A case report of efficiency of double filtration plasmapheresis in treatment of Goodpasture's syndrome.

Published on Aug 1, 2009in Therapeutic Apheresis and Dialysis1.041
· DOI :10.1111/J.1744-9987.2009.00687.X
Goodpasture's syndrome is characterized by pulmonary hemorrhage, rapid progressive glomerulonephritis and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. Here, we report a case of Goodpasture's syndrome that we treated with double filtration plasmapheresis (DFPP) combined with immunosuppression therapy. The patient was a 32-year-old man with the main complaints of low-grade fever, general fatigue and dyspnea. The clinical diagnosis was renal-pulmonary syndrome based on pulmonary hemorrhage on chest X-ray, rapid progressive renal insufficiency, and elevated C-reactive protein (CRP). Goodpasture's syndrome was diagnosed because the patient was negative for MPO-ANCA and PR3-ANCA, and positive for anti-GBM antibodies. Renal biopsy showed crescentic glomerulonephritis. Hemodialysis, immunosuppression therapy (methylprednisolone and cyclophosphamide) and DFPP were performed. Anti-GBM antibodies were followed pre- and post-DFPP, and removal efficiency, cost performance and complications were evaluated. The antibody levels were 121 and 84 EU/mL before and after the first DFPP procedure, respectively, giving a removal efficiency of 24%. Subsequently, the removal efficiencies were 52%, 55% and 60% after the second, third and fourth DFPP procedures. For comparison, the immunoglobulin G (IgG) removal efficiencies were 53%, 57%, 60% and 55% after the four respective DFPP procedures; therefore, the removal efficiencies were similar for anti-GBM antibodies and IgG in all except the first DFPP procedure. The serum anti-GBM antibody and IgG concentrations decreased from pre- to post-DFPP, indicating that DFPP may be an effective therapeutic approach in Goodpasture's syndrome.
📖 Papers frequently viewed together
4 Citations
It is well known that anti-ABO antibodies in the recipient's serum cause hyperacute rejection in ABO-incompatible kidney transplantation. To perform successful ABO-incompatible kidney transplantation, temporary elimination of the anti-ABO antibodies from the recipient's serum is mandatory. Several methods of removing anti-ABO antibodies have been reported: plasmapheresis or immunoadsorption is widely employed in ABO-incompatible kidney transplantation. Two plasmapheresis methods are available, n...
44 CitationsSource
Systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens (ANCA) is the most frequent cause of rapidly progressive glomerulonephritis. Renal failure at presentation carries an increased risk for ESRD and death despite immunosuppressive therapy. This study investigated whether the addition of plasma exchange was more effective than intravenous methylprednisolone in the achievement of renal recovery in those who presented with a serum creatinine > 500 mu mol/L (5.8 mg/d...
758 CitationsSource
#1Victor Tuan Giam Chuang (University of Auckland)H-Index: 24
#2Masaki OtagiriH-Index: 73
: Human serum albumin (HSA) is responsible for 80% of the colloid osmotic pressure of plasma (25-33 mmHg). Its main clinical use is in maintaining colloid oncotic pressure and increasing circulating plasma volume with the typical dosage in excess of 10 g per dose. HSA is isolated by fractionating human plasma, which entails possible contamination by viruses or prions. Recombinant HSA (rHSA) has been successfully produced using a methylotrophic yeast, Pichia pastoris. Due to the fact that the cli...
47 CitationsSource
#1Yoshitaka Uji (Shiga University of Medical Science)H-Index: 6
#2Tomoharu Shimizu (Shiga University of Medical Science)H-Index: 26
Last. Toru Tani (Shiga University of Medical Science)H-Index: 12
view all 6 authors...
: This case describes a 40-year-old man complaining of general malaise, dyspnea with hemoptysis and anuria. Laboratory data indicated renal failure and the presence of systemic inflammation. His chest radiograph and computed tomography showed bilateral diffuse interstitial alveolar infiltration. These findings indicated acute deterioration of chronic renal dysfunction complicated by interstitial pneumonitis. He initially received daily conventional hemodialysis (HD), an antibiotic and oxygen the...
4 CitationsSource
#1Hirotsugu Iwatani (Osaka University)H-Index: 16
#2Takashi UzuH-Index: 50
Last. Atsushi YamauchiH-Index: 25
view all 9 authors...
We report a case of a 41-year-old Japanese man who presented with rapidly progressive glomerulonephritis, chronic sinusitis, and positive cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA). Renal biopsy showed crescentic glomerulonephritis, and he was diagnosed as having Wegener’s granulomatosis. During the clinical course, he suffered from pulmonary bleeding, and combination therapy of steroid, immunosuppressant, and double filtration plasmapheresis (DFPP) was started. He rapidly entered ...
16 CitationsSource
#1Billy G. Hudson (Vandy: Vanderbilt University)H-Index: 77
#2Karl Tryggvason (KI: Karolinska Institutet)H-Index: 67
Last. Eric G. Neilson (Vandy: Vanderbilt University)H-Index: 82
view all 4 authors...
Defects in type IV collagen, a collagenous protein involved in the formation of basement membranes, have been implicated in hereditary Alport's syndrome and acquired Goodpasture's syndrome. Mutations in genes corresponding to the building blocks of type IV collagen cause Alport's syndrome, whereas autoantibodies against structures that are usually hidden in the recesses of collagen IV cause Goodpasture's syndrome.
716 CitationsSource
#1Walter Samtleben (LMU: Ludwig Maximilian University of Munich)H-Index: 20
#2Nouhad Mistry-Burchardi (LMU: Ludwig Maximilian University of Munich)H-Index: 5
Last. T. Bosch (LMU: Ludwig Maximilian University of Munich)H-Index: 11
view all 5 authors...
: The potential to treat life-threatening conditions with therapeutic plasma exchange (TPE) is limited to a few situations. In severe pulmonary hemorrhage as a complication of several immune disorders (e.g., antiglomerular basement membrane antibody disease, Wegener's granulomatosus, lupus erythematosus), TPE should only be considered after conventional measures (mostly pulses of methylprednisolone) have been applied. Idiopathic familial and nonfamilial thrombotic thrombocytopenic purpura as wel...
34 CitationsSource
#1Klaus Laczika (University of Vienna)H-Index: 25
#2Sylvia Knapp (University of Vienna)H-Index: 55
Last. Wilfred Druml (University of Vienna)H-Index: 57
view all 6 authors...
Abstract Patients with Goodpasture's syndrome presenting with dialysis-dependent end-stage renal failure at diagnosis almost never regain independent renal function. We report a patient with a 100% crescentic lesion in whom reversal of dialysis dependence was achieved by immunoadsorption together with immunosuppression. In a second patient, early initiation of immunoadsorption was able to completely restore normal renal function as early as 1 month after the start of treatment. These data give e...
42 CitationsSource
#1Andre A. Kaplan (University of Connecticut Health Center)H-Index: 21
: Therapeutic plasma exchange (TPE) has been widely accepted as a successful means of removing the antiglomerular basement membrane (anti-GBM) antibodies that result in the rapidly progressive glomerulonephritis (RPGN) of Goodpasture's syndrome. TPE has also been investigated as a means of removing the immune complexes associated with the glomerulonephritides of systemic lupus erythematosus, IgA nephropathy, Henoch Schonlein purpura, and cryoglobulinemia. Recently, an antineutrophil cytoplasmic ...
13 CitationsSource
#1Frank Merkel (FAU: University of Erlangen-Nuremberg)H-Index: 6
#2O. Pullig (FAU: University of Erlangen-Nuremberg)H-Index: 2
Last. M. Weber (FAU: University of Erlangen-Nuremberg)H-Index: 8
view all 5 authors...
Anti-basement membrane antibody (anti- BM Ab) mediated disease is reported to be a rare disorder frequently leading to severe deterioration of renal function. It was our purpose to work out para- meters necessary to predict the outcome reliably and to examine, who will benefit most from therapy. Data from 35 patients were evaluated retrospectively. Diagnosis was based on the detection of linear IgG staining (n = 28) along the glomerular basement membrane (GBM) in renal biopsies and/or on the dem...
83 CitationsSource
Cited By12
#1Joseph E. Schwartz (Columbia University)H-Index: 124
#2Jeffrey L. Winters (Mayo Clinic)H-Index: 53
Last. Beth H. Shaz (Emory University)H-Index: 41
view all 10 authors...
The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the Committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Seventh Edition of the JCA Special Issue continues to maint...
1,177 CitationsSource
#1Yoshihide FujigakiH-Index: 2
#2Chikayuki MorimotoH-Index: 4
Last. Shunya Uchida (Teikyo University)H-Index: 35
view all 12 authors...
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 ant...
1 CitationsSource
#1Joseph E. Schwartz (CUMC: Columbia University Medical Center)H-Index: 124
#2Anand Padmanabhan (MCW: Medical College of Wisconsin)H-Index: 18
Last. Beth H. Shaz (Emory University)H-Index: 41
view all 10 authors...
157 CitationsSource
#1China NaganoH-Index: 7
#2Yoshimitu GotoH-Index: 1
Last. Yoshiyuki KuroyanagiH-Index: 3
view all 4 authors...
Background Anti-glomerular basement membrane (GBM) antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis caused by autoantibodies against the α3-chain of type IV collagen in the GBM.
6 CitationsSource
#1Yiyan Zhang (NU: Nanjing University)H-Index: 3
#2Zheng Tang (NU: Nanjing University)H-Index: 16
Last. Zhihong Liu (NU: Nanjing University)H-Index: 65
view all 6 authors...
Background Double filtration plasmapheresis (DFPP) and (IA) are both used to clear antibody. However, the clinical efficacy and safety of DFPP in patients with anti-glomerular basement membrane (anti-GBM) disease are unclear.
35 CitationsSource
#1Joseph E. Schwartz (Columbia University)H-Index: 124
#2Jeffrey L. Winters (Mayo Clinic)H-Index: 53
Last. Beth H. Shaz (Emory University)H-Index: 41
view all 10 authors...
416 CitationsSource
#1Liqun Jiao (Capital Medical University)H-Index: 10
#2JF FanH-Index: 1
Last. Y ShenH-Index: 1
view all 4 authors...
Background: Good pasture syndrome (GPS) has been paid much attention recently for the dangerous illnessand high mortality. Objective: To investigate the efficiency of plasma exchange (PE) to treat Goodpasture syndrome (GPS) in children associated with Turner’s syndrome. Method: We report a case of a 15 year old female with GPS and Turner’s syndrome. The patient has intermittent fever and cough for 45 days and oliguria for 6 days. Turner’s syndrome was determined through blood karyotype analysis,...
4 CitationsSource
Therapeutic apheresis (TPE) is also known as plasmapheresis or therapeutic plasma exchange. Plasmapheresis is an extracorporeal blood clearance method. It may be explained as the extracorporeal blood's being separated to two parts as plasma and shaped elements of the blood and passing from a second membrane thereafter and transferring to body again after the substances which are wanted to be removed had been separated. More than 30 years ago, the first applications were made using therapeutic ap...
#1Timothy J. VeceH-Index: 8
Last. Leland L. FanH-Index: 41
view all 4 authors...
Abstract Diffuse alveolar hemorrhage most commonly refers to bleeding from the low-pressure pulmonary vascular bed and is a rare cause of chronic interstitial lung disease in children. Patients can present acutely with respiratory failure or more indolently with a history of worsening exercise intolerance and cough. Diffuse alveolar hemorrhage is divided into two broad categories: immune mediated and nonimmune mediated with immune-mediated hemorrhage being more common. The most common causes of ...
7 CitationsSource
#1Yalcin SolakH-Index: 28
#2Huseyin AtalayH-Index: 15
Last. Mehdi YeksanH-Index: 18
view all 7 authors...
Plasmapheresis is an extracorporeal procedure, which is often employed to rapidly lower circulating titers of autoantibodies, immune complexes or toxins. There are two types of plasmapheresis namely, regular plasmapheresis (RPP) by centrifugation and membrane fi ltration, and double fi ltration plasmapheresis (DFPP) which is a special form of membrane fi ltration in which two membranes called as plasma separator and plasma fractionator are employed to fi lter macromolecules more selectively. DFP...