Complement activation in Brazilian pemphigus foliaceus

Messias, I. T. De; Santamaria, J. N.; Ragiotto, R.; Doi, Elvira M.; Kajdacsy-Balla, André

doi:10.1111/j.1365-2230.1989.tb00883.x

Cited by 10 publications

(3 citation statements)

References 20 publications

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“…All patients had been hospitalized in the Hospital de Dermatologia SanitBria Sio Roque, in Piraquara, ParanB. Diagnosis was based on clinical and histopathological features, and could be confirmed by direct and indirect immunofluorescence in all 19 patients studied in this respect (Messias et al 1987). Furthermore, we studied three female PV patients of direct European ancestry: one had Italian and two had Ukranian and Polish parents.…”

Section: Methodsmentioning

confidence: 99%

Are HLA class II genes controlling susceptibility and resistance to Brazilian pemphigus foliaceus (fogo selvagem)?

Petzl-Erler

Santamaria²

1989

Tissue Antigens

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In order to investigate the possible association between Brazilian pemphigus foliaceus and HLA, we studied 48 patients and 74 matched controls, all Brazilian Caucasoids, for HLA-A,B,C; DR1 to DRw8 and DQw1 to DQw3. The frequencies of DR1, DR4 and B16 were significantly increased, while DR7 was significantly decreased among the patients. Furthermore DQw2, likewise the DR specificities associated with it - DR3 and DR7 - never occurred among the patients in the absence of the susceptibility markers DR1, DQw1 or DR4, DQw3. Acting on these findings, we suggest that at least two MHC-class II genes are involved in the pathogenesis of Brazilian pemphigus foliaceus: at least one gene, associated to DR1,DQw1 and to DR4,DQw3, confers susceptibility and at least one gene, associated to DR7,DQw2 and DR3,DQw2, confers resistance. The susceptibility gene(s) seem(s) to be epistatic to or dominant over (if allelic) the resistance gene(s). Both are dominant over other alleles at their locus (or loci).

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Section: Methodsmentioning

confidence: 99%

Are HLA class II genes controlling susceptibility and resistance to Brazilian pemphigus foliaceus (fogo selvagem)?

Petzl-Erler

Santamaria²

1989

Tissue Antigens

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“…Endemic pemphigus foliaceus (EPF) is a unique example of a disease that is both autoimmune and endemic. Although complement is not required for blister formation in pemphigus, 1 evidence has indicated a role for complement in the physiopathology of the disease 2,3 . Mannan‐binding lectin (MBL) is a plasma protein with a key role in innate immunity.…”

Section: Mbl and Masp‐2 Circulating Levels In Patients With Endemic mentioning

confidence: 99%

Circulating levels of mannan-binding lectin (MBL) and MBL-associated serine protease 2 in endemic pemphigus foliaceus

Messias-Reason¹,

Bosco²,

Nisihara³

et al. 2008

Clin Exp Dermatol

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Endemic pemphigus foliaceus (EPF) is an autoimmune disease, which occurs in Brazil and other regions of South America. Mannose-binding lectin (MBL) and MBL-associated serine protease (MASP-2) play a key role in innate immunity, and its deficiency has been related to increased susceptibility to infection and autoimmune diseases. MBL and MASP-2 serum levels were measured in 114 patients with EPF and in 100 healthy individuals in Brazil. MBL and MASP-2 levels were measured by sandwich assays (time-resolved immunofluorimetic assay) using monoclonal antibodies. No difference was observed in the MBL level in patients with EPF compared with controls [mean +/- SEM 1230.07 +/- 132.18 ng/mL (median 789.0 ng/mL) vs. 1036.98 +/- 117.99 ng/mL (median 559.5 ng/mL), P = 0.32]. Non-significant lower MASP-2 levels were observed in EPF [274.34 +/- 15.66 ng/mL (median 239.5 ng/mL ) vs. 304.72 +/- 15.28 ng/mL [median 261.0 ng/mL ), P = 0.06]. MBL deficiency (< 10 ng/mL) or MASP-2 deficiency (< 100 ng/mL) did not differ significantly between patients and controls. These data indicate that MBL and MASP-2 deficiency are not associated with susceptibility to EPF.

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“…CD4 + T cells of PF patients present upregulated C1QA gene expression, compared to controls ( 20 ). Protein levels and C1QA expression fall with therapeutic intervention ( 20 , 59 , 60 ). By contrast, anti-DSG1 IgG levels remain high during disease remission ( 61 , 62 ).…”

Section: Introductionmentioning

confidence: 99%

Sparking Fire Under the Skin? Answers From the Association of Complement Genes With Pemphigus Foliaceus

et al. 2018

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Skin blisters of pemphigus foliaceus (PF) present concomitant deposition of autoantibodies and components of the complement system (CS), whose gene polymorphisms are associated with susceptibility to different autoimmune diseases. To investigate these in PF, we evaluated 992 single-nucleotide polymorphisms (SNPs) of 44 CS genes, genotyped through microarray hybridization in 229 PF patients and 194 controls. After excluding SNPs with minor allele frequency <1%, out of Hardy–Weinberg equilibrium in controls or in strong linkage disequilibrium (r2 ≥ 0.8), 201 SNPs remained for logistic regression. Polymorphisms of 11 genes were associated with PF. MASP1 encodes a crucial serine protease of the lectin pathway (rs13094773: OR = 0.5, p = 0.0316; rs850309: OR = 0.23, p = 0.03; rs3864098: OR = 1.53, p = 0.0383; rs698104: OR = 1.52, p = 0.0424; rs72549154: OR = 0.55, p = 0.0453). C9 (rs187875: OR = 1.46, p = 0.0189; rs700218: OR = 0.12, p = 0.0471) and C8A (rs11206934: OR = 4.02, p = 0.0323) encode proteins of the membrane attack complex (MAC) and C5AR1 (rs10404456: OR = 1.43, p = 0.0155), a potent anaphylatoxin-receptor. Two encode complement regulators: MAC-blocking CD59 (rs1047581: OR = 0.62, p = 0.0152) and alternative pathway-blocking CFH (rs34388368: OR = 2.57, p = 0.0195). One encodes opsonin: C3 (rs4807895: OR = 2.52, p = 0.0239), whereas four encode receptors for C3 fragments: CR1 (haplotype with rs6656401: OR = 1.37, p = 0.0382), CR2 (rs2182911: OR = 0.23, p = 0.0263), ITGAM (CR3, rs12928810: OR = 0.66, p = 0.0435), and ITGAX (CR4, rs11574637: OR = 0.63, p = 0.0056). Associations reinforced former findings, regarding differential gene expression, serum levels, C3, and MAC deposition on lesions. Deregulation of previously barely noticed processes, e.g., the lectin and alternative pathways and opsonization-mediated phagocytosis, also modulate PF susceptibility. The results open new crucial avenues for understanding disease etiology and may improve PF treatment through additional therapeutic targets.

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Complement activation in Brazilian pemphigus foliaceus

Cited by 10 publications

References 20 publications

Are HLA class II genes controlling susceptibility and resistance to Brazilian pemphigus foliaceus (fogo selvagem)?

Are HLA class II genes controlling susceptibility and resistance to Brazilian pemphigus foliaceus (fogo selvagem)?

Circulating levels of mannan-binding lectin (MBL) and MBL-associated serine protease 2 in endemic pemphigus foliaceus

Sparking Fire Under the Skin? Answers From the Association of Complement Genes With Pemphigus Foliaceus

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