English Journal

Autoantibody stabilization of the classical pathway C3 convertase leading to C3 deficiency and Neisserial sepsis: C4 nephritic factor revisited.

Miller EC1, Chase NM, Densen P, Hintermeyer MK, Casper JT, Atkinson JP.Author information 1Division of Rheumatology, Department of Medicine, Washington University in St. Louis, St. Louis, MO 63110, USA.AbstractC3 deficiency is a rare disorder that leads to recurrent pyogenic infections. Here we describe a previously healthy 18 y/o Caucasian male with severe meningococcal disease. Total hemolytic activity was zero secondary to an undetectable C3. The C3 gene was normal by sequencing. Mixing the patient's serum with normal human serum led to C3 consumption. An IgG autoantibody in the patient's serum was identified that stabilized the classical pathway C3 and C5 convertases, thus preventing decay of these enzyme complexes. This autoantibody is an example of a C4 nephritic factor, with an additional feature of stabilizing the C5 convertase. Previous patients with C4 nephritic factor had membranoproliferative glomerulonephritis. Two years after presentation, this patient's C3 remains undetectable with no evidence of renal disease. We revisit the role of autoantibodies to classical pathway convertases in disease, review the literature on C4-NeF and comment on its detection in the clinical laboratory.
Clinical immunology (Orlando, Fla.).Clin Immunol.2012 Dec;145(3):241-50. doi: 10.1016/j.clim.2012.09.007. Epub 2012 Sep 28.
C3 deficiency is a rare disorder that leads to recurrent pyogenic infections. Here we describe a previously healthy 18 y/o Caucasian male with severe meningococcal disease. Total hemolytic activity was zero secondary to an undetectable C3. The C3 gene was normal by sequencing. Mixing the patient's s
PMID 23117396

Health-related quality of life in patients with systemic lupus erythematosus and proliferative lupus nephritis.

Daleboudt GM1, Berger SP, Broadbent E, Kaptein AA.Author information 1Department of Medical Psychology, Leiden University Medical Center, Leiden, The Netherlands. g.m.n.daleboudt@lumc.nlAbstractThis study investigated the influence of two different treatments for a kidney inflammation (i.e. proliferative lupus nephritis) on health-related quality of life (HRQoL) in patients with the chronic auto-immune disease systemic lupus erythematosus (SLE). One treatment protocol, the National Institutes of Health (NIH) protocol, was characterized by a high dose of cyclophosphamide (CYC, an immunosuppressive drug), and the second treatment, the Euro-Lupus protocol, involved a low-dose CYC. Thirty-two SLE patients were included based on the received treatment for an episode of proliferative lupus nephritis, according to either the Euro-Lupus or the NIH protocol. The two groups were compared on HRQoL as measured by the SF-36 and the SLE Symptom Checklist (SSC). The Euro-Lupus group (N = 16) tended to show a higher HRQoL than the NIH group (N = 16) on four of seven scales of the SF-36. In addition, the Euro-Lupus group experienced less burden from nausea or vomiting than the NIH group as assessed by the SSC. Fatigue was the most disturbing symptom in both groups. The most burdensome aspects of treatment were related to chemotherapy (55.2%) and use of prednisone (34.5%). Patients with a low HRQoL and high levels of fatigue were more likely to have low levels of serum complement C4 (i.e. elevated immune activity). In conclusion, patients who are treated according to the Euro-Lupus protocol may experience a higher HRQoL than patients who receive the NIH treatment. However, chemotherapy remains burdensome in the low-dose treatment regimen. Potential interventions to further enhance the HRQoL in SLE patients with proliferative lupus nephritis are discussed.
Psychology, health & medicine.Psychol Health Med.2011 Aug;16(4):393-404. doi: 10.1080/13548506.2011.554566.
This study investigated the influence of two different treatments for a kidney inflammation (i.e. proliferative lupus nephritis) on health-related quality of life (HRQoL) in patients with the chronic auto-immune disease systemic lupus erythematosus (SLE). One treatment protocol, the National Institu
PMID 21749237

[Membranoproliferative glomerulonephritis: Pathogenesis, pathophysiology and therapy].

Ohi H.
Nihon rinsho. Japanese journal of clinical medicine.Nihon Rinsho.2006 Feb;64 Suppl 2:413-6.
PMID 16523924

Japanese Journal

簡便なＣ４　Ｎｅｐｈｒｉｔｉｃ　ｆａｃｔｏｒ（Ｃ４ＮｅＦ）の検出法

日本腎臓学会誌 30(10), 1189-1195, 1988
NAID 130004063854

C3 nephritic factorならびにC4 nephritic factorのみいだされた2症例の検討

日本臨床免疫学会会誌 6(6), 584-589, 1983
NAID 130000848821

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