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PREVALENZA, CORRELATI CLINICO-SEROLOGICI E CARATTERISTICHE RADIOLOGICHE DELL'INTERSTIZIOPATIA POLMONARE IN UN'AMPIA COORTE DI PAZIENTI AFFETTI DA SINDROME DI SJOGREN
No full textIntroduzione
L’interstiziopatia polmonare (IP) rappresenta la complicanza polmonare più frequente e grave nella sindrome di Sjögren primaria (pSS), con una prevalenza che varia dal 6 al 70% dei pazienti, morbidità e mortalità significative. Tuttavia pochi studi hanno indagato la frequenza di IP in pSS, e in genere si tratta di studi retrospettivi su un numero di pazienti limitato e gravati da numerosi bias. I pattern non fibrotici, principalmente la polmonite interstiziale non specifica (NSIP), come sottotipo di ILD più comune, e la polmonite organizzante (OP), sono descritti come i più frequenti nella pSS-IP, in contrasto a quelli fibrotici, come l'NSIP fibrotico, OP fibrotica e polmonite interstiziale usuale (UIP). La polmonite interstiziale linfocitaria è molto specifica della pSS ma si verifica solo in pochi casi.
Questa complicanza è spesso sottovalutata e, al giorno d'oggi, non ci sono studi clinici randomizzati controllati a supporto delle linee guida terapeutiche.
Pertanto attualmente è necessario attuare studi prospettici per chiarire alcuni punti cruciali come la prevalenza della ILD, le sue caratteristiche cliniche e le caratteristiche radiologiche.
Obiettivi
Scopo di questo studio è valutare la prevalenza di ILD nei pazienti con pSS, per valutare le caratteristiche cliniche e radiologiche.
Metodi
Tutti i pazienti consecutivi affetti da pSS saranno sottoposti a screening per la ricerca di segni o sintomi che suggeriscono un coinvolgimento polmonare (tosse secca e/o dispnea progressiva, crepitii a velcro rilevati tradizionalmente e digitalmente, ecc.). L’IP sarà sospettata sulla base di parametri clinici e auscultatori e i soggetti con sospetto di IP saranno sottoposti a tomografia computerizzata ad alta risoluzione (HRCT torace), il gold standard per la diagnosi di IP. I pazienti saranno sottoposti a HRCT anche in presenza di sospetta IP in una radiografia del torace.
Per quanto riguarda la valutazione della prevalenza, tutti i soggetti saranno valutati in modo trasversale.
La prevalenza verrà fornita insieme ai rispettivi intervalli di confidenza al 95%.
Le immagini HRCT saranno rivalutate da un radiologo esperto, al fine di confermare la presenza di IP e classificarla secondo le attuali classificazioni radiologiche, in particolare discriminando tra pattern radiologici fibrotici e non fibrotici.
Risultati
Un totale di 265 soggetti sono stati coinvolti nel presente studio (241 femmine e 24 maschi). Tra questi, 70 hanno presentato IP (26,41%). Quattordici soggetti erano maschi e 56 femmine. Quattro pazienti sono in attesa di una TAC in conseguenza al rilevamento di crepitii in velcro.
Conclusioni
Nonostante le osservazioni precedenti, i nostri dati suggeriscono un'elevata prevalenza di pattern di IP fibrosante nei pazienti con pSS.
Rispetto agli attuali dati di prevalenza, il nostro studio potrebbe evidenziare una prevalenza ancora più elevata di IP clinicamente significativa.
Le HRCT eseguite nell'ultimo anno sono ancora in fase di valutazione da parte del radiologo esperto; dati preliminari suggeriscono una possibile diversa distribuzione nei sottoinsiemi di ILD, con un'elevata prevalenza di pattern IP fibrosante nei pazienti con pSS.Background
Interstitial lung disease (ILD) represents the most frequent and serious pulmonary complication in primary Sjögren's syndrome (pSS), with a prevalence ranging from 6 to 70% of patients, significant morbidity and mortality. However, few studies have investigated the frequency of ILD in pSS, generally referred to retrospective studies with small series of patients and numerous biases. Non-fibrosing patterns, primarily non-specific interstitial pneumonia (NSIP), as the most common ILD subtype, and organizing pneumonia (OP), are described as the most frequent in pSS-ILD, in opposition to fibrotic ones, such as fibrotic NSPI, fibrotic OP and usual interstitial pneumonia (UIP). Lymphocytic interstitial pneumonia is highly typical for pSS but it occurs only in a few cases.
This complication is often underrated and, nowadays, there are no randomized controlled clinical trials to support therapeutic guidelines.
Therefore, there is an unmet need of prospective studies to clarify some crucial points such as the prevalence of ILD, its clinical and radiological features.
Objectives
Aim of this study is to evaluate prevalence of ILD in patients with pSS and to assess clinical radiological features.
Methods
All consecutive pSS patients will be screened for signs or symptoms suggesting pulmonary involvement (dry cough and/or progressive dyspnea, Velcro crackles traditionally and digitally detected, etc.). An ILD will be suspected on the basis of clinical and auscultatory parameters and subjects with suspicion of pulmonary disease will undergo a high-resolution computed tomography (HRCT), the gold standard for the diagnosis of ILD. Patients will undergo HRCT even in presence of suspected ILD in a chest x-ray.
As regard the assessment of prevalence, all subjects will be evaluated cross-sectionally. Prevalence will be provided along with their 95% confidence intervals.
The HRCT images will be re-evaluated by an expert radiologist, in order to confirm the presence of ILD and to classify it according to the current radiological classifications, in particular discriminating between fibrotic and non-fibrotic radiological patterns.
Results
A total of 265 subjects have been involved in the present study (241 females and 24 males). Among them, 70 showed ILD (26,41%). Fourteen subjects were males and 56 females. Four patients are awaiting a CT-scan after the detection of Velcro-crackles.
Conclusion
Despite previous observations, our data suggest a high prevalence of fibrosing ILD pattern in pSS patients.
In comparison to current prevalence data, our study could evidence an even more high prevalence of clinically significant ILD. HRCTs performed during the last year are still under evaluation by the expert radiologist; preliminary data suggest a possible different distribution in ILD subsets, with a high prevalence of fibrosing ILD pattern in pSS patients
Vasculitis in a patient with mixed cryoglobulinemia treated with rituximab biosimilar CT-P10: a case report
No full textRituximab represents a milestone in the treatment of mixed cryoglobulinemic vasculitis. Despite usually well-tolerated, rituximab may induce different types of adverse drug reactions, including exacerbation of vasculitis. Rituximab biosimilar have been recently approved in Europe in the treatment of rheumatoid arthritis, but no data are available about effectiveness and safety of rituximab biosimilar in the treatment of mixed cryoglobulinemic vasculitis. We describe a severe skin vasculitis reactivation in a patient affected by rheumatoid arthritis and mixed cryoglobulinemic vasculitis treated with rituximab biosimilar. After 7 days from the first infusion, a severe purpuric rash at lower limbs appeared, that resolved in about 2 weeks with high dose-corticosteroid. Rituximab-induced vasculitis has also been described since 2001, but its pathophysiology is still controversial due to the anecdotical descriptions in literature and the variability of the time between the rituximab infusion and the onset of skin lesions. Up to date, this is the first report describing a vasculitic flare in a patient affected by mixed cryoglobulinemic vasculitis treated with rituximab biosimilar
Haematological Malignancies in Systemic Sclerosis Patients: Case Reports and Review of the World Literature
Full text linkBackground.The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reports
and cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. We reviewed the literature to
better describe SSc patients with haematological malignancies. Methods. SSc cases complicated by haematological malignancies
described in the world literature were collected; other 2 cases referred to our centre were reported. Results. One hundred-thirty SSc
subjects were collected from 1954 up to date. The mean age of patients at cancer diagnosis was 56.1 ± 16.7 years; 72% of patients
were females. In 60% of cases, the diagnosis of haematological malignancy was described within 5 years of SSc diagnosis. In 7.8% of
cases, coexistence of Sj ̈ogren’s syndrome or other autoimmune disorders was cited. Sixty-six cases with lymphoma (in the majority
of cases B-cell neoplasms), 28 with leukaemia (chronic lymphocytic form in 9), 14 with multiple myeloma plus one solitary IgM
plasmocytoma, and 16 with myeloproliferative disorders were found. No specific SSc subsets seem to be related to haematological
malignancies. Conclusions. We remarked the importance of clinical work-up in SSc, in order to early diagnose and treat eventual
occult haematological malignancies, especially during the first years of the disease
Efficacy and safety of rituximab in the treatment of connective tissue disease-related interstitial lung disease
No full textInterstitial lung disease (ILD) represents a severe pulmonary complication of connective tissue diseases, rheumatoid arthritis (RA), and antineutrophil cytoplasmic antibody-associated vasculitis. Treatment of ILD, mainly based on immunosuppression, remains challenging. Rituximab (RTX), a monoclonal antibody binding to CD20, is considered a valuable therapeutic choice in cases of refractory ILD. Here, we review the available efficacy and safety data on the use of RTX in the treatment of rheumatic disease-related ILD. Despite controversial efficacy data, RTX seems to be able to stabilize or improve ILD related to RA and antisynthetase syndrome and in established and severe ILD complicating systemic sclerosis. Fewer data are available regarding ILD related to Sjögren syndrome, systemic lupus erythematosus, and antineutrophil cytoplasmic antibody-associated vasculitis. To date, few prospective studies are available and randomized trials are still ongoing with the purpose of exploring the role of RTX in this condition, including the supposed relationship between efficacy and ILD radiologic patterns and safety data, up to now derived mainly from RA studies. Despite an overall acceptable safety profile, concerns remain regarding an increased infectious disease risk in patients with ILD as well as possible lung toxicity and the increased rate of immune-mediated reactions in patients with connective tissue diseases. In conclusion, RTX is a relevant therapeutic option for rheumatic disease-related ILD despite the existing uncertainties; ongoing trials are expected to clarify its use
Rheumatoid arthritis extra-articular lung disease: new insights on pathogenesis and experimental drugs
No full textIntroduction: Pulmonary involvement is one of the most common extra-articular manifestations of rheumatoid arthritis (RA), a systemic inflammatory disease characterized by joint swelling and tenderness. All lung compartments can be interested in the course of RA, including parenchyma, airways, and, more rarely, pleura and vasculature. Areas covered: The aim of this paper is to review the main RA lung manifestations, focusing on pathogenesis, clinical and therapeutic issues of RA-related interstitial lung disease (ILD). Despite an increasing number of studies in the last years, pathogenesis of RA-ILD remains largely debated and the treatment of RA patients with lung involvement is still challenging in these patients. Expert opinion: Management of RA-ILD is largely based on expert-opinion. Due to the broad clinical manifestations, including both joints and pulmonary involvement, multidisciplinary discussion, including rheumatologist and pulmonologist, is essential, not only for diagnosis, but also to evaluate the best therapeutic approach and follow-up. In fact, the coexistence of different lung manifestations may influence the treatment response and safety. The identification of biomarkers and risk-factors for an early identification of RA patients at risk of developing ILD remains a need that still needs to be fulfilled, and that will require further investigation in the next years
Procedural pain management in the treatment of scleroderma digital ulcers
No full textObjective: Digital ulcers (DU) may develop in half of systemic sclerosis (SSc) patients; they are often resistant to treatments. Deep wound debridement is crucial for DU healing, but very difficult to carry out without adequate procedural pain management. Here, we report the results of our experience on procedural pain management in scleroderma DU. Methods: The study included 51 DU observed in 32 consecutive SSc patients; procedural pain was treated following a definite schedule: local lidocaine and prilocaine (25 mg of either agent per gram of cream, EMLA 5%) were initially used in all cases, followed by local and oral morphine, according to the severity of pain scored on a 10 cm visual analogue scale (VAS). Results: At baseline, higher pain VAS was recorded in more severe (p=0.0001) and/or infected DU (p=0.0001). Good compliance to DU debridement was observed in patients with mild pain (VAS ≤4) treated with only EMLA, and in 5 cases with moderate-severe pain (VAS >4) at baseline. While, the majority of DU with moderate-severe pain (34/39) needed a combined therapy with EMLA and local morphine (8/34) or with EMLA, local and oral morphine (26/34). On the whole, pain management during DU debridement required only EMLA application in 33% of cases, EMLA plus local morphine in 16%, while combined EMLA, local and oral morphine were necessary in 51%, generally with more severe and/or infected lesions. Conclusion: The present study showed valuable control of procedural pain during DU debridement with sequential, combined analgesic treatment
Breast cancer in systemic sclerosis: Results of a cross-linkage of an Italian Rheumatologic Center and a population-based Cancer Registry and review of the literature
No full textOBJECTIVE: Increased frequency of few types of cancer in systemic sclerosis (SSc) has been reported in the literature; in particular, breast carcinoma has been proposed as one of the most frequent malignancy in SSc patients, even though data are not univocal. The aim of the present study was to retrospectively evaluate the prevalence of breast cancer in our SSc series, compared with sex-/age-matched general population of the same geographical area, and the possible correlations with SSc features, including X-ray exposure for clinical investigations. A review of the world literature about this topic was also done.
METHODS: Clinical records of 318 consecutive SSc patients, 31 M and 287 F, age 51.5±14.5 SD years, disease duration 10±6.5 SD years, referred to our Rheumatology Unit between January 2002 and December 2012 were evaluated.
RESULTS: Twelve (3.8%) cases of breast cancer were recorded, including 11/287 females (3.8%) and 1/31 (3.2%) male patients. Considering the subgroup of 202 SSc patients resident in the Province of Modena compared with data of the local Tumor Registry, the incidence of breast cancer observed in our SSc series is significantly higher than expected (SIR 2.1; 95% interval of confidence: 1.13-3.90; p<0.01). On the whole, the comparison between SSc patients with cancer and those without did not show any significant differences with regard to SSc clinical features, including the X-ray exposure. Of note is the relatively shorter disease duration at the time of breast cancer detection (median 2.5years, range 1-21; disease duration of mean 10±6.5 SD years in the entire cohort). The review of the literature revealed that the observed incidence of breast cancer in our case series is comparable to the few studies reporting the highest percentages of this malignancy.
CONCLUSIONS: A significant increase of breast cancer incidence compared to sex-age-matched general population from the same geographic area was observed. Moreover, a close temporal relationship between SSc and breast cancer onset was found, independently from clinical, serological, and instrumental features of SSc. The possible pathogenetic link between this systemic autoimmune disease and complicating breast cancer, as well as the results of previous studies, are discussed
Radiological thymus alterations in systemic sclerosis: our experience and a review of the literature.
No full textObjective. Thymus alterations have been related to several autoimmune disorders. In particular, previous studies identified a significant frequency of gland abnormalities by chest high-resolution CT (HRCT) in SSc patients. In this study we aimed to investigate the prevalence of radiological thymic alterations and their correlation with clinical and serological features in a large SSc series.Methods. We retrospectively evaluated thymic shape on CT scans of 200 consecutive, unselected SSc patients aged over 30 years The presence of radiological abnormalities, i.e. enlarged gland >13 mm or nodular lesions >7 mm, was correlated with SSc clinico-serological features. Moreover, the patients were also classified using a second thickness cut-off of 7 mm in order to identify incomplete thymic involution.Results. Twenty-four of 200 (12%) SSc patients presented an abnormal thymus at HRCT, including hyperplasic (19/24) and nodular (5/24) glands. Otherwise, using the cut-off of 7 mm for gland thickness and excluding subjects with nodular thymus, 50/195 (25.6%) patients presented an incomplete thymic involution. Thymic radiological alterations are significantly correlated with younger age and diffuse cutaneous SSc. Moreover, an abnormally enlarged thymus tended to be more common in patients with shorter disease duration.Conclusion. The present report on a large series of SSc patients further reinforces previous data present in the literature that includes other cohort studies and a number of anecdotal observations. Even though the actual role of thymus radiological abnormalities remains unclear, possible involvement of the gland in the early phase of immune-mediated SSc pathogenesis might be supposed
Efficacy and safety of mycophenolate mofetil in the treatment of rheumatic disease-related interstitial lung disease: a narrative review
Full text linkMycophenolate mofetil (MMF) is an antimetabolite with a potent inhibitory effect on proliferation of T and B lymphocytes used since the early 1990s for the prevention of acute allograft rejection after organ transplant. MMF is also widely used for the treatment of a variety of rheumatic diseases (RDs) and their pulmonary involvement. Interstitial lung disease (ILD) is a heterogeneous group of progressive fibrotic diseases of the lung, which is often secondary to RD and represents a major cause of morbidity and mortality. MMF is considered the main alternative to cyclophosphamide as a first-line agent to treat RD-related ILD or as possible maintenance therapy after cyclophosphamide, with a lower rate of side-effects. However, as for other immunosuppressive agents, the use of MMF in RD-ILD is supported by poor scientific evidence. In this narrative review, we describe the available data and recent advances on the effectiveness and safety of MMF for the treatment of ILD related to RD, including rheumatoid arthritis, systemic sclerosis, primary Sjögren syndrome, systemic lupus erythematosus, idiopathic inflammatory myopathies, undifferentiated connective tissue disease, interstitial pneumonia with autoimmune features and antineutrophil cytoplasmic antibody-associated vasculitis
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