The correlation of disease activity and antibody titers in pemphigus

Fitzpatrick, Randal E.

doi:10.1001/archderm.116.3.285

Cited by 43 publications

(22 citation statements)

References 2 publications

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“…However, none of these recent reports have described patients with PV limited to cutaneous involvement only. Furthermore, a rise in the antibody titers preceding clinical exacerbation has also been documented [39]. It is possible that if therapeutic intervention had not produced clinical recovery, the evolution of clinical disease, in these patients, could have progressed to involve mucosal disease.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic Features of Pemphigus vulgaris in Patients with Bullous Pemphigoid

Sami

Bhol²,

Beutner³

et al. 2002

Dermatology

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Background: The simultaneous presence of features of pemphigus vulgaris (PV) in patients with bullous pemphigoid (BP) has previously been reported in the literature. Objective: The purpose of this retrospective study is to present 13 patients with an initial diagnosis of BP, who subsequently demonstrated coexistent serological features of both BP and PV. Methods: The following information on each patient was documented, at the time of initial diagnosis: clinical profile on presentation, histology, direct immunofluorescence, indirect immunofluorescence (IIF) using monkey esophagus as substrate, salt-split skin (SSS) and an immunoblot assay. Since all 13 patients failed to respond to conventional systemic therapy, intravenous immunoglobulin (IVIg) was used as an alternative treatment modality. Prior to initiating IVIg therapy, in all 13 patients, serological studies were performed. In addition to IIF using monkey esophagus, an immunoblot assay and SSS, an enzyme-linked immunosorbent assay (ELISA) was performed to detect antibodies to desmogleins. These different assays were done to identify pathological autoantibodies typical of BP and PV. A control group of 25 healthy normal individuals, 37 patients with BP, 17 patients with PV and 12 patients with pemphigus foliaceus were used for comparison of serological studies. Results: At the time of initial presentation, histological and immunopathological studies confirmed the diagnosis of BP in all 13 patients. Prior to the initiation of IVIg therapy, results of IIF using monkey esophagus as substrate demonstrated high levels of anti-intercellular cement substance (anti-ICS) or antikeratinocyte cell surface antibody. Sera of all 13 patients on SSS bound to the epidermal side of the split. In an immunoblot, using bovine gingival lysate as substrate, sera of 6 patients bound to both a 230-kD (BP Ag1) and 180-kD protein (BP Ag2), while 7 sera bound to only a 230-kD protein. All 13 patients had high levels of antibodies to desmoglein 3 on ELISA. In a pilot experiment, the anti-ICS antibody in sera from 6 random patients was found to be predominantly of the IgG4 subclass. Use of IVIg resulted in an effective clinical response and the maintenance of a prolonged clinical remission. Conclusion: In patients with BP, who are nonresponsive to conventional therapy, the presence of two autoimmune diseases or a dual diagnosis should be considered.

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Section: Discussionmentioning

confidence: 99%

Diagnostic Features of Pemphigus vulgaris in Patients with Bullous Pemphigoid

Sami

Bhol²,

Beutner³

et al. 2002

Dermatology

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“…Perilesional skin was biopsied and specimens snap frozen in liquid nitrogen until use. Cryostat sections (5 m) were used, and direct immunofluorescence studies were performed according to standard techniques (26). Briefly, specimens were washed in PBS for 10 min, incubated for 30 min with FITC-conjugated F(abЈ) 2 fragment of rabbit anti-human IgG, specific for ␥-chains (1:25; Dako, Glostrup, Denmark), and washed in PBS for 15 min.…”

Section: Tissue Specimens Stainingmentioning

confidence: 99%

“…Sera were collected 24 h after PV IgG treatment, PA treatment (IgG depleted), or sham treatment. Indirect immunofluorescence studies were performed according to standard techniques (22,26). Cryostat sections (5 m) of monkey esophagus were employed as substrate, washed for 10 min in PBS, incubated for 30 min with different concentrations of sera (1:1-1:600), washed in PBS for 15 min, labeled with FITC-conjugated F(abЈ) 2 fragment of rabbit anti human IgG (Dako) for 30 min, and then washed again in PBS for 15 min.…”

Section: Tissue Specimens Stainingmentioning

confidence: 99%

Immune Modulation in Pemphigus Vulgaris: Role of CD28 and IL-10

Toto

Feliciani

Amerio

et al. 2000

The Journal of Immunology

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Pemphigus vulgaris (PV) is an autoimmune bullous skin disease characterized by Abs to the desmosomal cadherin desmoglein-3. Although the autoantibodies have been shown to be pathogenic, the role of the cellular immune system in the pathology of pemphigus-induced acantholysis is unclear. To further delineate the potential role of T cell-signaling pathways in the pathogenesis of PV, we performed passive transfer experiments with PV IgG in gene-targeted mutant mice. Our results demonstrated that CD28-deficient mice (lacking a costimulatory signal for T cell activation) are 5-fold more sensitive to the development of PV than wild-type mice. To evaluate whether the higher incidence of disease was due to an impairment in intercellular adhesion of keratinocytes, we performed an in vitro acantholysis, using CD28−/− mice keratinocytes. No alteration in in vitro adhesion was detected in CD28−/−-type keratinocytes. Because the CD28 molecule plays a pivotal role in the induction of Th2 cytokines, we examined the levels of a prototypic Th2 cytokine (IL-10) in CD28−/− mice. Lower levels of IL-10 mRNA were found in lesions from CD28−/− mice. To determine whether pemphigus susceptibility in CD28−/− was related to IL-10 deficiency, we performed passive transfer experiments in IL-10−/− mice that demonstrated increased blisters compared with controls. To confirm that IL-10 is involved in the pathogenesis, rIL-10 was given with PV IgG. IL-10 significantly suppressed the disease activity. These data suggest a potential role of IL-10 in PV.

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“…Complement (C3) is usually demonstrable and, on occasion, C3 is present without detectable IgG [11]. Pa tients with linear IgG staining, but without C3, have IgG4 as the predominant IgG sub class [12], The presence of circulating autoantibodies may be demonstrated by indirect IF techniques [10]: sequential studies show that the antibody titre does not correlate with disease activity [13], which is in con trast to pemphigus [14], Patients with BP often have two features not commonly associated with auto-im mune diseases: the serum IgE levels tend to be high [10,15] and there may be a peripher al blood eosinophilia [16], These findings have not been explained. Also, BP has been observed in some patients with malignant disease, though this concurrence is still con troversial.…”

Section: Introductionmentioning

confidence: 99%

Clinical, Histological and Immunological Studies in 50 Patients with Bullous Pemphigoid

Hadi

Barnetson²,

Gawkrodger³

et al. 1988

Dermatology

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Fifty patients with bullous pemphigoid were investigated over periods of up to 7 years. Sequential studies of circulating basement membrane zone (BMZ) antibody titres revealed two groups of patients: 44% had a high initial titre (> 1:160) and 56% had a low initial titre (< 1:160). Subjects with titres > 1:160 continued to have high titres, whereas in those with lower titres the BMZ antibodies usually became undetectable after 4 months. The BMZ antibody titres were of no prognostic value. High titres remained high even in patients who were in remission and off treatment, and in these subjects, there was no evidence of a change in the IgG subclass. The initial BMZ IgG antibody titres showed a highly significant correlation with total serum IgE concentrations. A significant correlation also existed between total serum IgE and IgG4 concentrations, although not between the serum IgE and the peripheral blood eosinophil count. No anti-BMZ antibody of IgE class was found, nor specific IgE against inhalants and foods identified, and the significance of the high total serum IgE remains unexplained. The majority of those followed for more than 2 years were able to stop their steroid therapy without further blistering: this confirms that pemphigoid tends to be a self-limiting disease. Nine patients had a recent history of malignant disease, and this is no more than would be expected for this age group of patients: however, 3 patients presented with pemphigoid and figurate erythema and all died of neoplastic disease, suggesting that such patients should be investigated thoroughly for neoplasia.

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The correlation of disease activity and antibody titers in pemphigus

Cited by 43 publications

References 2 publications

Diagnostic Features of Pemphigus vulgaris in Patients with Bullous Pemphigoid

Diagnostic Features of Pemphigus vulgaris in Patients with Bullous Pemphigoid

Immune Modulation in Pemphigus Vulgaris: Role of CD28 and IL-10

Clinical, Histological and Immunological Studies in 50 Patients with Bullous Pemphigoid

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