients and matched controls basic characteristicsNo SLESLEp.overallN=24430N=4886Age51.2±16.551.2±16.51.000Gender: Female20100 (82.3%)4020 (82.3%)1.000FMF52 (0.21%)33 (0.68%)<0.001Table 2. StratificationTable 2.comparison of FMF patients with and without SLEFMF without SLEFMF with SLEp.overallN=52N=33Age44.6±13.750.5±17.70.106Gender: Female45 (86.5%)26 (78.8%)0.523Conclusion:FMF was found to be more common amongst SLE patients compared to matched controls.The current study results suggest that the occurrence of SLE turn patients with an appropriate genetic and environmental setting to develop also FMF. This cross-sectional study sheds light on the coexistence of these two diseases, autoimmune and autoinflammatory.References:[1]Kucuk A, Gezer IA, Ucar R, Karahan AY. Familial mediterranean fever.Acta Medica (Hradec Kralove). 2014;57(3):97-104.[2]Lidar M, Zandman-Goddard G, Shinar Y, Zaks N, Livneh A, Langevitz P. SLE and FMF: A possible negative association between the two disease entities–report of four cases and review of the literature.Lupus. 2008;17(7):663-669.[3]Erten S, Taskaldiran I, Yakut ZI. Are systemic lupus erythematosus patients carrying MEFV gene less prone to renal involvement? report of three cases and review of the literature.Ren Fail. 2013;35(7):1013-1016.[4]Shinar Y, Kosach E, Langevitz P, et al. Familial mediterranean Fever gene (MEFV) mutations as a modifier of systemic lupus erythematosus.Lupus. 2012;21(9):993-998.Disclosure of Interests: :None declared


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Eduardo Martín-Nares ◽  
Vanessa Saavedra-González ◽  
Reynerio Fagundo-Sierra ◽  
Blanca Estela Santinelli-Núñez ◽  
Teresa Romero-Maceda ◽  
...  

AbstractThe clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD. Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren’s syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry. sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) of IgG4-RD patients, respectively. There were no differences in sFLC among IgG4-RD phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher κ sFLC and λ sFLC. The AUC for κ sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for κ sFLC and κ/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels. sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.


Healthcare ◽  
2021 ◽  
Vol 9 (4) ◽  
pp. 478
Author(s):  
Joana Proença Becker ◽  
Rui Paixão ◽  
Manuel João Quartilho

(1) Background: Functional somatic symptoms (FSS) are physical symptoms that cannot be fully explained by medical diagnosis, injuries, and medication intake. More than the presence of unexplained symptoms, this condition is associated with functional disabilities, psychological distress, increased use of health services, and it has been linked to depressive and anxiety disorders. Recognizing the difficulty of diagnosing individuals with FSS and the impact on public health systems, this study aimed to verify the concomitant incidence of psychopathological symptoms and FSS in Portugal. (2) Methods: For this purpose, 93 psychosomatic outpatients (91.4% women with a mean age of 53.9 years old) and 101 subjects from the general population (74.3% women with 37.8 years old) were evaluated. The survey questionnaire included the 15-item Patient Health Questionnaire, the 20-Item Short Form Survey, the Brief Symptom Inventory, the Depression, Anxiety and Stress Scale, and questions on sociodemographic and clinical characteristics. (3) Results: Increases in FSS severity were correlated with higher rates of depression, anxiety, and stress symptoms. The findings also suggest that increased rates of FSS are associated with lower educational level and female gender. (4) Conclusion: Being aware of the relationship between FSS and psychopathological symptoms and the need to explore psychosocial issues during clinical interviews may favor early detection of these cases. The early detection of mental disorders is essential for individuals’ adherence to treatments, reflecting on healthcare costs.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Lynnea Myers ◽  
Mai-Lan Ho ◽  
Elodie Cauvet ◽  
Karl Lundin ◽  
Torkel Carlsson ◽  
...  

AbstractWhile previous research has investigated neuroradiological findings in autism spectrum disorder (ASD) and attention-deficit hyperactivity disorder (ADHD), the entire range of neurodevelopmental disorders (NDDs) has not yet been well-studied using magnetic resonance imaging (MRI). Considering the overlap among NDDs and simultaneous development of the brain and face, guided by molecular signaling, we examined the relationship of actionable and incidental (non-actionable) MRI findings and NDD diagnoses together with facial morphological variants and genetic copy number variants (CNVs). A cross-sectional study was conducted with a twin cohort 8–36 years of age (57% monozygotic, 40% dizygotic), including 372 subjects (46% with NDDs; 47% female) imaged by MRI, 280 with data for facial morphological variants, and 183 for CNVs. Fifty-one percent of participants had MRI findings. Males had a statistically significantly higher percentage of MRI findings (57.7%) compared with females (43.8%, p = 0.03). Twin zygosity was not statistically significantly correlated with incidence or severity of specific MRI findings. No statistically significant association was found between MRI findings and any NDD diagnosis or facial morphological variants; however, MRI findings were statistically significantly associated with the number of CNVs (OR 1.20, 95% CI 1.00–1.44, p = 0.05, adjusted OR for sex 1.24, 95% CI 1.03–1.50, p = 0.02). When combining the presence of MRI findings, facial morphological variants, and CNVs, statistically significant relationships were found with ASD and ADHD diagnoses (p = 0.0006 and p = 0.002, respectively). The results of this study demonstrate that the ability to identify NDDs from combined radiology, morphology, and CNV assessments may be possible. Additionally, twins do not appear to be at increased risk for neuroradiological variants.


Sign in / Sign up

Export Citation Format

Share Document