• ANCA-associated vasculitis
• Anti-GBM disease
• Crescentic glomerulonephritis
• Double-positive patients (DPPs)
• PRES

• Double-filtration plasmapheresis
• adverse events
• clinical efficacy
• hybrid
• severe lupus nephritis

• Humans
• Anti-Glomerular Basement Membrane Disease
• Prognosis
• Immunosuppressive Agents
• Basement Membrane

Besides conventional medical therapies, therapeutic apheresis has become an important adjunctive or alternative therapeutic option to immunosuppressive agents for primary or secondary kidney diseases and kidney transplantation. The available therapeutic apheresis techniques used in kidney diseases, including plasma exchange, double-filtration plasmapheresis, immunoadsorption, and low-density lipoprotein apheresis. Plasma exchange is still the leading extracorporeal therapy. Recently, growing evidence supports the potential benefits of double-filtration plasmapheresis and immunoadsorption for more specific and effective clearance of pathogenic antibodies with fewer side effects. However, more randomized controlled trials are still needed. Low-density lipoprotein apheresis is also an important supplementary therapy used in patients with recurrent focal segmental glomerulosclerosis. This review collects the latest evidence from recent studies, focuses on the specific advantages and disadvantages of these techniques, and compares the discrepancy among them to determine the optimal therapeutic regimens for certain kidney diseases.

• Therapeutic apheresis
• double-filtration plasmapheresis
• immunoadsorption
• kidney transplantation
• plasma exchange

• Anti-GBM
• Children
• Glomerulonephritis

• Adrenal Cortex Hormones
• Anti-Glomerular Basement Membrane Disease/diagnosis
• Anti-Glomerular Basement Membrane Disease/therapy
• Autoantibodies
• Child
• Collagen Type IV
• Glomerular Basement Membrane/pathology
• Hemorrhage
• Humans
• Nephritis

Double-filtration plasmapheresis (DFPP) is a blood cleaning technique that enables the removal of unwanted substances from the blood. In our case study, we performed near-infrared (NIR) spectroscopy measurements on the human hand tissue before and after a specific DFPP treatment (INUSpheresis with a TKM58 filter), along with NIR measurements of the substances extracted via DFPP (eluate). The spectral data were analyzed using the aquaphotomics approach. The analysis showed that the water properties in the tissue change after DFPP treatment, i.e., an increase in small water clusters, free water molecules and a decrease in hydroxylated water as well as superoxide in hydration shells was noted. The opposite effect was observed in the eluates of both DFPP treatments. Our study is the first that documents changes in water spectral properties after DFPP treatments in human tissue. The changes in tissue water demonstrated by our case study suggest that the positive physiological effects of DFPP in general, and of INUSpheresis with the TKM58 filter in particular, may be associated with improvements in water quality in blood and tissues.

• INUSpheresis
• aquaphotomics
• double-filtration plasmapheresis
• near-infrared spectroscopy
• water

• Filtration
• Humans
• Plasmapheresis/methods

Anti-glomerular basement membrane (anti-GBM) disease is a rare but serious autoimmune disease, which is characterized by the development of pathogenic antibodies to type IV collagen antigens in the glomerular and alveolar basement membranes. This results in rapidly progressive glomerulonephritis (GN), alveolar hemorrhage, or both. A variety of environmental factors can trigger the disease in genetically predisposed patients. Temporal associations with influenza, SARS-CoV-2 infection, and COVID-19 vaccination have been described although there is insufficient evidence to suggest causality. Anti-GBM disease accounts for approximately 20% of the cases of rapidly progressive GN cases secondary to crescentic GN, but is an uncommon cause of end-stage kidney disease. Early diagnosis by detection of circulating antibodies, increased awareness of atypical as well as complex clinical variants of the disease, and combined therapy with immunosuppression and plasma exchange has improved the prognosis of patients with this potentially fatal disease. Progress has been hampered by the rarity of anti-GBM disease, but new agents and therapeutic regimens are emerging.

• Anti-GBM antibody
• Anti-glomerular basement
• Double positivity
• Membrane disease
• Plasmapheresis
• Rapidly progressive glomerulonephritis
• Rituximab

• clotting factors
• double-filtration plasmapheresis
• fibrinogen
• plasma filter

Nearly 18% of patients on a waiting list for kidney transplantation (KT) are highly sensitized, which make access to KT more difficult. We assessed the efficacy and tolerance of different techniques (plasma exchanges [PE], double-filtration plasmapheresis [DFPP], and immunoadsorption [IA]) to remove donor specific antibodies (DSA) in the setting of HLA-incompatible (HLAi) KT. All patients that underwent apheresis for HLAi KT within a single center were included. Intra-session and inter-session Mean Fluorescence Intensity (MFI) decrease in DSA, clinical and biological tolerances were assessed. A total of 881 sessions were performed for 45 patients: 107 DFPP, 54 PE, 720 IA. The procedures led to HLAi KT in 39 patients (87%) after 29 (15–51) days. A higher volume of treated plasma was associated with a greater decrease of inter-session class I and II DSA (p = 0.04, p = 0.02). IA, PE, and a lower maximal DSA MFI were associated with a greater decrease in intra-session class II DSA (p < 0.01). Safety was good: severe adverse events occurred in 17 sessions (1.9%), more frequently with DFPP (6.5%) p < 0.01. Hypotension occurred in 154 sessions (17.5%), more frequently with DFPP (p < 0.01). Apheresis is well tolerated (IA and PE > DFPP) and effective at removing HLA antibodies and allows HLAi KT for sensitized patients.

• desensitization
• donor specific antibody
• kidney transplantation
• plasmapheresis

• antibody-mediated rejection
• apheresis
• double filtration plasmapheresis
• kidney transplantation
• therapeutic plasma exchange

Systemic vasculitides represent uncommon conditions characterized by the inflammation of blood vessels that can lead to different complex disorders limited to one organ or potentially involving multiple organs and systems. Systemic vasculitides are classified according to the diameter of the vessel that they mainly affect (small, medium, large, or variable). The pathogenetic mechanisms of systemic vasculitides are still partly unknown, as well as their genetic basis. For most of the primary systemic vasculitides, a single gold standard test is not available, and diagnosis is often made after having ruled out other mimicking conditions. Current research has focused on new management protocol and therapeutic strategies aimed at improving long-term patient outcomes and avoiding progression to multiorgan failure with irreversible damage. In this narrative review, authors describe different forms of systemic vasculitides through a review of the literature, with the aim of highlighting the current knowledge and recent findings on etiopathogenesis, diagnosis and therapy.

• Behcet's disease
• Giant cell arteritis
• Kawasaki disease
• Polyarteritis nodosa
• Systemic vasculitides
• Takayasu arteritis

• ANCA
• anti-GBM disease
• autoantibodies
• crescentic glomerulonephritis
• glomerulonephritis

Sensitization to human leukocyte antigens (HLAs) has been one of the major clinical challenges for successful kidney transplantation. In end-stage renal disease, kidney transplantation provides benefits compared with dialysis in terms of improved patient survival better quality of life, and lower ongoing costs after the first year. Living donor kidney transplantation has an advantage with improved allograft survival, and performed earlier and electively compared with deceased donor transplantation. However sensitized patients are increasing in number on transplant waiting lists, and their prospect of getting a transplant is less than nonsensitized patients due to immunological incompatibility with the donor. Strategy for sensitized patients are listing for a compatible deceased donor transplant or, if they have a living donor, either selecting a kidney exchange program or undergoing a desensitization procedure. Desensitization procedures may be undertaken to increase access to either living or deceased donor transplants, and in some situations may also be employed to facilitate participation in a kidney exchange, in less immunological barrier to be overcome. The question of whether individuals are better off with a desensitization treatment followed by HLA-incompatible living donor transplantation or waiting on the deceased donor kidney transplant list for a compatible transplant has recently been addressed by two large multicenter studies, with conflicting results. A multicenter study from the United States published in the New England Journal of Medicine [365;318 326.2011] concluded that there was a strong survival benefit for sensitized patients undergoing desensitization followed by HLA-incompatible living donor kidney transplant compared with those remaining on the waiting list. Of interest, a second study, published in the Lancet, [389;727 734.2017] found no significant survival advantage for desensitized patients compared with similar patients remaining on the waiting list in the United Kingdom. Controversies still remain regarding how desensitization can be achieved and which techniques are effective and safe.

In this chapter various complications from the desensitization will be dealt with in current use of medications or armamentum.

• Antibody-mediated rejection
• Desensitization
• Double-filtration plasmapheresis
• Extracorporeal photopheresis
• Focal segmental glomerulosclerosis
• Immunoadsorption
• Kidney transplantation
• Therapeutic plasma exchange

• 紫癜性肾炎
• 双重血浆置换
• 甲泼尼龙
• 环磷酰胺
• 儿童
• henoch-schönlein purpura nephritis
• double filtration plasmapheresis
• methylprednisolone
• cyclophosphamide
• child

• Child
• Glucocorticoids
• Humans
• IgA Vasculitis
• Immunosuppressive Agents
• Nephritis
• Plasmapheresis

• 紫癜性肾炎
• 双重血浆置换
• 甲泼尼龙
• 环磷酰胺
• 儿童
• henoch-schönlein purpura nephritis
• double filtration plasmapheresis
• methylprednisolone
• cyclophosphamide
• child

• Child
• Glucocorticoids
• Humans
• IgA Vasculitis
• Immunosuppressive Agents
• Nephritis
• Plasmapheresis

Schwere, organ- oder lebensbedrohliche Manifestationen entzündlicher rheumatischer Erkrankungen, wie z. B. eine diffuse alveoläre Hämorrhagie im Rahmen einer Kleingefäßvaskulitis, sprechen nicht immer ausreichend oder mit zeitlicher Verzögerung auf eine immunsuppressive Therapie an. Bei einem drohenden oder bereits eingetretenen Organversagen besteht dann nicht selten die Notwendigkeit, die immunsuppressive Therapie auf der Intensivstation um rasch wirksame Rescue-Therapieverfahren zu ergänzen. Aufgrund der Seltenheit vieler rheumatischer Erkrankungen ist die Evidenz zum Einsatz von Rescue-Therapieverfahren wie der Plasmapherese, der extrakorporalen Membranoxygenierung (ECMO) oder der Gabe von intravenösen Immunglobulinen (IVIG) für viele Indikationen eher gering. Der Einsatz der Plasmapherese wird bei einer akuten Anti-GBM(glomeruläre Basalmembran)-Erkrankung (Goodpasture Syndrom) oder einem katastrophalen Antiphospholipidantikörpersyndrom (CAPS) als sinnvoll angesehen. Eine ECMO-Therapie kann bei persistierender respiratorischer Insuffizienz trotz mechanischer Beatmung als Folge einer diffusen alveolären Hämorrhagie oder eines Acute-Respiratory-Distress-Syndroms (ARDS) anderer Ursache erwogen werden. Eine Gabe von IVIG ist bei einer akuten kardialen Beteiligung im Rahmen einer Kawasaki-Erkrankung indiziert und kann beim CAPS sowie bei therapierefraktären Myositiden erwogen werden.

• Collagen vascular diseases
• Extracorporeal membrane oxygenation
• Intravenous immunoglobulins
• Plasmapheresis
• Vasculitis

• Anti-glomerular basement membrane autoantibodies
• Artérite à cellules géantes
• Auto-anticorps anti-membrane basale glomérulaire
• Giant cell arteritis
• Goodpasture's syndrome
• Pulmonary renal syndrome
• Syndrome de Goodpasture
• Syndrome pneumo-rénal

• Anti-Glomerular Basement Membrane Disease
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
• Autoantibodies
• Autoantigens
• Autoimmunity
• Capillaries
• Glomerular Basement Membrane
• Glomerulonephritis
• Hereditary
• Humans
• Immunology and pathology
• Inflammation
• Membranous
• Nephritis
• Plasma Exchange
• Plasmapheresis
• Prognosis
• Recurrence
• Renal Insufficiency
• Retrospective Studies
• glomerulonephritis
• kidney
• kidney transplantation
• renal dialysis
• vasculitis

• Animals
• Anti-Glomerular Basement Membrane Disease/diagnosis
• Anti-Glomerular Basement Membrane Disease/epidemiology
• Anti-Glomerular Basement Membrane Disease/immunology
• Anti-Glomerular Basement Membrane Disease/therapy
• Autoantibodies/immunology
• Autoimmunity
• Humans
• Immunosuppressive Agents/therapeutic use
• Kidney Glomerulus/drug effects
• Kidney Glomerulus/immunology
• Kidney Glomerulus/pathology
• Kidney Transplantation
• Plasma Exchange
• Predictive Value of Tests
• Recurrence
• Risk Factors
• Steroids/therapeutic use
• Treatment Outcome

• G0901997 Medical Research Council

• Cholestasis
• cirrhosis
• itch
• rifampicin

A 26-year-old man highly suspected of having antiglomerular basement membrane (GBM) disease was treated with corticosteroid pulse therapy 9 days after initial infection-like symptoms with high procalcitonin value. The patient required hemodialysis the next day of the treatment due to oliguria. In addition to corticosteroid therapy, plasmapheresis was introduced and the patient could discontinue hemodialysis 43 days after the treatment. Kidney biopsy after initiation of hemodialysis confirmed anti-GBM disease with 86.3% crescent formation. Physician should keep in mind that active anti-GBM disease shows even high procalcitonin value in the absence of infection. To pursue recovery of renal function, the challenge of the immediate and persistent treatment with high-dose corticosteroids plus plasmapheresis for highly suspected anti-GBM disease is vitally important despite the presence of reported predictors for dialysis-dependence including oliguria and requiring hemodialysis at presentation.

Goodpasture’s syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described.

The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours. An analytical study showed normocytic normochromic anemia with a hemoglobin level of 7.2 g/dL and leukocytosis with normal renal function and coagulation times. A blood transfusion was performed without complications. Chest computed tomography revealed a reticulonodular infiltrate of both lungs. Bronchoscopy showed no apparent lesions. Sputum cultures, rapid urine antigens for Legionella pneumophila and Streptococcus pneumoniae, studies for Influenza, virologic markers and serologic studies for autoimmunity were all negative. At the end of the tenth day his general state deteriorated with fatigue, hematuria, and in 3 days he developed aggravation of renal function with recurrent hemoptysis and anemia. Immunosuppression with daily prednisolone 1 g administered intravenously was initiated. An urgent bronchoscopy showed no lesions. A kidney biopsy showed fibrinoid necrosis and cellular crescents. Immunofluorescence revealed a linear immunoglobulin G deposition compatible with Goodpasture’s syndrome. Immunosuppressive therapy with daily cyclophosphamide 120 mg orally was added. Subsequently he was transferred to a referral center at which 21 sessions of plasmapheresis and four sessions of hemodialysis were performed with good response; he currently has no need of hemodialysis.

The absence of circulating anti-glomerular basement membrane antibodies in Goodpasture’s syndrome adds complexity to the diagnosis creating an unusual setting in a rare disease. In our case a kidney biopsy was essential for diagnosis and clinical approach. Studies have shown that early aggressive therapy leads to an improved prognosis. Physicians should consider tissue diagnoses such as bronchoscopy and kidney biopsy in pulmonary renal syndrome.

• Anti-GBM antibodies
• Case report
• GBM
• Glomerular basement membrane
• Glomerulonephritis
• Goodpasture’s syndrome
• Rapidly progressive glomerulonephritis
• Renal failure

• Adolescent
• Adrenal Cortex Hormones/therapeutic use
• Adult
• Combined Modality Therapy
• Drug Therapy, Combination
• Female
• Humans
• Immunosuppressive Agents/therapeutic use
• Lupus Nephritis/complications
• Lupus Nephritis/therapy
• Male
• Middle Aged
• Plasmapheresis
• Remission Induction/methods
• Retrospective Studies
• Severity of Illness Index
• Thrombotic Microangiopathies/complications
• Thrombotic Microangiopathies/therapy
• Treatment Failure
• Young Adult

• Antiphospholipid syndrome
• Complement
• Extracorporeal treatment
• Immunoadsorption
• Lupus nephritis
• Plasma exchange
• Plasmapheresis
• Refractory SLE
• Systemic lupus erythematosus

Rapidly progressive glomerulonephritis (RPGN) results from severe crescentic damage to glomeruli and leads to irreversible kidney failure if not diagnosed and managed in a timely fashion. Traditional treatment has relied on glucocorticoids and cyclophosphamide, with additional plasmapheresis for certain conditions. Here we describe updates in the management of RPGN, according to the underlying renal pathology. However, there remains a paucity of trials that have enrolled patients with more advanced renal disease, dialysis dependence or with RPGN, and we are therefore still reliant on extrapolation of data from studies of patients with a less severe form of disease. In addition, reporting bias results in publication of cases or cohorts showing benefit for newer agents in advanced disease or RPGN, but it remains unclear how many unsuccessful outcomes in these circumstances take place. Since clinical trials specifically in RPGN are unlikely, use of biologic registries or combination of sufficient sized cohort series may provide indications of benefit outside of a clinical trial setting and should be encouraged, in order to provide some evidence for the efficacy of therapeutic regimens in RPGN and advanced renal disease.

• AKI
• IgA nephropathy
• glomerulonephritis
• systemic lupus erythematosus
• vasculitis