Granulomatous Polyangiitis Refractory to Induction With Rituximab in 3 Patients.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of immune-mediated diseases characterized by inflammation of small vessels, leading to endothelial injury with subsequent tissue damage. Current guidelines recommend induction therapy with rituximab over cyclophosphamide for severe disease activity. In this case series-based review, the authors discuss 3 cases of granulomatosis with polyangiitis (GPA) with proteinase-3 (PR3) disease that deteriorated following induction therapy with rituximab combined with mycophenolate mofetil and high-dose steroids. All 3 patients subsequently required salvage therapy with cyclophosphamide. Our experience suggests there is a temporal window where induction with rituximab is not fully effective, and deterioration or death can ensue. Expert recommendations do not offer a preferential protocol for induction with either rituximab or cyclophosphamide, with some even using a combination of both.

Pulmonary artery aneurysm in a young female with Takayasu arteritis.

Key Clinical Message: In suspected cases of systemic vasculitis, imaging studies should include the pulmonary artery. This is a rare case of Takayasu arteritis with a large pulmonary aneurysm. Medical management is the first line and vascular intervention if fails prior. Abstract: Takayasu arteritis (TA) should be suspected in young women presented with hypertension, carotidynia, and claudications. Pulmonary artery involvement is frequent, occurring in 20%-50% of patients with TA. However, this case highlights the rare presentation of TA with a large pulmonary aneurysm and minimal aortic involvement. Medical management including immunosuppressive agents and biological therapies remains an important role, with vascular intervention remains as an option if medical therapy failed.

Transient diabetes insipidus after vasopressin discontinuation in cystic fibrosis with septic shock.

Central diabetes insipidus (DI) is an uncommon condition caused by reduced or lack of vasopressin secretion from the posterior pituitary gland, typically caused by gland destruction. Several other causes for central DI have also been proposed. Here we present a case of transient central DI after discontinuation of vasopressin used for septic shock without evidence of overt pituitary damage in a cystic fibrosis patient. The serum sodium concentration peaked at 137 mmol/L in the setting of polyuria within 3 days of vasopressin discontinuation without other identified alternative etiologies. Sodium levels and urine output trended down dramatically with desmopressin administration.

Refractory Shock Secondary to Denosumab-induced Severe Hypocalcaemia.

Denosumab is one of the most commonly used antiresorptive drugs for osteoporosis treatment and the prevention of skeletal-related events in cancer patients. The purpose of this case report is to highlight potentially life-threatening severe hypocalcaemia as a side effect of denosumab complicated by refractory shock that failed to respond to medical management including intravenous calcium, vasopressors and inotropes in an elderly man with a history of prostatic cancer. LEARNING POINTS: Denosumab is a commonly used antiresorptive drugs for the treatment of osteoporosis and to prevent skeletal-related events in patients with cancer.A common side effect of denosumab is hypocalcaemia; conditions associated with a higher risk of hypocalcaemia include chronic kidney disease, pre-existing hypocalcaemia, and metastatic cancer.Severe hypocalcaemia may induce cardiovascular manifestations such as hypotension, bradycardia, impaired cardiac contractility, impaired vascular tone, and arrhythmias.Shock results from diminished vascular smooth muscle tone and tends to occur with rapid severe hypocalcaemia; it is usually refractory to fluid and pressor therapy until hypocalcaemia is corrected.

Usefulness of statins in end-stage renal disease.

End-stage renal disease (ESRD) is considered an independent risk factor of cardiovascular and cerebrovascular events. This review highlights atherosclerotic risk, lipid metabolism alterations, and four studies on the use of statins in ESRD-two of which showed a statistically significant effect of statins on the primary endpoints and two of which did not. Since effects were seen with higher doses of statins, further research is needed on the protective effects of intermediate to higher doses of statins in ESRD patients.

Urinary Cellular Profile as a Biomarker for Lupus Nephritis.

BACKGROUND/OBJECTIVE: A search for the ideal biomarker for lupus nephritis (LN) is still underway, one that can be used for early detection and correlate with the class and activity of LN. Urine is normally devoid of leukocytes; however, it has been observed that macrophages and T lymphocytes are routinely present in the urine of LN patients and those with other proliferative renal diseases. This provides the idea for their potential use as biomarkers for proliferative LN. Here, we measured the urinary CD4+, CD8+ T lymphocytes, and CD14+ monocytes in patients with systemic lupus erythematosus (SLE) as potential biomarkers for LN. METHODS: A longitudinal case-control study included 30 SLE patients with LN, 30 SLE patients without past or current LN, and 20 healthy subjects as a control group. The flow cytometric analysis was done using BD FACS Calibur multiparameter flow cytometer equipped with BD CellQuest Pro software for data analysis. RESULTS: CD14+ cells were the most abundant cells in the urine of LN patients. The mean numbers of urinary CD8+, CD4+, and CD14+ cells/mL were significantly higher in patients with LN than in those without. The cell counts correlated significantly with proteinuria. Urinary CD14+ cells seem to occur in much higher counts in class IV than class III LN. CONCLUSIONS: Urinary CD8+, CD4+, and CD14+ cells are highly sensitive and specific markers for detecting proliferative LN. A low CD4:CD8 ratio provides a further clue. Urinary CD14 cell counts may be a potential biomarker to differentiate between the different classes of proliferative LN.

Coronavirus Disease 2019 Myocarditis: Insights into Pathophysiology and Management.

The world is dealing with a global pandemic of severe acute respiratory coronavirus 2 (SARS-CoV-2). Coronavirus disease 2019 (COVID-19), which is the illness caused by SARS-CoV-2, is overwhelming healthcare systems around the world. Although the main clinical manifestations of COVID-19 are respiratory symptoms, several reports have noted myocarditis, cardiomyopathy, arrhythmias and cardiac arrests as COVID-19 complications. Here, the authors highlight the current understanding of the pathophysiology of myocarditis related to COVID-19 and its management.

Ortner's syndrome due to large bilharzial pulmonary artery aneurysm.

Ortner's syndrome (cardiovocal syndrome) is a rare cause of unilateral vocal cord paralysis that results from compression of the left recurrent laryngeal nerve by enlarged vascular structures. Most commonly, it is caused by an enlarged left atrium, but other rare causes include ascending aortic aneurysm or pulmonary artery aneurysm. Although very rarely encountered nowadays, bilharziasis is still one of the main causes of pulmonary artery aneurysm in endemic areas. Hereby, we report a case of Ortner's syndrome due to a large pulmonary artery aneurysm in a woman with history of bilharziasis.

COVID-19 discharge and follow-up recommendations.

Currently, the world is facing a global pandemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), also known as COVID-19. So far, there are no clear recommendations regarding hospital discharge and aftercare for COVID-19. Here, we briefly discuss the current understanding of recommendations for discharge criteria, discharge treatment regimens, and follow-up.

Aortic valve perforation in the setting of Cogan's syndrome.

Cogan's syndrome is a rare disorder characterized by the coexistence of ocular and audio-vestibular manifestations. Systemic manifestations are quite unusual with pan-vasculitis and cardiac involvement reported in the form of aortitis with aortic aneurysm, dissection, or extremely rare aortic valve perforation. Hereby, we report a case of a 56-year-old woman presented with ocular, audio-vestibular, and systemic manifestations with medium-sized vasculitis in the form of multiple splenic artery aneurysms, superior mesenteric artery thrombosis, and cardiovascular involvement in the form of aortic regurgitation due to noncoronary cusp perforation. To the best of our knowledge, this is the second case to report aortic perforation in the setting of Cogan's syndrome.