A Systematic Review of the Key Predictors of Progression and Mortality of Rheumatoid Arthritis-Associated Interstitial Lung Disease.

Background: 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.

Current Perspectives in Giant Cell Arteritis: Can We Better Connect Pathogenesis and Treatment?

Giant cell arteritis (GCA) is a large-vessel vasculitis affecting elderly patients and targeting the aorta and its main branches, leading to cranial and extracranial manifestations. The mechanism behind the ischemia is a granulomatous-type inflammation with potentially critical lesions, including visual loss involving the ophthalmic artery. Despite significant progress in unraveling the pathophysiology of this disease, treatment options still rely on glucocorticoids (GCs) to overcome active vascular lesions and disease flares.

Calcinosis in Rheumatic Disease Is Still an Unmet Need: A Retrospective Single-Center Study.

Patients with immune-mediated rheumatic disease-related calcinosis comprise a subgroup at risk of encountering a more severe clinical outcome. Early assessment is pivotal for preventing overall disease progression, as calcinosis is commonly overlooked until several years into the disease and is considered as a 'non-lethal' manifestation. This single-center retrospective study explored the prevalence, clinical associations, and impact on survival of subcutaneous calcinosis in 86 patients with immune-mediated rheumatic diseases (IMRD).

The Role of Mannose-Binding Lectin and Inflammatory Markers in Establishing the Course and Prognosis of Community-Acquired Pneumonia in Children.

Background: Community-acquired pneumonia (CAP) is one of the most significant childhood diseases worldwide and a leading infectious cause of death in children. This study aimed to evaluate the prognostic value of the inflammatory markers-C-reactive protein (CRP) and procalcitonin (PCT)-and the polymorphic glycoprotein mannose-binding lectin (MBL), deficiency of which is associated with severe infections, in the determination of the optimal type and timing of therapeutic intervention for CAP in childhood.

Methods: Retrospective evaluation was conducted on a cohort of 204 children aged 4 months-17 years hospitalized with CAP.

A "Mix and Match" in Hemochromatosis-A Case Report and Literature Focus on the Liver.

Hemochromatosis is a genetic disorder characterized by increased iron storage in various organs with progressive multisystemic damage. Despite the reports dating back to 1865, the diagnosis of hemochromatosis poses a challenge to clinicians due to its non-specific symptoms and indolent course causing significant delay in disease recognition. The key organ that is affected by iron overload is the liver, suffering from fibrosis, cirrhosis or hepatocellular carcinoma, complications that can be prevented via early diagnosis and treatment.

A new CECR1 mutation associated with severe hematological involvement in ADA2 deficiency.

Background: The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessively inherited disease resulting from loss-of-function mutations in ADA2, formerly named CECR1 (cat eye syndrome chromosome region, candidate 1) gene. Disease manifestations could be separated into three major phenotypes: inflammatory/vascular, immune dysregulatory, and hematologic; however, most patients presented with significant overlap between these three phenotype groups.

Case Presentation: We present a case of DADA2 deficiency with disease onset at 3 years old, not recognized till the age of 18 with severe gastrointestinal vasculitis and recurrent episodes of neutropenia associated with a new CECR1 mutation.

BILATERAL CAROTID BODY PARAGANGLIOMAS - LITERATURE REVIEW AND COMMENTS IN A PATIENT WITH NO SIGNS OF MEN SYNDROME.

Unlabelled: Paragangliomas are rare neuroendocrine slow-growing tumors, often asymptomatic, that originate from embryonic neural crest cell. In the head and neck area, the most common location is the carotid body, followed, with decreasing frequency, in jugular, tympanic and vagal sites. Bilateral carotid body tumors are extremely rare.

Oculo-orbital complications of odontogenic sinusitis.

Odontogenic sinusitis is a well-known, but under-studied bacterial infection of the maxillary sinus that can extend to other sinuses, the orbit, or even the endocranium. We performed an observational retrospective study on the patients with odontogenic sinusitis treated in our hospital over a five-year period. We included patients over 18 years old diagnosed with odontogenic sinusitis and ocular complications and we excluded patients with ocular complications nonrelated to dental-originated sinusitis or patients with odontogenic sinusitis without orbital-ocular complications.

The Spectrum of Extraglandular Manifestations in Primary Sjögren's Syndrome.

Extraglandular manifestations (EGMs) in primary Sjogren's syndrome (pSS) represent the clinical expression of the systemic involvement in this disease. EGMs are characterized by a wide heterogeneity; virtually any organ or system can be affected, with various degrees of dysfunction. The existing gaps of knowledge in this complex domain of extraglandular extension in pSS need to be overcome in order to increase the diagnostic accuracy of EGMs in pSS.

Comparative Analysis of Hematological and Immunological Parameters in Patients with Primary Sjögren's Syndrome and Peripheral Neuropathy.

Background: Primary Sjögren syndrome (pSS) is a multisystem disorder of autoimmune etiology, frequently involving peripheral nerves. Early detection of peripheral neuropathy (PN) manifestations might improve prognosis and disease control. The purpose of the study was to evaluate the predictive potential of hematological and immunological parameters associated with PN development in pSS patients.

The Use of Esophageal Stents in the Management of Postoperative Fistulas-Current Status, Clinical Outcomes and Perspectives-Review.

Unlabelled: Esophageal fistula remains one of the main postoperative complications, with the treatment often requiring the use of stents. This article reviews the updates on the use of endoscopic stents for the treatment of postoperative esophageal leakage in terms of indications, types of stents used, efficiency, specific complications and perspectives.

Materials And Methods: We searched the PubMed and MEDLINE databases for the keywords postoperative esophageal anastomotic leak and postoperative esophageal anastomotic leak stent, and retrieved relevant papers published until December 2022.

Eosinophilic Fasciitis: Current and Remaining Challenges.

Eosinophilic fasciitis (EF), defined as diffuse fasciitis with eosinophilia by Shulman in 1974, is a disease with unknown etiology and whose pathogenesis is still being researched. The diagnosis is based on the clinical aspects (skin induration with an "orange peel" appearance), the lab results (eosinophilia, increased inflammatory markers), the skin biopsy with the pathognomonic histopathological result, as well as the typical MRI changes. The treatment includes glucocorticoids and immunosuppressive drugs.

Hypocomplementemic Urticarial Vasculitis Associated With Hashimoto's Thyroiditis and Hepatitis B Virus Infection: A Case Report.

Urticarial vasculitis (UV) is an uncommon condition characterized by recurrent episodes of urticarial lesions and angioedema and the pathological features of leukocytoclastic vasculitis. UV divides into two subgroups based on the level of serum complement. Usually, patients with hypocomplementemia experience internal organ involvement and an unfavorable prognosis.

The Predictive Role of Neutrophil-to-Lymphocyte Ratio (NLR), Platelet-to-Lymphocyte Ratio (PLR), Monocytes-to-Lymphocyte Ratio (MLR) and Gammaglobulins for the Development of Cutaneous Vasculitis Lesions in Primary Sjögren's Syndrome.

Background: In primary Sjögren’s Syndrome (pSS), cutaneous vasculitis lesions (CVL) are extraglandular manifestations with an important clinical and prognostic impact and their early detection might contribute to the improvement of disease control and even patients’ survival. The aim of this study was to evaluate the predictive potential of hematological elements in the development of CVL in pSS patients. Methods: In this single center, retrospective study, a total of 245 participants were included (124 pSS patients and 121 healthy controls).

Pancreatic Tuberculosis-A Condition That Mimics Pancreatic Cancer.

Tuberculosis is a disease with serious consequences in terms of morbidity and mortality. Pancreatic localization is very rare and is mostly encountered in patients with immunosuppressive disorders. A 59-year-old woman with arterial hypertension, grade 2 obesity, and a history of cholecystectomy, was admitted for fever (38.

New approach for hepatocellular carcinoma treatment.

Hepatocellular carcinoma (HCC) is the fifth most common cancer, with an increasing incidence in recent years. The prognosis is unfavorable, representing the third most frequent cause of cancer-related death worldwide. This is because it generally develops in patients with pre-existing liver pathology, thus limiting therapeutic options.

A clinical case of orbital inflammatory pseudotumor as the primary expression of eosinophilic angiocentric fibrosis.

Eosinophilic angiocentric fibrosis (EAF) is an infrequent and slowly progressive disease, represented by fibroinflammatory lesions of unknown origin, which mainly involves the sinonasal structures and upper respiratory tract. Occasionally, it can affect the orbit and ocular adnexa causing symptoms such as proptosis, globe displacement and periorbital edema. In very rare cases, ocular manifestation as an orbital inflammatory pseudotumor can be the primary localization of the disease.

A clinical case of recurrent episcleritis as the initial manifestation of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is a type of small-sized blood vessel vasculitis that predominantly affects the upper airways, lungs and kidneys and associates with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Nevertheless, any organ of the body can be affected by GPA, including the eye. Occasionally, ocular involvement can be the initial manifestation, thus representing an essential clue for the physician in the early diagnosis of the disease.

Do We Have Good Activity Indices in Systemic Sclerosis?

Background: No fully validated index is available for assessing overall disease activity in systemic sclerosis (SSc).

Objectives: To estimate the effect of disease activity as measured by different disease activity indices on the risk of subsequent organ damage.

Methods: The European Systemic sclerosis study group activity index (EScSG AI), the European Scleroderma Trials and Research Group Activity Index (r-EUSTAR AI), 12 point activity index proposed by Minier (12point AI) were calculated for 91 patients; the CRISS (The Composite Response Index for Systemic Sclerosis) for patients included after 2016.

Gastrointestinal Stromal Tumors-A Mini Review.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. They are potentially malignant, and have an unpredictable evolution. The origin of these tumors is in the interstitial cells of Cajal, which are cells that are interposed between the intramural neurons and the smooth muscle cells of the digestive tract.

The role of lung ultrasonography in predicting the clinical outcome of complicated community-acquired pneumonia in hospitalized children.

Aims: This study's objective was to analyze lung ultrasonography (LUS) characteristics in hospitalized pediatric patients with complicated community-acquired pneumonia (CAP). We hypothesized that LUS could be correlated with the clinical outcome in these cases.

Materials And Methods: In this retrospective study, we evaluated the LUS appearances (at admission and five days after the beginning of the treatment) and the progression of complicated CAP.