Systemic lupus erythematosus and common variable panhypogammaglobulinemia: A patient with absence of circulating B cells

Abstract

An association between systemic lupus erythematosus (SLE) and common variable panhypogammaglobulinemia (CVP) has recently been reported (1,2). It is of particular interest that the 2 disorders represent the extremes of B cell dysfunction-SLE characterized by B cell hyperactivity, and CVP by B cell hypoactivity. The results of investigations of the 3 previous cases of SLE and CVP revealed the presence of circulating B cells exhibiting decreased responses to mitogens in all instances (1 ,2) and to vaccination in I case studied for this (2), as well as excessive T cell suppression and subnormal T cell help in the 2 cases studied in vitro (I). We report the fourth case: a patient with an association of SLE and CVP in whom an absence of circulating B cells was the most significant lymphocyte abnormality seen.Case report. A 61-year-old woman had polyarthralgias and dry eyes since the age of 33. She had had 7 pregnancies-6 ending in spontaneous abortions and 1 in a live birth.

Association of common variable immunodeficiency and rare and complex connective tissue and musculoskeletal diseases. A systematic literature review

“…Reviewing the literature, we identified 15 cases with concurrent SLE and CVID (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) In one case with PM, the diagnosis of PM was made 7 years before the diagnosis of CVID, in the other case (22), the patient had suffered from recurrent infections in the ten years preceding the diagnosis of PM. In the patient with ASS, the IIM was diagnosed 12 years before CVID (22).…”

Immunodeficiency in the lupus clinic

“…Various case reports describe the association of CVID with SLE in various case reports, [19][20][21] and it is important to appreciate that CVID does differ from drug-related antibody deficiency. 22 In CVID, which is a primary antibody deficiency, IgG and IgA are usually low and also IgM in 50% of cases.…”

Alterações oftalmológicas em pacientes com imunodeficiência comum variável

“…Tal reforça a existência de mais de um mecanismo envolvido nestes dois processos30 . Estudos in vitro apontam uma considerável redução na resposta aos mitógenos pelas células B de pacientes com ambas as entidades clínicas, bem como uma expressiva supressão de células T34 . Manifestações articulares similares à artrite reumatoide ou artrite idiopática juvenil (AIJ) ocorrem em 1-10% dos pacientes com ICV, geralmente acometendo simetricamente as articulações, em especial joelhos, tornozelos e mãos, podendo levar à destruição articular.…”