Frequency Of Lung Involvement Research Articles

#2700 Serum C3/C4 levels in renal antineutrophil cytoplasmic antibody-associated vasculitis: predictors of lung disease and worse prognosis

Abstract Background and Aims Complement activation, specially the alternative pathway, has been implicated in the development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This was further corroborated with the efficacy of Avacopan, a selective C5a receptor inhibitor used as a corticosteroids reduction strategy in the treatment of AAV [1]. Low complement factor 3 (C3) levels without real hypocomplementemia are predictors of prognosis in AAV [2]. Low C4 levels have not yet been associated with worst outcomes. We aimed to evaluate the association between circulating C3 and C4 levels at diagnosis of AAV with clinical and biochemical features and with relevant outcomes: relapse, dialysis and death. Method This is a retrospective study including 63 patients with AAV with renal involvement followed between 2003-2023, with C3 and C4 levels measurements at diagnosis. Data was recorded from clinical registries. Patients were grouped according to the median C3 and/or C4 levels, as previously performed [2]. Groups were compared using the chi-square test for categorical variables and non-parametric tests for continuous variables. We created a logistic regression model for the outcomes, adjusting for covariates. Results Mean age at diagnosis was 61.1 ± 13.4 years old and most patients had ANCA MPO specificity (69.8%, n = 44). Only 3 patients had low C3 levels (< 83 mg/dL) and C4 hypocomplementemia (<12 mg/dL) was present in other 3 patients. Median C3 levels were 127.5 mg/dL (107.3-139.0) and median C4 levels were 29.5 mg/dL (22.0-35.0). Groups were defined according to these values: low C3 levels (C3 <127.5 mg/dL, n = 31), low C4 levels (C4 <29.5 mg/dL, n = 31) and low C3 and/or C4 levels, when either of these conditions were met (n = 40). There was a positive correlation between C3 and C4 levels (r = 0.57, p < 0.001). Patients with low C3 levels had more frequent lung involvement than patients with higher C3 levels (71% vs 31.3%, p = 002), as well as alveolar hemorrhage (38.7% vs 12.9%, p = 0.020). Regarding the outcomes, there was a tendency for more patients requiring acute dialysis (first month) and progressing to dialysis during follow-up in the low C3 subgroup. Relapse and death rates were similar between the two groups. Low C4 levels were associated with older age (75 ± 11.8 vs 67.5 ± 13.7 years, p = 0.043), higher relapse rates (50% vs 25.8%, p = 0.05) and faster progression to dialysis (1.0 (1.0-5.0) vs 9.5 (1.8-54) months, p = 0.034). When combining low C3 and/or C4 levels, lung involvement was again more frequent in patients with lower complement levels (62.5% vs 30.4%, p = 0.014). Time to dialysis was lower and relapse rates were higher in the subgroup with low complement levels, but not statistically significant. No association with death was found. Interestingly, acute dialysis requirement was more prevalent in the low C3/C4 levels subgroup (32.5% vs 8.7%, p = 0.033). After adjustment for baseline renal function, low C3/C4 levels showed an increase of 7 times the odds of a patient requiring acute dialysis (OR 6.98; 95% CI 1,196-40,839, p = 0.031, Table 1). Low C3/C4 levels independently predicted the odds of lung disease in AAV (OR = 5,069; 95% CI 1,475-17,612 p = 0.010, Table 2). Conclusion Complement activation in AAV has been previously associated to renal outcomes, but our results also highlight a possible role of complement in the pathophysiology of lung disease. Low C4 levels were associated with higher relapse rates and faster progression to dialysis, revealing that C4 activation impacts outcomes in AAV and that both classic and alternative complement pathways are involved. Combining low C3 and/or C4 levels identified patients at higher risk of acute dialysis requirement, independently of renal function at admission. Further insights in the specific effects of C3/C4 are needed to stipulate the value of monitoring and targeting complement factors in AAV.

Prognostic value and predictors of the alteration of the diffusing capacity of the lungs for carbon monoxide in systemic lupus erythematosus.

SLE is a systemic autoimmune disease characterized by heterogeneous manifestations and severity, with frequent lung involvement. Among pulmonary function tests, the measure of the diffusing capacity of the lungs for carbon monoxide (DLCO) is a noninvasive and sensitive tool assessing pulmonary microcirculation. Asymptomatic and isolated DLCO alteration has frequently been reported in SLE, but its clinical relevance has not been established. This retrospective study focused on 232 SLE patients fulfilling the 2019 EULAR/ACR classification criteria for SLE. Data were collected from the patient's medical record, including demographic, clinical and immunological characteristics, while DLCO was measured when performing pulmonary function tests as part of routine patient follow-up. At the end of follow-up, DLCO alteration (<70% of predicted value) was measured at least once in 154 patients (66.4%), and was associated with a history of smoking as well as interstitial lung disease, but was also associated with renal and neurological involvement. History of smoking, detection of anti-nucleosome autoantibodies and clinical lymphadenopathy at diagnosis were independent predictors of DLCO alteration, while early cutaneous involvement with photosensitivity was a protective factor. DLCO alteration, at baseline or any time during follow-up, was predictive of admission in intensive care unit and/or of all-cause death, both mainly due to severe disease flares and premature cardiovascular complications. This study suggests a link between DLCO alteration and disease damage, potentially related to SLE vasculopathy, and a prognostic value of DLCO on death or intensive care unit admission in SLE.

Change in the Clinical Picture of Hospitalized Patients with COVID-19 between the Early and Late Period of Dominance of the Omicron SARS-CoV-2 Variant.

This study aimed to compare the clinical picture of COVID-19 in the initial and later period of Omicron dominance and to identify populations still at risk. A retrospective comparison of the clinical data of 965 patients hospitalized during the early period of Omicron's dominance (EO, January-June 2022) with 897 patients from a later period (LO, July 2022-April 2023) from the SARSTer database was performed. Patients hospitalized during LO, compared to EO, were older, had a better clinical condition on admission, had a lower need for oxygen and mechanical ventilation, had less frequent lung involvement in imaging, and showed much faster clinical improvement. Moreover, the overall mortality during EO was 14%, higher than that in LO-9%. Despite the milder course of the disease, mortality exceeding 15% was similar in both groups among patients with lung involvement. The accumulation of risk factors such as an age of 60+, comorbidities, lung involvement, and oxygen saturation <90% resulted in a constant need for oxygen in 98% of patients, an 8% risk of mechanical ventilation, and a 30% mortality rate in the LO period. Multiple logistic regression revealed lower odds of death during the LO phase. Despite the milder course of infections caused by the currently dominant subvariants, COVID-19 prophylaxis is necessary in people over 60 years of age, especially those with comorbidities, and in the case of pneumonia and respiratory failure.

Pulmonary Manifestations In Patients With Rheumatoid Arthritis Visiting Tertiary Care Hospital.

Rheumatoid arthritis is a common problem in elderly individuals. It is reported that lung involvement in these patients is widely present. This study was done with the purpose to assess the burden and characteristics of lung involvement in rheumatoid arthritis patients attending rheumatology clinic. This descriptive cross-sectional study, in which data was retrospectively collected, was carried out from April 2019 to December 2020 at the Rheumatology Department of Jinnah Postgraduate Medical Centre (JPMC), Karachi, Pakistan. All adult rheumatoid arthritis individuals, irrespective of gender or duration of disease, were consecutively enrolled. Information regarding the baseline characteristics, medication history for rheumatoid arthritis, findings of immunological tests along with the frequency of lung involvement and its pattern were observed. Of 254 patients, the mean age was 37.46 ±12.39 years. Females were predominantly higher as compared to males, i.e., 232 (91.3%) vs. 22 (8.7%) respectively. Current smoking status was found positive in 7 (2.8%) patients. The mean disease duration was 4.41±3.96 years. Furthermore, frequency of pulmonary manifestation was observed in 45 (17.7%) patients. A significantly higher mean difference of age (p-value <0.001) and disease duration (p-value <0.001) was observed among patients with and without pulmonary manifestation. Moreover, a significant association of current smoker was also observed with pulmonary manifestation. A considerable number of patients with rheumatoid arthritis had pulmonary manifestation. Furthermore, a significant relationship was observed with age, duration of disease, and current smokers.

A Comprehensive Review of Sarcoidosis Treatment for Pulmonologists.

Due to frequent lung involvement, the pulmonologist is often the reference physician for management of sarcoidosis, a systemic granulomatous disease with a heterogeneous course. Treatment of sarcoidosis raises some issues. The first challenge is to select patients who are likely to benefit from treatment, as sarcoidosis may be self-limiting and remit spontaneously, in which case treatment can be postponed and possibly avoided without any significant impact on quality of life, organ damage or prognosis. Systemic glucocorticosteroids (GCs) are the drug of first choice for sarcoidosis. When GCs are started, there is a > 50% chance of long-term treatment. Prolonged use of prednisone at > 10 mg/day or equivalent is often associated with severe side effects. In these and refractory cases, steroid-sparing options are advised. Antimetabolites, such as methotrexate, are the second-choice therapy. Biologics, such as anti-TNF and especially infliximab, are third-choice drugs. The three treatments can be used concomitantly. Regardless of whether treatment is started, the clinician needs to organize regular follow-up to monitor remissions, flares, progression, complications, toxicity and relapses in order to promptly adjust the drugs used.

THU0153 TCZ MIGHT BE A RISK FACTOR FOR WORSENING OF ILD, PARTICULARLY OF CHRONIC ILD

Background:Interstitial lung disease (ILD), frequent lung involvement, determine the prognosis of patients with RA. The presence of ILD also influences the selection of RA therapy. However, it is not fully elucidated what groups of RA-ILD patients worsen ILD.Objectives:To identify the risk factors for worsening of ILD in RA patients under biological DMARD (bDAMARDs) therapy; particularly to determine whether types of bDMARDs are associated with exacerbation of ILD.Methods:A retrospective cohort study was conducted. Subjects were consecutive 91 RA-ILD patients who received HR-CT examination at starting and during bDMARDs therapy. Clinical data were collected by reviewing.ILD was diagnosed, when patients showed ground-glass opacity (GGO), consolidation, reticular pattern or honeycomb; the former two were acute and the latter two were chronic ILD lesions. The extent of each lesion was scored and recorded. When the score was increased, the lesion was judged as worsened. The incidence of exacerbation of ILD under an indicated agent was calculated as follows; the sum of (exposure period of the agent/exposure period of any agent) in patients with the exacerbation was divided by total exposure period of the agent.Results:Subjects were 36 males and 55 females with a mean age of 66.8 years. At the entry, GGO, consolidation, reticular and honeycomb were found in16, 20, 63 and 17, respectively. Acute and chronic ILD were observed in 34 and 68, respectively. ILD worsened in 35 (38.5%). Between patients with and without ILD exacerbation, no differences were found in demographics (sex, age, disease duration, the positivity for anti-CCP ab and RF) and radiographic findings of pulmonary abnormalities. Disease activities at as 1st and sequential CT examinations were similar in both groups.The incidences of the worsening of ILD varied according to bDMARDs (Fig 1). The incidence of total ILD worsening was high in TCZ compared to TNF and ABT. The incidence of acute ILD worsening was similar among the three groups. However, the worsening of chronic ILD was more frequently found in TCZ than other bDMARDs. The incidence of death by respiratory failure was similar in TNF, ABT, and TCZ (Fig. 2).Fig. 1Conclusion:TCZ might be a risk factor for exacerbation of ILD, particularly of chronic ILD.Fig. 2Disclosure of Interests:None declared

THU0616-HPR EXPIRATORY FLOW ACCELERATOR (EFA) IN SYSTEMIC SCLEROSIS PATIENTS WITH MUCUS HYPERSECRETION, PRODUCTIVE COUGH AND DYSPNOEA: PRELIMINARY RESULTS FROM A HOME-BASED AIRWAY CLEARANCE TECHNIQUE DAILY PROGRAM

Background:Systemic sclerosis (SSc) is a chronic disease with frequent lung involvement. As mucociliary clearance is impaired, mucus retention and frequent pulmonary infections, increase morbidity and mortality (1).Airway clearance techniques (ACT) enhance removal of mucus from the airways. Expiratory flow accelerator (EFA) is a new technology that promotes deep and gentle drainage of the bronchial secretions, through the Venturi effect. No respiratory effort is required and no negative pressure is generated, avoiding risk of bronchial collapse (2).Objectives:The aim of this study was to describe the effectiveness of EFA in improving pulmonary symptoms in SSc patients.Methods:SSc patients with daily productive cough, frequent pulmonary exacerbations, exertional dyspnea and/or reduced physical activity were selected. All of them underwent a home-based ACT program with EFA. A Respiratory Physiotherapist (RT) trained each patient to use the device 3 times a day, 15 minutes each session. Every subject compiled the Saint George’s Respiratory Questionnaire (SGRQ) and scleroderma Health Assessment Questionnaire (SHAQ) at baseline, 30, 90 and 180 days from the beginning. Statistical analysis has been carried out with General linear model for repeated measures. A value of p&lt;0.05 was considered statistically significant.Results:8 patients were enrolled (M:F=1:7), median age 54 (IC95% 46-69) years. Interstitial lung disease affected the majority of them (7/8). SGRQ total score and SHAQ domain for respiratory symptoms decreased over time (p= 0.003 and p= 0.005). In particular, there was an improvement in two SGRQ domains: activities (p= 0.013) and symptoms (p= 0.005) (fig.1).Figure 1Conclusion:This is the first study to investigate the effect of EFA technology on airway clearance in SSc patients. The observations suggest the importance of a daily ACT program with EFA in improving respiratory symptoms. This technology appear to be extremely promising in SS patient management as it is well tolerated and it has the potential to slow down the pulmonary disease progression by limiting bronchial infections.

МАГНИТНО-РЕЗОНАНСНО-ТОМОГРАФИЧЕСКАЯ ДИАГНОСТИКА ПОРАЖЕНИЯ ЛЁГКИХ ПРИ ЛИМФОМЕ

Aim: to study the MRI semiotics of lung involvement and compare the diagnostic efficacy of MRI, CT and PET/CT in lymphomas. Material and methods: the study included 314 patients with morphologically verified lymphoma, who before the start of treatment underwent MRI of the whole body and CT of the thorax and/or PET/CT of the whole body. Results. The frequency of lung involvement was 14%. The lesions were in the form of nodules/masses in 76% of cases, consolidation in 29% and interstitial changes in 9%. The sensitivity, specificity, and accuracy of MRI were 85%, 96%, and 94%, respectively, and the diagnostic efficiency was high (AUC 0.901). There were no significant differences in the sensitivity and efficiency of MRI and CT, MRI and PET/CT. Conclusion. MRI of the thorax is an effective method for diagnosing lung involvement in lymphoma and is recommended for practical use, especially in patients with variable fluorodeoxyglucose-avid lymphomas, young patients and pregnant women.

Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases

Introduction: Connective Tissue Diseases (CTDs) are systemic autoimmune conditions characterized by frequent lung involvement. This usually takes the form of Interstitial Lung Disease (ILD), but Obstructive Lung Disease (OLD) and Pulmonary Artery Hypertension (PAH) can also occur. Lung involvement is often severe, representing the first cause of death in CTD. The aim of this study is to highlight the role of Pulmonary Function Tests (PFTs) in the diagnosis and follow up of CTD patients. Main body: Rheumatoid Arthritis (RA) showed mainly an ILD with a Usual Interstitial Pneumonia (UIP) pattern in High-Resolution Chest Tomography (HRCT). PFTs are able to highlight a RA-ILD before its clinical onset and to drive follow up of patients with Forced Vital Capacity (FVC) and Carbon Monoxide Diffusing Capacity (DLCO). In the course of Scleroderma Spectrum Disorders (SSDs) and Idiopathic Inflammatory Myopathies (IIMs), DLCO appears to be more sensitive than FVC in highlighting an ILD, but it can be compromised by the presence of PAH. A restrictive respiratory pattern can be present in IIMs and Systemic Lupus Erythematosus due to the inflammatory involvement of respiratory muscles, the presence of fatigue or diaphragm distress. Conclusions: The lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respiratory disease related to each disease.

Pulmonary hypertension on systemic sclerosis-lupus erythematosus overlap syndrome

PurposeThere are authentic observations of combination of systemic lupus erythematosus (SLE) with systemic sclerosis (SS) and with polymyositis defined as overlap syndromes. The prevalence of pulmonary hypertension is unknown in SS-SLE overlap syndrome because of its rarity. The aim of our study was to precise clinical, paraclinical and evolutive features of pulmonary hypertension in patients with systemic sclerosis-systemic lupus erythematosus (SS-SLE) overlap syndrome. MethodsSixteen cases of SS-SLE overlap syndrome were retrospectively studied in a period of 16 years (2000–2015). SS-SLE overlap syndrome was diagnosed in the presence of at least 4 criteria of the American College of Rheumatology (ACR) for the diagnosis of SLE and a major criterion or 2 minor criteria of ACR of SS classification. Pulmonary arterial pressure (PAP) was estimated with doppler echocardiography. Pulmonary hypertension (PAH) was defined by a PAP superior than 30mmHg. We distributed groups according to the existence (Group 1) or not (Group 2) of a PAH. Epidemiological, clinical and evolutive features were compared between the two groups with bilateral fisher test (P significant if inferior at 0.05) ResultsSixteen cases of female patients with SS-SLE overlap syndrome with a middle-age of 39 years, extreme (29–58 years) were studied. PAH complicated the evolution of SS-SLE overlap syndrome in six cases with a middle-age of 41 years. Ten patients of the group 2 had an average age of 40 years. The average age of the beginning of the disease was 28 years in the group 1 and 31 years in the group 2. SS preceded SLE in 6 among 16 cases (Group 1: 2/6, Group 2: 4/10). SS was revealed most frequently by Raynauw's Syndrome in both groups (Group 1: 4/6, Group 2: 7/10). Cutaneous and articular involvements were the most frequent observed manifestations of SLE (Group 1: 5/6, Group 2: 6/10). In the group 1, the PAH was discovered approximatively11 years after the beginning of the SS-SLE overlap syndrome. The average PAP was 52mmHg, extreme (32–80mmHg). A right cardiac insufficiency complicated the evolution of the PAH in 3 cases. The PAH was primitive in 3 cases. There was no significant difference concerning the SS-SLE overlap syndrome onset disease symptoms, the frequency of lung involvement and esophageal, neurological, articular and trophic manifestations. PAH was not associated with lupic proliferative renal disease, neither with cutaneous proximal sclerosis nor with anti-Scl70 positivity. Patients were treated with vasodilator treatment in eleven cases: prostacyclin derivates in five cases and endothelin receptor antagonist in six cases. Two patients received corticosteroids and boli of cyclophosphamide for renal involvement and neurologic involvement in each case. Stabilization of PAP was observed in these two cases. Mean follow-up disease was 67 months, extreme (4–124 months) Cutaneous sclerosis evolution was not significantly different between both groups. Global cardiac insufficiency secondary to PAH caused death in one case. ConclusionAccording to the results of our study, SS-SLE overlap syndrome complicated with PAH seems to be associated more frequently with limited and distal cutaneous manifestations. Patients that have developed lupus nephropathy and/or had positive anti-Scl70 seem to be protected from appearance of PAH during the SS-SLE overlap syndrome.

Anti -citrullinated peptide antibodies profiling in established rheumatoid arthritis

ObjectivesAnti-citrullinated protein/peptide antibodies (ACPA) represent an important tool for the diagnosis of rheumatoid arthritis (RA) and the presence of multiple ACPA specificities is highly correlated with the evolution towards RA. However, little is known about the association of single specificities with disease manifestations and response to therapy in established RA. The aim of this work is to evaluate in a retrospective study the clinico-serological association of ACPA detected using VCP1 and VCP2 (EBV-derived citrullinated peptides) and HCP1 and HCP2 (histone-H4-derived citrulinated peptides) in established RA. MethodsIn 413 RA patients, anti-VCP1, -VCP2, -HCP1, -HCP2 were measured by ELISA. Patients were evaluated for systemic involvement, disease activity/severity, ongoing and past therapies. Data were analyzed by cluster analysis (CA) and principal component analysis (PCA). ResultsAnti-VCP1 were detected in 44% of RA patients; anti-VCP2 in 52%; anti-HCP1 in 46% and anti-HCP2 in 63%. CA and PCA independently demonstrated that ACPA levels are associated with RF positivity, and lung involvement. Subdividing patients in 5 groups according to the number of anti-peptide antibodies, mean antibody level and RF positivity, as well as the frequency of lung involvement, progressively increase in parallel with the number of ACPA specificities. ConclusionsHigher number/levels of ACPA subtypes is associated with lung involvement but not with erosive disease. Moreover, a broader ACPA repertoire may identify patients treated with biological therapy, probably affected by a more severe disease. In conclusion, ACPA typing might be relevant for a better characterization of some disease features in established RA.

Pleuropulmonary manifestation in patients with rheumatoid arthritis in Saudi Arabia.

BACKGROUND AND OBJECTIVES:Pleuropulmonary (PP) involvement in rheumatoid arthritis (RA) is associated with high morbidity and mortality. Nevertheless, limited data are available regarding lung complications in the Middle East, especially in Saudi Arabia. The objectives of the current study were to determine the prevalence of PP manifestations and to identify the associated risk factors.METHODS:This was a retrospective study involving 419 patients diagnosed at a tertiary center over a 12.5-year period. The frequency of pulmonary manifestations was recorded based on combined results from chest X-rays, pulmonary function tests, and high-resolution computed tomography scan of the chest.RESULTS:The overall frequency of lung involvement was 25.8%. Pneumonia, bronchiectasis, and interstitial lung disease were the most common abnormalities (36%, 35%, and 23%, respectively). The presence of comorbid illness (odds ratio [OR]: 3.19; 95% confidence interval [CI]: 2.02–5.1), male gender (OR: 2.4; 95% CI: 1.3–4.24), and the presence of extra-articular manifestations of RA (ExRA) (OR: 2.35; 95% CI: 0.4–4.01) were predictive of lung involvement.CONCLUSIONS:Pneumonia, bronchiectasis, and interstitial lung disease were the most common abnormalities seen in RA patients. The presence of comorbidity, male gender, and ExRA was significantly associated with lung involvement.

Factors Associated with Airway Disease and Interstitial Lung Disease in Rheumatoid Arthritis

Objective. This study examined lung involvement in patients with rheumatoid arthritis (RA) and identified factors associated with airway disease (AD) and interstitial lung disease (ILD). Methods. A total of 507 RA patients were enrolled in a cross-sec- tional study. Lung involvement was assessed by high-resolution computed tomography scan. The patient groups were classified according to normal, AD, and ILD. Multinomial logistic regression analysis was performed to identify factors associated with AD and ILD. Results. The most frequent lung involvement was AD (38.3%) followed by ILD (12.6%). Old age (adjust odds ratio (aOR) 2.58, 95% confidence interval (CI) 1.70 to 3.90 for AD; aOR 4.38, 95% CI 2.30 to 8.35 for ILD), male gender (aOR 2.57, 95% CI 1.22 to 5.42 for AD; aOR 5.48, 95% CI 2.20 to 13.65 for ILD) were factors associated with AD and ILD in RA patients. ILD was associated with short disease duration (aOR 0.30, 95% CI 0.14 to 0.62), AD was associated with high titers of anti-cy- clic citrullinated peptides antibodies (anti-CCP; aOR 1.61, 95% CI 1.07 to 2.44). Conclusion. AD was the most frequent lung involvement in patients with RA. Old age and male gender were both associated with AD and ILD. Short disease duration was associated with ILD. High titers of anti-CCP was associated with AD. (J Rheum Dis 2016;23:101-108)

ACPA-positive primary Sjögren's syndrome: true primary or rheumatoid arthritis-associated Sjögren's syndrome?

ObjectivesAnticyclic citrullinated protein antibodies (ACPA) are highly specific of rheumatoid arthritis (RA). However, they have also been detected in 5–10% of primary Sjögren's syndrome (pSS). We compared ACPA-positive and negative...

Pancreatic solitary and synchronous metastasis from breast cancer: a case report and systematic review of controversies in diagnosis and treatment

BackgroundMetastases from breast cancer cause the frequent involvement of lung, bone, liver, and brain, while the occurrence of metastases to the gastrointestinal tract is rare, and more frequently discovered after a primary diagnosis of breast cancer. Solitary pancreatic metastases from breast cancer, without widespread disease, are actually unusual, and only 19 cases have been previously described; truly exceptional is a solitary pancreatic metastasis becoming evident together with the primary breast cancer.Case presentationA 68-year-old woman reported general fatigue, lethargy, and jaundice. Abdominal ultrasound (US) and magnetic resonance imaging (MRI) showed an ampulloma of Vater’s papilla; moreover, a neoplastic nodule in the left breast was diagnosed. She underwent surgery for both breast cancer and ampulloma of Vater’s papilla. Pathological examination of pancreatic specimen, however, did not confirm primary carcinoma of the duodenal papilla, but showed a metastatic involvement of pancreas from lobular breast cancer. Immunohistochemistry has been essential to confirm the origin of the malignancy: hormone receptors and mammaglobin were expressed in both the primary breast tumor and the pancreatic metastasis.ConclusionsThis is one of the few reported cases in literature of an isolated and synchronous pancreatic metastasis from breast cancer, where the definitive diagnosis was obtained only after surgery. We discuss the controversies in this diagnosis and the choice of correct treatment. The surgical resection of solitary metastases can be performed in the absence of disseminated disease.

Eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome) is a peculiar hybrid condition of a systemic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and a hypereosinophilic disorder with frequent lung involvement that occurs in people with asthma. This review focuses on areas of evidence or persistent uncertainty in the classification, epidemiology, clinical presentation, diagnosis, prognosis and management of EGPA and attempts to identify clues to the mechanisms in the development or course of the disease. The 2013 revision of the EGPA definition formally placed the disease in the subset of ANCA-associated vasculitides. Recently published large case series underlined that the presence of ANCAs, found in 30-40% of EGPA, determines distinct but partly overlapping disease expression and the major detrimental effect of heart involvement on survival. There is some evidence that asthma in EGPA resembles a nonallergic eosinophilic asthma phenotype. Encouraging results have been reported for the treatment of EGPA with rituximab or with the eosinophil-targeted antiinterleukin-5 agent mepolizumab. The understanding of EGPA continues to advance, but many gaps in knowledge remain. The nomenclature remains a source of conceptual variance in terms of demonstrated presence or not of vessel inflammation or ANCAs in the diagnosis of EGPA. Distinguishing EGPA from hypereosinophilic syndromes can be problematic, and an understanding of the mechanistic relation between the vasculitis and the eosinophilic proliferation is profoundly lacking. Some evidence suggests distinct disease phenotypes, but this concept has not yet been translated to phenotype-adapted therapy.

Middle-segment preserving subtotal pancreatectomy for treating multifocal lesions in pancreas

Middle-segment preserving subtotal pancreatectomy for treating multifocal lesions in pancreas

Pediatric sarcoidosis: report of seven cases

Results 5/7 patients had early onset(before 5 birthday) another 2 had late onset sarcoidosis. All patients had a delayed diagnosis and were treated for other diseases. Early onset sarcoidosis was more multisystemic. Arthritis and uveitis were more common. The triad of: skin, joint and eye compromise was observed. The frequency of lung involvement was similar on both groups and 1/7 had mediastinal involvement. No patients with Loefgren triad were identified. A course with flares and remissions was a cardinal feature.

Epidemiology and Etiology of Wegener Granulomatosis, Microscopic Polyangiitis, Churg-Strauss Syndrome and Goodpasture Syndrome: Vasculitides with Frequent Lung Involvement

This review focuses on the epidemiological characteristics and etiologies of four primary systemic vasculitides with frequent lung involvement, namely Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS), and Goodpasture syndrome (GPS). Elucidation of the mechanisms underlying these vasculitides with frequent lung involvement is complicated by their rarity, which hampers the undertaking of large-scale studies; difficulties in classification; and their multifaceted clinical presentations, which infer the existence of several etiologic pathways. Notwithstanding, epidemiological research showed some evidence for international, interethnic, and temporal variations of the frequencies of these four vasculitides; led to the identification of several genetic and environmental risk factors; and provided insight on the extent to which genes and environment might contribute to their development. Available data support the concept that WG, MPA, CSS, and GPS have unique and shared risk determinants. Although the precise causes of these vasculitides are not yet fully understood and the development of prevention strategies is out of our reach at present, current knowledge enables the formulation of etiologic hypotheses to provide caregivers and their patients with valuable information on the nature of these rare entities.

CD8+, CD56+ (natural killer‐like) T‐cell lymphoma involving the small intestine with no evidence of enteropathy: Clinicopathology and molecular study of five Japanese patients

The present study reports five CD8+, CD56+ (natural killer (NK)-like) T-cell lymphomas involving the small intestine without evidence of enteropathy, from Japan. Three were intestinal T-cell lymphoma. The site of origin of the other two was not definitive. Four of five patients underwent emergency operation because of intestinal perforation. The small intestines of these patients had multiple ulcerative lesions with or without demarcated tumors. Histologically, the lymphoma cells were monomorphic or slightly pleomorphic and displayed epitheliotropism of varying degrees. Lymphoma cells of all patients shared the common phenotype: CD3+, CD4-, CD5-, CD8+, CD56+, CD57-, T-cell intracellular antigen-1+, granzyme B+. In contrast to nasal/nasal type NK-cell lymphomas, they had clonal rearrangement of T-cell receptor(TCR) genes and were negative for EBV-encoded RNA. Immunohistochemistry and genetics suggested that three cases were of alpha beta T-cell origin and two cases were of gamma delta T-cell origin. There was no evidence of enteropathy in any patient. The cases followed a clinically aggressive course with a frequent involvement of lung. According to the classification based on the recent genetic studies of European enteropathy-type intestinal T-cell lymphoma (ETL), the present cases could be classified as type 2 ETL.