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International Journal of Environmental... Jul 2022We aimed to evaluate the clinical course and impact of the SARS-CoV-2 pandemic on the rate of diagnosis and therapy in the complete Polish population of patients (pts)...
We aimed to evaluate the clinical course and impact of the SARS-CoV-2 pandemic on the rate of diagnosis and therapy in the complete Polish population of patients (pts) with pulmonary arterial hypertension (PAH-1134) and CTEPH (570 pts) treated within the National Health Fund program and reported in the national BNP-PL database. Updated records of 1704 BNP-PL pts collected between March and December 2020 were analyzed with regard to incidence, clinical course and mortality associated with COVID-19. Clinical characteristics of the infected pts and COVID-19 decedents were analyzed. The rates of new diagnoses and treatment intensification in this period were studied and collated to the proper intervals of the previous year. The incidence of COVID-19 was 3.8% (n = 65) (PAH, 4.1%; CTEPH, 3.2%). COVID-19-related mortality was 28% (18/65 pts). Those who died were substantially older and had a more advanced functional WHO class and more cardiovascular comorbidities (comorbidity score, 4.0 ± 2.1 vs. 2.7 ± 1.8; = 0.01). During the pandemic, annualized new diagnoses of PH diminished by 25-30% as compared to 2019. A relevant increase in total mortality was also observed among the PH pts (9.7% vs. 5.9% pre-pandemic, = 0.006), whereas escalation of specific PAH/CTEPH therapies occurred less frequently (14.7% vs. 21.6% pre-pandemic). The COVID-19 pandemic has affected the diagnosis and treatment of PH by decreasing the number of new diagnoses, escalating therapy and enhancing overall mortality. Pulmonary hypertension is a risk factor for worsened course of COVID-19 and elevated mortality.
Topics: COVID-19; Comorbidity; Humans; Hypertension, Pulmonary; Pandemics; SARS-CoV-2
PubMed: 35886278
DOI: 10.3390/ijerph19148423 -
Journal of Vascular Surgery Cases and... Sep 2019Mikulicz's disease is one of the IgG4-related diseases (IgG4-RDs) that involves the cardiovascular system; however, small-sized vasculitis is rare in IgG4-related...
Mikulicz's disease is one of the IgG4-related diseases (IgG4-RDs) that involves the cardiovascular system; however, small-sized vasculitis is rare in IgG4-related diseases. A 64-year-old man presented with distal occlusive disease and developed left cerebrovascular infarction with occlusion of the middle cerebral artery and diseased temporal artery branches. He underwent superficial temporal artery-middle cerebral artery anastomosis surgery. Histology of the temporal artery biopsy showed smooth muscle cell proliferation with many IgG4-positive plasma cells. He then developed salivary gland inflammation, and Mikulicz's disease was diagnosed. Small-sized occlusive vasculitis was observed in this IgG4-RD. Low-dose corticosteroid therapy is effective in preventing progressive occlusive disease.
PubMed: 31334401
DOI: 10.1016/j.jvscit.2019.03.019 -
Archives of Rheumatology Sep 2019Mikulicz's disease (MD), an immunoglobulin G4 (IgG4)-related disease, is a fibrosis-associated inflammatory disease characterized by painless bilat- eral swelling of...
Mikulicz's disease (MD), an immunoglobulin G4 (IgG4)-related disease, is a fibrosis-associated inflammatory disease characterized by painless bilat- eral swelling of lacrimal and salivary glands. Although thrombocytopenia is associated with many rheumatologic disorders, it is rare in IgG4-related MD. This novel study aims to report a rare case of IgG4-related MD complicated with severe autoimmune thrombocytopenia without any hemato- logical malignancies and review all cases of IgG4-related disease associated with thrombocytopenia in the literature.
PubMed: 31598602
DOI: 10.5606/ArchRheumatol.2019.7070 -
The Journal of Medical Investigation :... 2023Heineke-Mikulicz (HM) strictureplasty is commonly used to treat short stenoses in Crohn's disease. However, the degree to which intestinal motility is maintained remains...
BACKGROUND
Heineke-Mikulicz (HM) strictureplasty is commonly used to treat short stenoses in Crohn's disease. However, the degree to which intestinal motility is maintained remains unclear. We compared the peristalsis and transport capacity of the sutured intestines with HM configuration and transverse (TS) and longitudinal (LS) incisions.
METHODS
The intestinal diameter, intraluminal pressure, and bead transit time of each sutured group were compared with that of the non-treatment (NT) group in the isolated proximal colon of rats. Propulsive contractions were induced using hydroxy-?-sanshool (HAS), a constituent of Japanese pepper.
RESULTS
There was no change in the intestinal diameter between HM, TS, and NT groups ; however, it was significantly narrowed at the suture site and its distal side in the LS group. After HAS administration, the intestinal diameter at the suture site in the HM group was higher than that in the LS group. The intraluminal pressure was higher and the transit time was shorter in the HM group compared to those in the LS group.
CONCLUSIONS
The HM configuration, which widens the incision site and distal diameter and shortens the cut surface of the circular muscle in the longitudinal direction, may help maintain basal and HAS-induced intestinal peristalsis and motility. J. Med. Invest. 70 : 180-188, February, 2023.
Topics: Rats; Animals; Intestines; Crohn Disease; Constriction, Pathologic; Colon; Anastomosis, Surgical
PubMed: 37164718
DOI: 10.2152/jmi.70.180 -
BMC Pulmonary Medicine Mar 2024IgG4-related disease is a systemic fibroinflammatory disease that is mainly seen in older men, and involves multiple organs, such as the pancreas and lungs. However, 75%...
BACKGROUND
IgG4-related disease is a systemic fibroinflammatory disease that is mainly seen in older men, and involves multiple organs, such as the pancreas and lungs. However, 75% of patients with IgG4-related lung disease are asymptomatic (if they are symptomatic, they mainly complain of nasal congestion, rhinorrhoea, chest pain, and cough) and are incidentally diagnosed through chest computed tomograph. Although, nodules in the airway and bronchial wall thickening are criteria for diagnosis, it is important that nodules have been reported in peripheral airways in several cases and rarely in the central airway.
CASE PRESENTATION
A 74-year-old woman previously diagnosed with Mikulicz's disease presented with swelling of the eyelid margin on both sides and visual disturbances. Computed tomography revealed extensive multiple nodules and mucosal oedema of the trachea and both bronchi. On flexible bronchoscopy under local anaesthesia, extensive lesions were observed from the middle of the trachea to the carina, extending into both segmental bronchi. The nodules were continuous with the normal respiratory tract mucosa, and the surfaces were smooth with minimal neovascularisation. Due to the solid nature of the lesion, obtaining an adequate amount of specimen was challenging. Therefore, we used a 1.9 mm cryoprobe under intubation, resulting in minimal bleeding. Subsequently, the patient was diagnosed with IgG4-related lung disease.
CONCLUSIONS
The present case is very rare because of the presence of multiple nodules, severe mucosal edema of the central airway and the absence of mediastinal lymphadenopathy, ground glass nodules, and lung masses. Therefore, it is important to consider differential diagnoses. Thus, we emphasise the importance of endobronchial cryobiopsy for obtaining an adequate number of tissue specimens in such cases to establish a definitive pathological diagnosis.
Topics: Male; Female; Humans; Aged; Trachea; Immunoglobulin G4-Related Disease; Bronchi; Edema; Lung Diseases; Immunoglobulin G
PubMed: 38443819
DOI: 10.1186/s12890-024-02926-w -
Cureus Aug 2020Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity of rare and complex immune-mediated fibroinflammatory conditions that can affect any organ. The...
Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity of rare and complex immune-mediated fibroinflammatory conditions that can affect any organ. The concomitance of IgG4 sclerosing sialadenitis and dacryoadenitis with rhinosinusitis is extremely rare. We report a case of IgG4 sclerosing sialadenitis and dacryoadenitis (Mikulicz's disease) diagnosed in a middle-aged African American man with a long-standing history of chronic rhinosinusitis who presented with progressively worsening bilateral salivary and lacrimal glands swelling. Imaging revealed pansinusitis, symmetric enlargement of the lacrimal glands, parotid glands, and submandibular glands. Serological IgG4 level was significantly elevated and the diagnosis of IgG4 sclerosing sialadenitis was confirmed by histopathology. A robust clinical response in the facial swelling and nasal manifestations was noted after the initiation of immunotherapy with corticosteroids.
PubMed: 32821631
DOI: 10.7759/cureus.9756 -
Allergy, Asthma, and Clinical... May 2021Worldwide, allergy affects more than one billion people, with particularly rising prevalence in industrialised areas. Specifically, young adults appear to be...
BACKGROUND
Worldwide, allergy affects more than one billion people, with particularly rising prevalence in industrialised areas. Specifically, young adults appear to be predominantly targeted for an allergy diagnosis. Allergic diseases in pregnancy are mainly pre-existing but could also occur de novo. The immunological changes while pregnant, with increased Th2 lymphocyte activity, can facilitate allergen sensitisation.
OBJECTIVE
The aim of this study was to evaluate the pattern of specific IgE (sIgE) sensitisation to common inhalant and food allergens in pregnancy, and assess its relationship to self-reported allergic disease.
METHODS
We assessed 200 pregnant women, aged 20-38 years (mean age = 29 years), participant of ELMA (Epigenetic Hallmark of Maternal Atopy and Diet) study, living in a metropolitan area, with no pregnancy associated metabolic complications, for total IgE and allergen specific IgE to 20 allergens.
RESULTS
48% of pregnant women were sensitised to at least one allergen, at a cut-off point of 0.35 kU/L and they were assigned as atopic. However 42% in atopic group were not reporting any allergic disease. The most common inhalant allergens were: pollen (24.5%) and animal dander (23.5%). The most common food allergens were: cow's milk (5.5%) and apples (4.5%). 7.5% of women reported asthma, 21.5% allergic rhinitis, 11.5% atopic dermatitis and 18.5% food allergy. 8.5% of were taking medication for asthma or allergies. Atopic dermatitis had the highest tendency to become more severe during pregnancy. Total IgE values were significantly higher in atopic women.
CONCLUSIONS
Allergic sensitisation is a common phenomenon in pregnancy. Some sensitisations could be asymptomatic. Further studies should investigate if sensitisation in mothers confers risks for immune alterations in their children.
PubMed: 33971945
DOI: 10.1186/s13223-021-00547-0 -
Journal of Clinical Medicine Mar 2022Living donor kidney transplantation is a widely performed medical procedure. Living kidney donation requires an in-depth health assessment of candidates. The potential... (Review)
Review
Living donor kidney transplantation is a widely performed medical procedure. Living kidney donation requires an in-depth health assessment of candidates. The potential living kidney donor must remain healthy after kidney removal. A consequence of donation can be a decrease in glomerular filtration rate (GFR), and donors can become at risk of developing chronic kidney disease (CKD). We present a rationale for potential living kidney donor withdrawal due to Paget's disease of bone (PDB) based on a literature review. The treatment for PDB includes the use of, for example, non-steroidal anti-inflammatory drugs (NSAIDs), which can lead to acute kidney injury (AKI) as well as CKD, or bisphosphonates, which are not recommended for patients with decreased GFR.
PubMed: 35329811
DOI: 10.3390/jcm11061485 -
AME Case Reports 2020Immunoglobulin G4 (IgG4) related disease is a rare autoimmune disease involving multiple organs and tissues. A diagnosis of IgG4-related disease (IgG4-RD) is mainly...
Immunoglobulin G4 (IgG4) related disease is a rare autoimmune disease involving multiple organs and tissues. A diagnosis of IgG4-related disease (IgG4-RD) is mainly based on serum IgG4 concentration, imaging, pathology, and effective glucocorticoids therapy. In this paper, we report a 53-year-old male with typical signs and symptoms of IgG4-RD successfully treated with glucocorticoids. This patient had experienced bilateral mumps for more than 8 months and intermittent abdominal pain spreading to his lower back for 2 months before his admission. During his hospitalization, based on the characteristic appearance of magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), and computed tomography with positron emission tomography, a diagnosis of autoimmune pancreatitis (AIP), cholangitis with biliary obstruction, lachrymal adenitis, and submandibular adenitis was made. A high serum IgG4 concentration further supported a diagnosis of IgG4-RD. Then, the treatment was promptly initiated with corticosteroids. MRI, MRCP, and IgG4 concentration were re-examined during his follow up, suggesting that glucocorticoid treatment resulted in a resolution of his disease. The dosage of glucocorticoid had been gradually decreased. Now, he is stable with oral low-dose glucocorticoids. Certainly, long-term follow up of this patient with such a rare disease is very essential to observe the possibility of disease recurrence and glucocorticoids related complications.
PubMed: 33178994
DOI: 10.21037/acr-20-43 -
International Journal of Molecular... Dec 2022Pulmonary arterial hypertension (PAH) is an increasingly frequently diagnosed disease, the molecular mechanisms of which have not been thoroughly investigated. The aim...
Pulmonary arterial hypertension (PAH) is an increasingly frequently diagnosed disease, the molecular mechanisms of which have not been thoroughly investigated. The aim of our study was to investigate subpopulations of lymphocytes to better understand their role in the molecular pathomechanisms of various types of PAH and to find a suitable biomarker that could be useful in the differential diagnosis of PAH. Using flow cytometry, we measured the frequencies of lymphocyte subpopulations CD4+CTLA-4+, CD8+ CTLA-4+ and CD19+ CTLA-4+ in patients with different types of PAH, namely pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH), pulmonary arterial hypertension associated with connective tissue disorders (CTD-PAH), chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (iPAH), and in an age- and sex-matched control group in relation to selected clinical parameters. Patients in the iPAH group had the significantly highest percentage of CD4+CTLA-4+ T lymphocytes among all PAH groups, as compared to those in the control group (p < 0.001), patients with CTEPH (p < 0.001), CTD-PAH (p < 0.001) and CHD-PAH (p < 0.01). In iPAH patients, the percentages of CD4+CTLA-4+ T cells correlated strongly positively with the severity of heart failure New York Heart Association (NYHA) Functional Classification (r = 0.7077, p < 0.001). Moreover, the percentage of B CD19+CTLA-4+ cells strongly positively correlated with the concentration of NT-proBNP (r = 0.8498, p < 0.001). We have shown that statistically significantly higher percentages of CD4+CTLA-4+ (p ≤ 0.01) and CD8+ CTLA-4+ (p ≤ 0.001) T cells, measured at the time of iPAH diagnosis, were found in patients who died within 5 years of the diagnosis, which allows us to consider both of the above lymphocyte subpopulations as a negative prognostic/predictive factor in iPAH. CTLA-4 may be a promising biomarker of noninvasive detection of iPAH, but its role in planning the treatment strategy of PAH remains unclear. Further studies on T and B lymphocyte subsets are needed in different types of PAH to ascertain the relationships that exist between them and the disease.
Topics: Humans; Hypertension, Pulmonary; Familial Primary Pulmonary Hypertension; Pulmonary Arterial Hypertension; CTLA-4 Antigen; Biomarkers; Cell Differentiation
PubMed: 36555549
DOI: 10.3390/ijms232415910