IgG4-related disease and isolated retroperitoneal fibrosis: A narrative review.

Retroperitoneal fibrosis (RPF) can occur due to many etiologies and is categorized into idiopathic and secondary. Etiologies of secondary RPF include medications, autoimmune disease, malignancy, and IgG4-related disease (IgG4-RD). Although IgG4-RD usually involves multiple systems synchronically including the pancreas, aorta, and kidneys, it can present with isolated RPF without involvement of other organ systems. Caution must be exercised in these instances as the diagnosis should be confirmed based on specific clinical, radiographic, and histopathologic criteria. Such confirmation can affect the work-up and therapeutic approach as treatment with corticosteroids can lead to remission, both clinically and radiographically.

Hemorrhagic Cardiac Tamponade as a Complication of Limited Cutaneous Systemic Sclerosis.

Cardiac tamponade is an uncommon complication of systemic sclerosis (SSc) with a high mortality rate. Here, we report a case of a 58-year-old patient with limited cutaneous systemic sclerosis (lcSSc), gastroesophageal reflux disease (GERD), diabetes mellitus, pulmonary hypertension (PHTN), and COVID-19 infection, which occurred one month ago, presenting with a large hemorrhagic pericardial effusion and early cardiac tamponade. The patient had an acute onset of progressive dyspnea and anasarca. On examination, she was tachypneic, tachycardic, desaturating on room air, and hypotensive. Pitting edema up to thighs and bilateral basilar crackles were also appreciated. Labs were remarkable for negative troponin, chest X-ray with pulmonary congestion, D-dimer at 6.01, CT angiogram negative, brain natriuretic peptide level at 73 pg/mL, C-reactive protein level at 7.64 mg/dL, normal complement levels, and negative COVID-19 test results. Echocardiography showed early tamponade and a large circumferential effusion with chamber collapse. Right heart catheterization was performed finding PHTN at 54 mmHg. Pericardiocentesis drained 500 mL of the hemorrhagic effusion. Fluid analysis showed RBC at 220,000/uL, WBC at 5000/uL, protein 4.8 g/dL, lactate dehydrogenase level of 1275 U/L, and negative cytology. The patient was treated for serositis from lcSSc flare with mycophenolate mofetil and steroids, and responded very well. Hemorrhagic cardiac tamponade is a very rare phenomenon in limited scleroderma. A recent COVID-19 infection could have served as a trigger factor for our patient's lcSSc in long remission to flare up. Clinicians should maintain a high index of suspicion and a low threshold for intervention when lcSSc patients have an acute onset of cardiac compromise, especially with a history of a recent COVID-19 infection.

Nationwide analysis of adult hospitalizations with hematologic malignancies and systemic sclerosis.

Introduction Systemic sclerosis (SSc) is a connective tissue disease with multi-system involvement and it has an increased risk of developing hematologic malignancies. This study aims to report the association between hematologic malignancies with SSc and to characterize in-hospital demographics and outcomes in patients with hematologic malignancies with and without SSc. Methods We performed a retrospective review of pooled data from the National Inpatient Sample (NIS) database from 2016 to 2020. Crude prevalence of hematologic malignancies among hospitalized patients with and without SSc was calculated. Logistic regression was used for statistical significance of differences in prevalence while adjusting for confounders. Demographic characteristics and outcomes of patients with hematologic malignancies with and without SSc was compared. Statistical analysis was done using chi-square and multivariate logistic regression. Results Among all adult hospitalizations, the prevalence of hematologic malignancy was 1.87% compared to 2.66% among patients with SSc (adjusted odds ratio (aOR) 1.52, p <0.01). Relative to the non-SSc group, the SSc group had higher odds of in-patient mortality (OR 1.43; 95% confidence interval (CI) 1.11 - 1.87; p<0.01). The prevalence of lymphoma was 0.71% compared to 1.04% among patients with SSc (aOR 1.6, p < 0.01). Relative to the non-SSc group, the lymphoma-SSc group had similar odds of in-patient mortality (OR 0.93; 95% CI 0.55 - 1.59; p=0.80). The prevalence of leukemia was 0.79% compared to 1.28% among patients with SSc (aOR 1.74, p < 0.01). The leukemia-SSc group had higher odds of in-patient mortality (OR 1.78; 95% CI 1.29 - 2.46; p<0.01). For myeloma, there was no difference in the prevalence in adults with and without SSc (0.4 vs. 0.38%, aOR 0.96, p=0.64) and there was no difference of in-hospital mortality. Conclusions There is a positive significant association between hematologic malignancies including lymphoma and leukemia, and SSc. This association was not seen between myeloma and SSc. There is increased in-hospital mortality of patients with leukemia and SSc.

Transport of Peripartum Patients for Medical Management: Predictors of Any Intervention During Transport.

Background When obstetric patients present to a hospital without obstetric consultants for medical care, they are often transferred for a higher level of care. Factors associated with patient care during transport between hospitals are unknown. Our study investigated factors associated with care intensity. Methods We retrospectively reviewed charts from peripartum adult patients who were transported to our academic quaternary center between January 1, 2012, and April 19, 2020. We excluded patients transported for gynecologic or fetus-related complications. We defined any transport interventions as any ventilator management, any titration of continuous infusions, and any continuation or initiation of medication during transport. Results We included 126 patients, and the mean age (SD) was 29 (7) years. There were 87 (695) antepartum patients, with nine (7%) requiring mechanical ventilation. Of the patients, 98 (78%) received at least one intervention during transport. Transport time (OR: 1.03, 95% CI: 1.001-1.06, P = 0.040), preeclampsia (OR: 3.8, 95% CI: 1.1-12.7, P = 0.030), and any obstetric hemorrhage (OR: 8.3, 95% CI: 1.03-68, P = 0.047), either antepartum or postpartum, were associated with higher likelihood of receiving any intervention. Conclusions Peripartum patients often received interventions during transport. Preeclampsia and hemorrhage were associated with the likelihood of interventions. Transport clinicians should be prepared when transporting these patients. Further studies are needed to confirm our observations.

A Rare Case of Homocysteinemia Presenting With Multiple Aneurysms in an Adolescent Boy.

Thoracoabdominal aortic aneurysm (TAAA) is primarily a disorder of the elderly; the condition, however, is rare in children, for whom the misdiagnosis is not uncommon. It is one of the leading causes of death in the older age group worldwide, with a 4:1 male to female ratio. There are no real data about the incidence of aortic aneurysms in childhood. Although rare, an aortic aneurysm can be an important cause of mortality in children and adolescents. We present a case of an adolescent boy with a left coronary artery aneurysm, left axillary artery aneurysm, and TAAA type-IV caused by the metabolic disease homocysteinemia. He was referred to our facility when the complicated picture of the disease was discovered.

Physician Gender Preference Amongst Females Attending Obstetrics/Gynecology Clinics.

INTRODUCTION: The objective of our study was to explore the views and perceptions of female patients attending the obstetrics and gynecology (OB/GYN) outpatient department towards the gender of their healthcare provider, to look for any preference that might exist in this regard, and to highlight any discrimination towards male obstetricians/gynecologists. MATERIAL AND METHODS: A cross-sectional study was conducted from November 2020 to March 2021 at Combined Military Hospital, Lahore. A total of 280 female patients were included in the study and interviewed consecutively. A self-designed questionnaire was administered. Data were analyzed using Statistical Package for Social Sciences (SPSS) version 22 (IBM Corp., Armonk, NY). Chi-square test was used to determine for any statistical significance and p≤0.05 was considered significant. Numerical data were represented as percentages. RESULTS: Over 280 female patients participated in our study, out of which 132 were married and 148 were single. Thirteen of these patients were uneducated, 40 had completed high school and 227 patients had obtained a bachelor's level of education; 120 patients were aged 15-25 years, 95 patients were aged 26-35 years, 30 patients were aged 36-45 years, and 35 patients were aged >46. Women with less education preferred to be seen by a female obstetrician/gynecologist, whereas those with higher education were less biased (p=0.0001). Married patients preferred to be seen by female obstetrician/gynecologists as compared to single patients (p=0.0004). A significant proportion of females were impartial in terms of physician competence but those who did have a preference preferred female obstetrician/gynecologist based on three significant variables: competence, rapport building and empathy, and personal comfort. CONCLUSION: A female gender preference exists in obstetrics and gynecology clinic attendees. This is significant in those who are married and those with no formal education. Overall, a significant proportion of women feel a higher comfort level with female doctors and find it easier to discuss their medical issues and develop rapport.

Arterial or cuff pressure: Clinical predictors among patients in shock in a critical care resuscitation unit.

OBJECTIVES: Blood pressure (BP) measurement is essential for managing patients with hypotension. There are differences between invasive arterial blood pressure (IABP) and noninvasive blood pressure (NIBP) measurements. However, the clinical applicability of these differences in patients with shock [need for vasopressor or serum lactate ≥ 4 millimole per liter (mmol/L)] has not been reported. This study investigated differences in IABP and NIBP as well as changes in clinical management in critically ill patients with shock. METHODS: This was a retrospective study involving adult patients admitted to the Critical Care Resuscitation Unit (CCRU). Adult patients who received IABP upon admission between 01/01/2017-12/31/2017 with non-hypertensive diseases were eligible. The primary outcome, clinically relevant difference (CRD), was defined as difference of 10 mm of mercury (mmHg) between IABP and NIBP and change of blood pressure management according to goal mean arterial pressure (MAP) ≥ 65 mmHg. We performed forward stepwise multivariable logistic regression to measure associations. RESULTS: Sample size calculation recommended 200 patients, and we analyzed 263. 121 (46%) patients had shock, 23 (9%) patients had CRD. Each mmol/L increase in serum lactate was associated with 11% higher likelihood of having CRD (OR 1.11, 95%CI 1.002-1.2). Peripheral artery disease and any kidney disease was significantly associated with higher likelihood of MAP difference ≥ 10 mmHg. CONCLUSION: Approximately 9% of patients with shock had clinically-relevant MAP difference. Higher serum lactate was associated with higher likelihood of CRD. Until further studies are available, clinicians should consider using IABP in patients with shock.

Predictors of clinically relevant differences between noninvasive versus arterial blood pressure.

OBJECTIVE: Blood pressure (BP) measurements are important for managing patients with hypertensive emergencies (HE). Previous studies showed that there was significant difference between IABP and NIBP but no information whether these differences changed management. Our study investigated the factors associated with the differences affecting BP management of patients with HE. METHODS: This was a retrospective study involving adult patients admitted to a resuscitation unit. We screened all patients who received IABP upon admission between 06/01/2017 and 12/31/2017 as sample size calculation recommended 64 patients. Primary outcome was the clinical relevance of the difference of IABP vs. NIBP, which was defined as having both: a) difference of 10 mm of mercury (mmHg), and b) resulting in possible change of blood pressure managements according to treatment guidelines. We performed backward stepwise multivariable logistic regression to measure associations. RESULTS: We analyzed 147 patients whose mean age was 69 (±16) years and included 69 (47%) patients with spontaneous intracerebral hemorrhage (sICH). Mean difference between IABP and NIBP was 21 (±16) mmHg while 41 (28%) patients who had difference affecting managements. In multivariable regression, sICH (Odd Ratios 13.5, 95%CI 2.3-79.5, p-value < 0.001) was significantly associated with clinically relevant difference between the two modalities of BP monitoring. CONCLUSIONS: There was a large difference between IABP and NIBP among patients with hypertensive emergencies. Up to 30% of patients had clinically relevant differences. Patients with sICH were more likely to have differences affecting BP management. Further studies are needed to confirm our observation.