34 research outputs found
Interstitial lung disease in rheumatoid arthritis
No full textRheumatoid arthritis (RA) is a systemic inflammatory disorder mainly involving joints but almost 50% of the patients develop extra-articular manifestation, one of the most common being lung involvement. While essentially any of the lung compartments can be involved, interstitial lung disease (ILD) is associated with significant morbidity and mortality. In this review, we discuss main current concepts in the screening, diagnostic and monitoring strategies of RA-ILD as well as therapeutic recommendations
A Systematic Review of the Key Predictors of Progression and Mortality of Rheumatoid Arthritis-Associated Interstitial Lung Disease
No full textBackground: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is an important extra-articular manifestation of rheumatoid arthritis (RA). Identifying patients at risk of progression and death is crucial for improving RA-ILD management and outcomes. This paper explores current evidence on prognostic factors in RA-ILD. Methods: We conducted a systematic literature review to examine the impact of clinical, radiological, and histological factors on lung function decline and the survival of RA-ILD patients. We searched electronic databases, including Medline and EMBASE, from inception to date. The incidence and prognosis of predictors were qualitatively analyzed, and univariate results were combined when feasible. Following the “Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)” guidelines, our systematic literature review involved a five-step algorithm. Out of 2217 records, 48 studies were eligible. These studies reported various prognostic factors, including demographic variables, clinical risk factors, serum markers, and preexisting treatments. Results: Lung function declined over time in 1225 subjects, with significant variability in smoking history and radiological/pathological UIP patterns. Severe lung fibrosis and abnormal pulmonary function tests (PFTs) were key univariate prognostic indicators, while age at initial presentation, RA disease activity, predicted DLCO percentage, and UIP pattern were the most reliable multivariate risk factors for ILD progression. Age, male gender, disease duration, RA activity, acute phase reactants, and specific serum biomarkers (Krebs vin den Lungen 6, surfactant protein D, and interleukin 6) were significantly associated with all-cause mortality. Conclusions: RA-ILD is a severe complication of RA characterized by significant prognostic variability. Key prognostic factors include extensive fibrosis observed on imaging, a marked decline in lung function, high RA disease activity, and specific biomarkers. These factors can guide treatment strategies and improve patient outcomes
Innate Immunity in Systemic Sclerosis – Role of Toll-Like Receptors, Interferon, and the Potential Impact of Vitamin D
No full textSystemic sclerosis (SSc) is an autoimmune disease in which vascular damage and immune activation leads to excessive accumulation of extracellular matrix in the skin and internal organs. Although the focus has been on adaptive immunity in SSc, recent data suggest that innate immunity is critically important. The innate immune system, the first-line barrier against pathogens, modulates mechanisms which activate adaptive immunity. Dysregulation of the innate immune system and toll-like receptors (TLRs) may link immune abnormalities and fibrosis in SSc. TLR signalling pathways might induce production of Type I interferon (IFN) and other cytokines, and represent one of the mechanisms that initiate and develop autoimmunity and subsequent fibrosis. Vitamin D displays many immunomodulatory effects on both innate and adaptive immune responses. Active vitamin D will produce signals via vitamin D receptor and influences TLR stimulation, IFN response, and antimicrobial peptide production. Vitamin D deficiency has been associated with many autoimmune disorders, and can influence clinical phenotype and immune disorders in SSc patients
Statins and muscle – friends or foes? Discussions based on a case report
No full textImmune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement Anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) myopathy is a distinct subtype of INMN, often associated with statin exposure. Treatment of IMNM consists of immunosuppression with steroids, steroid-sparing agents, intravenous immune globulin and/or biologics. We present here a case of anti-HMCGR-associated IMNM and a brief review the pathophysiology, diagnosis and treatment to increase physician awareness of this rare and debilitating condition
Neurological manifestations in systemic lupus erythematosus: A case with an individualized therapeutic approach
No full textVasculitis occurs frequently in systemic lupus erythematosus (SLE) patients with an active disease and poor prognosis. Nervous system vasculitis – specifically, multiple mononeuropathy, seizures, and transverse myelitis – is among the most severe complications in SLE that need timely aggressive therapy. Unfortunately, no robust literature is available to guide their management, and therapeutic recommendations are commonly based upon other autoimmune conditions or case reports, case series, and expert opinions. We hereby present a case of a 27-year-old female patient with SLE that sequentially developed an asymmetric distal axonal sensorimotor neuropathy in her limbs and digital ischemic lesions of the hands, that progressed despite maximal treatment with standard therapies, thus requiring several sessions of plasmapheresis
Connections between rheumatology and oncology – discussions based on a case report
No full textTumor necrosis factor (TNF) inhibitors are commonly utilized medications for the treatment of immune mediated conditions. Based on recent data from most registries there is no conclusive evidence for an increased risk of solid tumours or lymphoproliferative disease linked with biologic therapy, but on-going vigilance is required.
We present a case of ankylosing spondylitis with extraarticular manifestations with a premalignant condition treated with TNF inhibitors, which developed a basal cell cancer, was switched on secukinumab and back again on antiTNF due to severe panuveitis
Systemic sclerosis and primary biliary cholangitis : Longitudinal data to determine the outcomes
No full textBackground: Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking. Aims: To describe the systemic sclerosis–primary biliary cholangitis phenotype, including baseline characteristics and outcomes. Methods: We performed a multicentre the European Scleroderma Trials and Research Group study of systemic sclerosis patients with primary biliary cholangitis or with primary biliary cholangitis–specific antibodies, matched with systemic sclerosis controls free from hepatobiliary involvement matched for disease duration and cutaneous subset. Data were recorded at baseline and at the last available visit. Results: A total of 261 patients were enrolled (115 primary biliary cholangitis–systemic sclerosis, 161 systemic sclerosis). At baseline, systemic sclerosis–primary biliary cholangitis patients had a higher prevalence of anti-centromere antibodies (p = 0.0023) and a lower prevalence of complete absence of digital ulcers. The milder vascular involvement was confirmed at follow-up when crucial differences emerged in the percentage of patients experiencing digital ulcers; a significantly higher number of patients who never experienced digital ulcers were observed among primary biliary cholangitis–systemic sclerosis patients (p = 0.0015). Moreover, a greater incidence of pulmonary arterial hypertension (p < 0.001) and of conduction blocks (p = 0.0256) was observed in systemic sclerosis patients without primary biliary cholangitis. Patients with primary biliary cholangitis had higher levels of liver enzymes at baseline than systemic sclerosis patients; a significant decrease in liver enzymes was observed at follow-up. Out of 18 patients with cholangitis, one received a liver transplant at follow-up. Conclusion: Our data show that systemic sclerosis–primary biliary cholangitis exhibit a mild systemic sclerosis and primary biliary cholangitis phenotype with outcomes being in general favourable
Is there an early ultrasonographic pattern in salivary glands in both primary and secondary Sjögren syndrome?
No full textBackground. Sjögren syndrome (SS) is a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs. Ultrasonography (US) demonstrates specificity and sensibility in major salivary glands (SG) evaluation. Recent data confirm US might be used as primary evaluation technique for its ability to show structural alterations of parenchyma (1).
Objective. To assess the gray scale (GS) parenchymal inhomogeneity of major SG in patients with established primary and secondary SS and correlate with clinical and biological data. Methods. Consecutive patients with SS were recruited and SG US was performed. Inhomogeneity of glandular parenchyma was quantified binary on each gland. ESSDAI and ESSPRI scores were calculated. Statistics was
performed with SPSS. Results. Twenty one (42.85% primary SS, 90.47% female) consecutive patients were included. Mean age was 53.66+/-12.99 years and disease duration 5.33+/-3.74 years. Antibody SSA/SSB presence was found in 85.7% (18/21). ESSDAI mean was 8.67+/-8.9 (0-29), ESSPRI 10.13+/-5.59(0-20). There were no differences regarding ESSDAI and ESSPRI in the two groups (primary and secondary SS). Right parotid gland showed alterations in 71.4% patients (77% with primary SS, 66% with secondary SS). Frequently inhomogeneity was found in all major SG (33%, 22% left and right submandibular, 77%, 44.4% left and right parotid glands) in primary SS. Both
submandibular glands were symmetrically involved (p<0.02). Duration of disease was negatively correlated to inhomogeneity of right parotid gland (p<0.02).
Conclusion. Inhomogeneity in major SG in GS US was found in the majority of patients with primary and secondary SS. The symmetrical involvement of submandibular glands was significant. The inhomogeneity appears in the
early period of diagnosis. No major differences were found between two groups
ULTRASONOGRAPHIC SALIVARY GLAND RESPONSE TO RITUXIMAB IN SECONDARY SJÖ GREN’S SYNDROME
No full textB cell hyper-reactivity implied in the pathogenesis of Sjö gren’s syndrome (SS) justifies the therapy that involves B
cell depletion. Ultrasound of salivary glands demonstrated alterations during disease progression.
Objective. To evaluate changes in salivary gland parenchyma with ultrasound after rituximab treatment in patients’ with secondary SS (sSS).
Methods. 7 patients evaluated at baseline (under treatment with rituximab) and after 6 months, underwent ultrasonography of major salivary gland (submandibular and parotid). Clinical data as dryness, pain and diseases
activity evaluated with ESSDAI were registered. Salivary gland ultrasound (SGUS) was performed to asses echogenity (using a semiquantitative score from 0-4, with improvement defined as an at least 1 point decrease), size of gland and glandular borders.
Results. Of 7 patients, 14% had clinically detectable bilateral parotid tumefaction at baseline. 6 patients (85.71%) showed ultrasonographic alterations at baseline. Parotid parenchyma echostructure improved in 42.85% of patients versus 14.28% in control group (p=0.05). At the submandibular glands, both submandibular glands showed changes in 28.57% of patients, while control group showed no changes. 42.85% patients showed parotid tumefaction which changed in 66.6% of patients at 6 months.
Conclusion. Ultrasonography showed improvement in salivary gland echostructure in secondary Sjogren Syndrome with rituximab
