- Pulmonary vasculitis. [Review]
- JTJ Thorac Dis 2018; 10(9):5560-5575
- Systemic vasculitides frequently affect the pulmonary vasculature. As the signs and symptoms of pulmonary vasculitis are variable and nonspecific, diagnosis and treatment represent a real challenge. ...
Systemic vasculitides frequently affect the pulmonary vasculature. As the signs and symptoms of pulmonary vasculitis are variable and nonspecific, diagnosis and treatment represent a real challenge. Vasculitides should be given consideration, as these diseases present severe manifestations of rapidly progressing pulmonary disease. Examining other organs usually affected by vasculitides (e.g., the skin and kidneys) and determining autoantibody levels are essential to a better management of the disease. A radiological study would also contribute to establishing a diagnosis. The lungs are commonly involved in small-vessel vasculitis, anti-glomerular basement membrane disease, and vasculitides associated with antineutrophil cytoplasmic antibodies. Associated life-threatening diffuse alveolar haemorrhages and irreversible damage to other organs-usually the kidneys-are severe complications that require early diagnosis. Vasculitides are rare diseases that affect multiple organs. An increasing number of treatments-including biological agent-based therapies-requiring cooperation between specialists and centers have become available in the recent years. In the same way, clinicians should be familiar with the complications associated with immunosuppressive therapies.
- Alveolar Hemorrhage in Vasculitis (Primary and Secondary). [Journal Article]
- SRSemin Respir Crit Care Med 2018; 39(4):482-493
- Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast ...
Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Bronchoalveolar lavage, retrieving bright red fluid, is the best diagnostic clue, also excluding infection and other causes of hemoptysis. Although not recommended, lung biopsy is the gold standard for the diagnosis of DAH and pulmonary capillaritis. Pulmonary capillaritis may be primary as in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis or secondary to drugs (especially antithyroid drugs such as propylthiouracil), infections, connective tissue diseases especially systemic lupus erythematosus, or other small vessel vasculitides. Newer toxic causes of drugs of abuse may be difficult to diagnose. Granulomatosis with polyangiitis and microscopic polyangiitis are the most common causes of capillaritis and DAH, whereas DAH is extremely rare in eosinophilic granulomatosis with polyangiitis. When pulmonary capillaritis is not secondary to underlying systemic vasculitis, idiopathic pauci-immune pulmonary capillaritis may be considered, with or without ANCA. Supportive treatment strategy is mandatory in all cases of DAH. Mechanical ventilation and extracorporeal membrane oxygenation may be used in severe cases. Early identification and removal of the putative drug is crucial in drug-induced vasculitis/DAH and may obviate the need for immunosuppressive therapy. High-dose corticosteroids, intravenous cyclophosphamide, and recently rituximab are the mainstay of treatment in vasculitis. Plasma exchange is recommended in anti-glomerular basement membrane disease and in severe DAH associated with systemic lupus erythematosus and is used in selected cases in ANCA-associated vasculitis.
- Preoperative Management of the Geriatric Patient: Frailty and Cognitive Impairment Assessment. [Review]
- ACAnesthesiol Clin 2018; 36(4):599-614
- As the population ages, more geriatric patients will be presenting for surgical procedures. Preoperative evaluation seeks to assess patients for geriatric syndromes: frailty, sarcopenia, functional d...
As the population ages, more geriatric patients will be presenting for surgical procedures. Preoperative evaluation seeks to assess patients for geriatric syndromes: frailty, sarcopenia, functional dependence, and malnutrition. Age-related changes in physiology increase risk for central nervous system, cardiovascular, pulmonary, renal, hepatic, and endocrine morbidity and mortality. Identification of various comorbidities allows for preoperative optimization and for opportunities for intervention including nutritional supplementation and prehabilitation, which may improve postoperative outcomes.
- Anti-glomerular basement membrane disease: an update on subgroups, pathogenesis and therapies. [Journal Article]
- NDNephrol Dial Transplant 2018 Oct 29
- Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis with or without pulmonary haemorrhage; however, there are several variants ...
Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis with or without pulmonary haemorrhage; however, there are several variants and vigilance is necessary to make a correct diagnosis. Such variants include overlap with anti-neutrophil cytoplasm antibodies-associated vasculitis and membranous nephropathy as well as anti-GBM occurring de novo after renal transplantation. Moreover, patients can present with isolated pulmonary haemorrhage as well as with negative tests for circulating anti-GBM. Virtually all patients with anti-GBM disease have autoantibodies that react with two discrete epitopes on the α3 chain of type IV collagen. Recent evidence suggests that healthy persons have low-affinity natural antibodies reacting with the same epitopes, but most people are protected from developing disease-causing high-affinity autoantibodies by human leukocyte antigen-dependent regulatory T-cells (Tregs). The α3 chain-derived peptides presented by the HLA-DR15 antigen lack the ability to promote the development of such Tregs. The detection of anti-GBM in circulation using the rapid assay test has led to early diagnosis and improved prognosis. However, our present tools to curb the inflammation and to eliminate the assaulting antibodies are insufficient. Only about one-third of all patients survive with functioning native kidneys. More effective therapies need to be developed; agents that inhibit neutrophil recruitment, deplete B cells and cleave immunoglobulin G (IgG) in vivo may become new weapons in the arsenal to combat anti-GBM disease.
- Membranous nephropathy followed by anti-glomerular basement disease: A case report and review of clinical presentation and treatment. [Journal Article]
- SOSAGE Open Med Case Rep 2018; 6:2050313X18807621
- Membranous nephropathy is a common cause of nephrotic syndrome in adults and can be primary or secondary through autoimmune disease, medication, infection, or malignancy. Rapidly progressive glomerul...
Membranous nephropathy is a common cause of nephrotic syndrome in adults and can be primary or secondary through autoimmune disease, medication, infection, or malignancy. Rapidly progressive glomerulonephritis with crescent formation is rare in patients with membranous nephropathy. Thus, in cases with rapid decline in renal function, after excluding complications such as malignant hypertension, acute hypersensitivity interstitial nephritis, and bilateral renal vein thrombosis, the simultaneous occurrence of a superimposed glomerulonephritis should be considered. We report a 55-year-old man suffering from a biopsy-confirmed primary membranous nephropathy, who developed rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibodies after being affected with membranous nephropathy for 8 years. The kidney biopsy revealed a concurrence of membranous nephropathy and anti-glomerular basement membrane disease. Clinical presentation and treatment of membranous nephropathy followed by anti-glomerular basement membrane disease are discussed based on our observation with promising follow-up.
- Falsely positive anti-glomerular basement membrane antibodies in a patient with hantavirus induced acute kidney injury - a case report. [Journal Article]
- BNBMC Nephrol 2018 Oct 22; 19(1):286
- CONCLUSIONS: Hantavirus infection may induce anti-GBM antibodies, falsely suggestive of anti-GBM glomerulonephritis. Anti-GBM antibodies are supposed to be 100% specific. No earlier reports of false positive anti-GBM titers were reported. Nevertheless, the anti-GBM antibodies in this case were seen as an innocent bystander effect. Considering the need of urgent initiation of plasmapheresis and administration of immunosuppressants it may lead to diagnostic dilemmas with crucial therapeutic consequences. Knowledge of this anomaly when diagnosing acute renal failure, is very important.
- Properdin binds independent of complement activation in an in vivo model of anti-glomerular basement membrane disease. [Journal Article]
- KIKidney Int 2018 Oct 10
- Properdin is the only known positive regulator of complement activation by stabilizing the alternative pathway convertase through C3 binding, thus prolonging its half-life. Recent in vitro studies su...
Properdin is the only known positive regulator of complement activation by stabilizing the alternative pathway convertase through C3 binding, thus prolonging its half-life. Recent in vitro studies suggest that properdin may act as a specific pattern recognition molecule. To better understand the role of properdin in vivo, we used an experimental model of acute anti-glomerular basement membrane disease with wild-type, C3- and properdin knockout mice. The model exhibited severe proteinuria, acute neutrophil infiltration and activation, classical and alternative pathway activation, and progressive glomerular deposition of properdin, C3 and C9. Although the acute renal injury was likely due to acute neutrophil activation, we found properdin deposition in C3-knockout mice that was not associated with IgG. Thus, properdin may deposit in injured tissues in vivo independent of its main ligand C3.
- Clinicopathological features and outcome of antibody-negative anti-glomerular basement membrane disease. [Journal Article]
- JCJ Clin Pathol 2018 Oct 12
- CONCLUSIONS: Anti-GBM antibody-negative anti-GBM disease was different from classic anti-GBM disease clinically and pathologically. The pathogenesis of the renal injury in these patients has not been elucidated until now and it should be studied and identified further.
- Epidemiology of Cardiorenal Syndrome. [Review]
- ACAdv Chronic Kidney Dis 2018; 25(5):391-399
- Cardiorenal syndrome is a spectrum of disorders that emphasizes the bidirectional nature of cardiac and kidney injury. Observational and retrospective studies have helped us to understand the prevale...
Cardiorenal syndrome is a spectrum of disorders that emphasizes the bidirectional nature of cardiac and kidney injury. Observational and retrospective studies have helped us to understand the prevalence and burden of each of the 5 types of cardiorenal syndromes. Cardiorenal syndrome type 1 is the most common. The nature of epidemiologic data limits clear delineation between cardiorenal syndrome types 2 and 4. Overall, the presence of cardiac or renal dysfunction strongly predicts a poor outcome of the contrary organ.
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- Anti-glomerular basement membrane disease due to monoclonal IgG Lambda antibodies: a very rare case of monoclonal gammopathy of renal significance. [Letter]
- AHAnn Hematol 2018 Oct 09