Neutrophilic Dermatoses in a Seronegative Rheumatoid Arthritis Patient: a case report

Rheumatoid arthritis (RA) is a chronic inflammatory disease, characterized by symmetric and destructive polyarthritis with a broad spectrum clinical manifestation of various organs. Rheumatoid neutrophilic dermatitis (RND) is an unusual distinctive manifestation of RA and typically develops in severe RA. 51-year-old female presented with a skin rash involving her extremities.

Clinicopathological Manifestations and Immune Phenotypes in Adult-Onset Immunodeficiency with Anti-Interferon-γ Autoantibodies

Purpose Anti-interferon (IFN)-γ autoantibodies (anti-IFN-γ Abs) is an emerging adult-onset immunodeficiency syndrome. Immune dysfunction in this distinct disorder remains to be clarified. Methods We prospectively collected blood samples of 20 patients with anti-IFN-γ Abs and 40 healthy normal subjects. The percentages of lymphocyte subpopulations, most relevant to T, B, and NK cells, and the percentages of stimulated lymphocytes with cytokine production were assessed using eight-color flow cytometry. The results were adjusted to age and absolute lymphocyte counts. Results Most (85%) patients presented nontuberculous mycobacterial infection. Skin lesions were predominantly manifested by neutrophilic dermatoses. The involved lymph nodes had granulomatous inflammation, except 22.2% showing atypical lymphoid hyperplasia without granuloma formation. The percentages of CD4 + T cells and non-activated subpopulations (recent thymic emigrants and naïve subtypes) decreased significantly with increased expression of activation markers and polarization to differentiated cells. The percentage of NK cells increased, but that of two major NK subpopulations, CD56bright and CD56 + CD16 + subsets decreased. Furthermore, the expression of NKp30 and NKp46 decreased with increased CD57 expression. Intracellular cytokine production of the lymphocyte subpopulations were significantly low in the patients compared with the control group. Conclusion We conclude that the immune system in patients with anti–IFN-γ Abs could be exhausted, contributing to the distinct clinicopathologic features.

Clinicopathological Manifestations and Immune Phenotypes in Adult-onset Immunodeficiency With Anti-interferon-γ Autoantibodies

Purpose Adult-onset immunodeficiency with anti-interferon (IFN)-γ autoantibodies (anti-IFN-γ Abs) is an immunodeficiency syndrome. Immune dysfunction in this distinct disorder remains to be clarified. Methods We prospectively collected blood samples of 20 patients with anti-IFN-γ Abs and 65 healthy normal subjects. Percentages of lymphocyte subpopulations, most relevant to T-, B-, and NK-cells, and percentages of stimulated lymphocytes with cytokine production were assessed using eight-color flow cytometry. The results were adjusted to age and absolute lymphocyte counts.Results Most (85%) patients presented non-tuberculous mycobacterial infection. Skin lesions were predominantly manifested by neutrophilic dermatoses. Involved lymph nodes had granulomatous inflammation, except 22.2% showing atypical lymphoid hyperplasia without granuloma formation. In the multiple linear regression model, CD4+ T cells and non-activated subpopulations (recent thymic emigrants and naïve subtypes) were significantly decreased with increased expression of activation markers and polarization to Th1, Th17, and Treg. The percentage of NK cells was increased, but two major NK subpopulations, CD56bright and CD56+CD16+ subsets were decreased. Furthermore, NK cells diminished expression of NKp30 and NKp46 with increased CD57 expression. The cytokine production was significantly lower, namely TNF-α in CD4+ T cells (P = 0.009), CD8+ T cells (P < 0.001), and NK cells (P = 0.002); IFN-γ in CD8+ T cells (P = 0.002) and NK cells (P = 0.001); and IL-2 in CD4+ (P < 0.001), and CD8+ (P = 0.005) T cells. Conclusion We conclude that the immune system in patients with anti–IFN-γ Abs could be exhausted which may contribute to the distinct clinicopathologic features.