Mediterranean Journal of Rheumatology最新文献

Objective: Behçet's disease (BD) may experience heightened pain sensitivity, potentially related to central sensitisation (CS). The hypersensitive central nervous system causes physical inactivity and kinesiophobia due to overreacting to stimuli that would not usually cause pain. This study aims to determine the frequency of CS in patients with BD and to evaluate the relationship between CS and kinesiophobia, exercise capacity, disease activity, and quality of life (QoL).

Methods: The study, which included 55 patients with BD and 55 healthy controls (HCs), employed a comprehensive approach. All participants were administered the Tampa Kinesiophobia Scale (TKS), the 6-minute walk test (6MWT), and the central sensitisation inventory.

Results: CS was detected in 61.4% of with BD. Among the 55 patients, 24 (45.5%) were male, with a median age of 42 years (IQR 17) and a median disease duration of 8 years (IQR 10). Compared to age- and gender-matched HCs, patients with BD exhibited higher CS, increased kinesiophobia, and shorter walking distances. There were moderate correlations between CS scores and the 6MWT, TKS, BDCAF, and BDQoL in patients with BD (Rho = 0.51, Rho = 0.56, Rho = 0.48, and Rho = 0.56, respectively; all p-values < .001). Hierarchical linear regression analysis demonstrated a significant association between QoL and the presence of CS (95% Confidence Interval (CI) 0.033-0.562, p=0.028) and kinesiophobia (95% CI 0.245-0.766, p < 0.001).

Conclusion: The prevalence of CS and kinesiophobia in BD patients is a significant finding, shedding light on the factors contributing to reduced QoL and functional exercise capacity.

Background: Juvenile Dermatomyositis (JDM) is a systemic autoimmune disease in children, characterised by skin and muscle inflammation, with incidence of 1.6 to 4 cases per million children annually. Calcinosis, affecting 20% to 70% of JDM patients, can lead to significant morbidity. The association of calcinosis with anti-Mi-2 antibodies is rare and complicates management.

Case: We present a rare case of a 12-year-old girl diagnosed with JDM, extensive calcinosis, and positive anti-Mi-2 antibodies. The patient exhibited significant muscle weakness, skin manifestations, and painful calcinosis leading to contractures. Initial management included tofacitinib and intravenous pamidronate, resulting in no new calcinosis formation.

Discussion: A comprehensive review of existing literature highlights the rarity of calcinosis in anti-Mi-2 positive patients. While traditional treatments have shown variable effectiveness, emerging therapies like JAK (Janus Kinase) inhibitors may offer new avenues for management. The literature underscores the need for personalised treatment strategies given the atypical presentations and outcomes.

Conclusion: This case adds to the limited documentation of calcinosis in JDM with anti-Mi-2 antibodies, emphasising the need for increased awareness and research. Personalised treatment approaches are crucial, and future studies should focus on larger datasets and emerging therapeutic modalities to optimise management and improve patient outcomes.

Objective/aim: Differentiating the gastrointestinal (GI) involvement of Behçet's disease (BD) and inflammatory bowel diseases (IBD) can be a diagnostic challenge. We previously reported that the wall thickness of the common femoral vein (CFV) is higher in BD patients compared to Crohn's disease (CD) with a limited number of IBD patients with only CD. This study aimed to evaluate the CFV thickness measurement in BD patients and in a larger group of IBD patients including both ulcerative colitis (UC) and CD.

Methods: The study included patients with BD (n=117), IBD (n=87, [53 CD, 34 UC]), and healthy gender-matched controls (HC) (n=85). CFV wall thicknesses were measured with Doppler ultrasonography.

Results: Among BD patients, 70 (59.8%) had major organ (48[41.0%] vascular, 21[30.0%] ocular, 11[15.7%] gastrointestinal, and 8[11.4%] neurological) involvement. The mean right CFV thickness was 0.75±0.21 mm, 0.32±0.08 mm, and 0.28±0.13 mm for BD, IBD, and HC, respectively (p<0.001). BD patients had significantly higher CFV wall thicknesses compared to IBD patients and HC (adj. p<0.001 for both), and CFV thicknesses in IBD were comparable to HC (adj.p>0.05). Among BD patients, CFV thicknesses did not differ in patients with and without GI involvement. CFV thicknesses were also similar in patients with CD and UC.

Conclusion: CFV thickness was significantly higher in BD compared to CD and UC patients. These results suggest that the CFV wall thickness measurement may be used in daily practice to differentiate GIBD from IBD.

Introduction: Inadequate production of cortisol in relation to increased demands of chronic inflammation, a phenomenon coined as the "disproportion-principle", occurs in some patients with active rheumatoid arthritis (RA). Moreover, relatively lower diurnal cortisol production prior to antirheumatic treatment initiation/escalation for active RA has been associated with inadequate corresponding treatment responses after 6-months.

Objective: To evaluate whether individual levels of endogenous cortisol in active RA patients followed in an Academic Rheumatology Unit may predict the type of response to subsequent antirheumatic treatment regimens after two years.

Methods: We measured morning circulating ACTH, cortisol and DHEAS blood levels, as well as saliva diurnal-cortisol levels (collected samples at 08:00, 12:00, 18:00, 22:00), prior to treatment initiation/escalation in RA patients with active disease. In a pilot study, we prospectively examined for possible associations between these measurements and treatment responses at two years in those 24 patients who were under optimal management according to standard protocols.

Results: The ratio of circulating cortisol/ACTH, as well as diurnal cortisol production at baseline were significantly lower in patients with moderate response or no response to treatment (7/24, 29%), than in those having disease remission at two-years (17/24, 71 %). Baseline diurnal-cortisol-production greater than 81.3 (calculated as area-under-the-curve) could predict remission at 24 months with 86% specificity and 65% sensitivity, independently of age, sex and baseline CRP levels (p=0.03).

Conclusions: Further studies to confirm that lower diurnal cortisol production prior to treatment initiation/escalation in patients with active RA may predict inadequate corresponding responses in the long-term, are warranted.

Background: Sjögren's syndrome (SS) may accompany psoriatic arthritis (PsA), if ever, and its presence during psoriasis (PsO) is rare. Recent observations of an association with predominantly axial forms of PsA are intriguing. Whether SS complicates PsO and PsA to the same extent is not known.

Objectives: To investigate the frequency of SS in patients with PsO and PsA, and elucidate disease features associated with SS. Methods: In this retrospective analysis, we collected the data about previously recorded in the follow-up files of PsO and PsA patients. We classified patients who met the 2002 American-European Sjögren's syndrome Consensus Group Criteria (AECG) or the 2016 ACR-EULAR Classification Criteria for primary Sjögren's Syndrome as SS. Frequency rates of SS in PsA patients and in PsO patients without arthritis were compared and SS characteristics were described.

Results: Of the 184 PsO patients, 112 had PsA and 72 PsO alone. A substantial proportion of PsA patients had SS. This association was not present in the PsO group (PsA: 20(%17.9), PsO: 0(%0), p<0.001). Gender distribution, mean age, and PsO onset age were similar in both. Symmetric polyarticular involvement was rare, and most PsO patients with arthritis were ANA positive at a serum dilution level of 1:320 and beyond.

Conclusions: SS seems to be part of the clinical picture in a substantial proportion of PsA patients, and its absence in PsO implies that SS may be arthritis associated. Whether the type of arthritis is critical in developing SS in PsA deserves further studies with large populations.

Systemic Lupus Erythematosus (SLE) is an autoimmune disease with varied organ involvement. Gastrointestinal (GI) involvement in SLE is common, with oral mucosal lesions being the most frequently seen. Acute abdomen as a surgical emergency in SLE patients can have multiple causes. Lupus enteritis (LE)/Intestinal pseudo-obstruction (IPO) can present as acute abdomen, thereby requiring surgical reference. We hereby present a case of SLE presenting with acute abdomen due to LE/IPO, which prompted the surgeon for surgical intervention. Timely diagnosis and prompt response to steroids lead to resolution of symptoms in our case, and avoided unnecessary surgical exploration and further complications. One should keep a high degree of suspicion while managing SLE patients presenting with acute abdominal pain. Our case underscores the clinical significance of discerning lupus-related GI complications, aligning with existing literature emphasising the need for heightened clinical suspicion in acute abdominal scenarios in SLE. In this case, the patient's acute abdomen prompted surgical consideration, reflecting the challenges in differentiating lupus-related complications from surgical emergencies.

Background: Inflammatory bowel disease is closely associated with extraintestinal manifestations. Among them, joint involvement and enthesitis are the most frequent resembling a spondyloarthropathy. Enthesitis may be clinically silent in a high proportion of these patients without any clinical signs or a diagnosis of spondyloarthritis.

Objectives: To evaluate, with the use of musculo-skeletal ultrasound, the frequency and location of musculoskeletal manifestations in patients with inflammatory bowel disease.

Methods: Fifty patients with a definite diagnosis of inflammatory bowel disease were prospectively recruited and clinically evaluated by a rheumatologist for relevant musculoskeletal symptoms. All of the patients underwent a thorough musculo-skeletal ultrasound examination of both knees, patellae, as well as upper and lower limbs, in order to identify synovitis, and enthesitis. Musculo-skeletal ultrasound examination was performed at 12 entheses. The ultrasound abnormalities were scored according to the Madrid Ankylosing Spondylitis Enthesitis Index. Results: The musculo-skeletal ultrasound examination performed, revealed the presence of synovitis in 24% and enthesitis in 64% of these patients. More specifically, in 6.3% of the "Quadriceps tendon", in 40.6% in the "Achilles tendon", in 40.6% in the "Quadriceps and Achilles", in 9.4% in the "Triceps, Quadriceps and Achilles' and at a rate of 3.1% in the 'Patella'.

Conclusions: The musculo-skeletal ultrasound verified that a significant percentage of the patients exhibit some type of "musculoskeletal manifestations", with enthesitis to be the most common of them in patients suffering from inflammatory bowel disease.

Objectives: Central aim of this research was to investigate the experience of parents with a child diagnosed with Juvenile Idiopathic Arthritis (JIA) and the interplay of JIA, family dynamics, everyday life, and the developing coping mechanisms to deal with potential life changes.

Methods: A qualitative psychology-based research methodology was applied. Data were collected through online in-depth semi-structured interviews with parents of one offspring with JIA. The anonymised interview transcripts were analysed following the principles of interpretative phenomenological analysis. Nine parents (M:F, 3:6) aged 39-51 years (mean=47), were included in the study with their children having the diagnosis of different JIA subtypes.

Results: JIA appears to have led our sample to the creation of strong bonding between parents, siblings, and the patient (9/9). Additionally, JIA altered the family members' daily life by shifting their focus on the disease management (9/9). The upcoming stress led parents to develop a variety of coping mechanisms (9/9) with 3/9 parents sought reliable information from health professionals, 7/9 looked for psychological support and 6/9 shared their experience with other JIA affected families. Impressively, 5/9 reported that JIA eventually has a positive impact on their family, with JIA minors presented as disciplined and empathetic fighters (4/9).

Conclusion: JIA was found to be a great challenge from the parents' perspective, especially during the post-diagnosis period. However, most of the interviewed family members gradually accommodated to JIA and acknowledged even its beneficial contribution.

Objectives: We analysed interventions related to TAK, their pre-treatment clinical and angiographic associations, and prognostic relevance from a large ambispective, monocentric cohort of TAK from India.

Methods: Information regarding endovascular or open surgical interventions (aortoplasty, nephrectomy for refractory hypertension) was retrieved from a cohort of patients with TAK. Demographic characteristics, clinical features, and angiographic involvement were compared between patients with TAK who had undergone interventions with the rest of the cohort using multivariable-adjusted odds ratios (OR, with 95% CI). Hazard ratios were used to compare the mortality rate among TAK who had undergone interventions.

Results: Among 238 patients with TAK in the cohort, 41(17.23%) had undergone 69 interventions related to TAK (64 endovascular procedures, one open surgical aortoplasty, 4 nephrectomies) across 55 sittings (a single intervention sitting in 31, two in seven, three in two, and four in one). The most common arterial territories undergoing intervention were the renal arteries (n=21), subclavian arteries (n=8), and descending thoracic aorta (n=6). Six patients with TAK required repeated interventions in the same arterial territories. Patients with TAK who underwent interventions more frequently had abdominal angina (OR 5.12, 95%CI 1.36-19.26), and less often had constitutional features (OR 0.39, 0.18-0.84) at presentation without significant differences in angiography. Survival was similar in TAK who had undergone interventions to those without (hazard ratio for mortality 0.91, 95%CI 0.23-3.55).

Conclusion: About one-sixth of our cohort of TAK had undergone interventions, most often endovascular interventions. One-fourth required multiple interventions. Survival was similar in TAK with or without interventions.