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Vasculitis 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.

Rheumatoid 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.

Immune-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.

Systemic Lupus Erythematosus is the hallmark of autoimmune diseases, being characterized by multiple organ involvements and immune abnormalities, amongst which the presence of antiphospholipid antibodies with or without specific clinical manifestations (vascular thromboses and pregnancy morbidities) has a significant impact on the disease course, both short and long term, causing the accumulation of irreversible damage. This study evaluates the aforementioned impact, highlighting the importance of very early screening for these antibodies.

Systemic sclerosis is a complex autoimmune disorder marked by heterogeneous clinical manifestations and variable disease course. We present the case of a patient with diffuse cutaneous systemic sclerosis with anti-PM/Scl antibodies and associated calcinosis cutis. Currently, there is no uniformly effective therapy for calcinosis, but in the present case study combined therapy (calcium channel blocker, colchicine, bisphosphonate and minocycline) showed a good outcome with significant clinical improvement. Calcinosis in patients with systemic sclerosis is relatively common and it represents a challenge that requires appropriate management.

Introduction. The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results have often been observed. Material and method. We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096 . We looked at sex influence on disease characteristics at baseline and then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. Results. 173 patients with SSc were available for the baseline analyses. In the longitudinal analysis after a mean follow-up of 3.5(±0.65) years, male sex was associated with a higher risk of scleroderma renal crisis (OR:9.45 (1.49 to 59.69); p = 0.004), digital contractures (OR:8.2 (3.1 to 21.9); p < 0.001), arrhythmias (OR: 3.37 (1.36 to 8.34); p = 0.006), pulmonary fibrosis (OR: 3.56, (1.51 to 8.41); p = 0.003), pulmonary hypertension (OR: 3.01 (1.19 to 7.59); p = 0.016), severe vascular involvement (OR:2.86, (1.22 to 6.73); p = 0.013) and low ventricular ejection fraction (OR: 2.84, (1.2 to 6.73); p = 0.014). Males had significantly reduced survival time after diagnosis (p = 0.004). The most frequent causes of death were scleroderma renal crisis in males and pulmonary hypertension in females. Conclusions. Although more common in women, SSc appears as strikingly more severe in men. Our results demonstrate a higher risk of severe organ involvement and poor prognosis in men. These results raise the point of including sex in the management and the decision-making process.

Objective. To evaluate sexual function in a cohort of men with rheumatic disease compared to healthy controls. Patients and methods. The study included 60 patients (ankylosing spondylitis, rheumatoid arthritis, systemic sclerosis, gout, psoriatic arthritis) and 60 healthy age-matched controls. Sexual function of patients and controls was evaluated by the Sexual Health Inventory for Men and a detailed questionnaire. Results. The mean age of study group was 45,26 (7.8) years. In 90% of the cases sexual life changed after diagnosis: only 1.67% described their sexual life as being very satisfying, while 21.67% as being satisfying, 68.33% as not satisfying and 8.33% as absent. 40% of the patients had diminished libido, significantly lower compared to the study group (p = 0.043). Most patients presented with mild erectile dysfunction (60%), while most of the systemic sclerosis patients presented with mild to moderate erectile dysfunction. Erectile dysfunction scores were significantly lower compared to controls (0.009). 55% of the patients reported no influence of the treatment on their sexual life, 38% of the patients reported that specific treatment improved their sexual life.There was no statistical difference between SHIM scores of patients using biological therapies and other drugs. Conclusions. This work suggests that men with rheumatic conditions have a worse sexual function in comparison with healthy control. However, specific disease parameters or treatments were not linked to sexual dysfunction.

Availability of biosimilar medicines is rapidly increasing, potentially leading to a wider prescription and a support for healthcare system costs. Despite efforts of patient and physician educational initiatives there still remains a reluctance in prescribing or administering this type of products. A multi-centric survey was conducted on 336 patients with rheumatoid arthritis (RA), spondyloarthritis (SpA), psoriatic arthritis (PsA) or lupus (SLE) on biologic treatment. Half of the patients stated never hearing of biosimilars and one third feared adverse events (infections, cancer). Another 20% felt insecure on drug tolerability or efficacy. However, the majority declared relying on their rheumatologist if prescribing a biosimilar. Lack of patient information should be accurately addressed by health organizations so that the choice of treatment is done knowingly.

Tumor 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.

The usefulness of capillaroscopy in the follow-up of scleroderma patients and the possible prognostic role for the appearance of visceral involvement is suggested by many authors but still under debate.The aim of this study was to assess the role of monitoring capillaroscopic abnormalities (qualitative and semiquantitative) in relation with parameters of interstitial lung involvement and pulmonary arterial hypertension(PAH). A strong correlation was identified between initial capillaroscopy scores and FVC (r=-.47, p=0.002), DLCO (r=- .51, p< 0.001) and sPAP (r=0.34, p1) was asociated with worsening of FVC (r=0.32,p

B 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.

Autoimmune rheumatic disorders have a multifactorial determinism, caused by various environmental factors acting on the individual’s genetic susceptibility, destabilizing the systems which regulate the immune response. Epidemiological and genetic investigations are very important to demonstrate the contribution of genetic factors to the development of these autoimmune diseases. The contribution of genetic factors in causing autoimmune diseases has been demonstrated by familial aggregation. Moreover, it was also quantified by determining heritability, expressing the proportion of genetic factors in the etiology. It is now clear that common genes underlie multiple autoimmune disorders.

We are presenting the case of a 48 years old female diagnosed at the age of 28 with diffuse cutaneous systemic sclerosis. During the course of disease, despite the vasodilatator treatments and immunosuppresion for visceral involvement, the patient developed multiple infected digital ulcers both in the upper limbs and in the lower limbs, which complicated with wet gangrene, requiring transmetatarsal amputations. At that time, a macrovascular disease or a prothrombotic condition were excluded. Patient also developed infected nondigital lower extremity ulcers, which slowly healed after Alprostadil treatment. The evolution of our patient‘s disease demonstrate that the management of scleroderma vasculopathy represent a challange and a multidisciplinary approach is needed.

Introduction. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) represent a group of conditions evolving with necrotizing inflammation in small and medium-sized blood vessels. AAV are composed of GPA (granulomatosis with polyangiitis, former Wegener’s granulomatosis), MPA (microscopic polyangiitis) and EGPA (eosinophilic granulomatosis with polyangiitis, former Churg-Strauss syndrome). AAV receive immunosuppressive therapy associated with a high risk of complications. Objective. The aim of this study was to characterize a single center cohort of AAV patients regarding clinical, biological and therapeutic features. Method. We realized a cross-sectional study by consequently enrolling all the patients registered with AAV diagnosis between 2009 and 2017 in Department of Rheumatology of “Sfânta Maria” Hospital. Demographic, diseaserelated and therapeutic-related parameters were collected. The data was extracted from the clinical files. Results. The study sample included 26 cases, 15 females and 11 males: 20 patients GPA, 4 MPA and 2 cases EGPA. Mean age at the time of diagnosis was around 48 but 12 patients presented delays between age at the onset and age at the time of diagnosis (the mean delay was 2 years). The most frequent clinical manifestation identified where pulmonary, musculoskeletal and renal. 15 patients had a diagnostic biopsy performed. ANCA detection revealed 16 cases of c-ANCA and 7 cases of p-ANCA and 11 patients presented other positive serology. A combination of glucocorticoids and cyclophosphamide was used in most of the cases for remission-induction treatment and the same scheme was used for relapse cases. For maintenance phase a combination of glucocorticoids and azathioprine was preferred. 13 patients (50%) developed treatment related complications. Conclusion. Most of the patients were diagnosed with GPA (20) and the least were diagnosed with EGPA (2). Biopsy was performed in 15 cases and it was mostly nasal. For remission-induction prevailed the combination of glucocorticoids and cyclophosphamide. Most treatment related complications were due to glucocorticoids administration. Osteoporosis was predominant.

Background. 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

Background/Objective. Disease activity in spondyloarthritis (SpA) is evaluated through conventional scores such as BASDAI or ASDAS. The aim of this study was to determine disease activity in SpA patients under biological therapy with further assessment on the quality of each activity indicator and their clinical value together with their impact on NSAID intake. Materials and methods. Over nine months (January–August 2015) we enrolled 100 patients with SpA on anti-TNF therapy (33% IFX; 35% ADL; 32% ETA). We collected demographic, clinical (BASDAI, ASDAS, PtGA) and laboratory data (ESR, CRP). Frequency of NSAID ingestion expressed as days per week was included in a questionnaire. Considering their biologic treatment, patients were divided into disease activity groups and sample characteristics were correlated and compared. Statistical analysis was performed with SPSS 20.0, using specific tests such as Pearson, Spearman or ANOVA. Results. Patients with active disease, stated by both activity scores had an increased NSAID intake. Out of 86% of patients with a BASDAI under 4, only 82.6% had an ASDAS-CRP lower than 1.3. BASDAI correlated to both ASDAS scores (r = 0.65 and 0.71, P