IgG4-related Disease Clinical Trial
Official title:
Plasmablast Detection From IgG4-Related Disease Patients
IgG4-related disease (IgG4-RD) is an immune-mediated, fibro-inflammatory disease that leads to tissue damage, organ dysfunction and, if untreated, to organ failure. The disease can affect almost any anatomic location, but the sites involved most commonly are the pancreas, salivary glands, orbital adnexa, lymph nodes, and retroperitoneum. IgG4-RD, typically diagnosed among individuals who are middle-aged, is characterized by a male predominance except with regard to organs of the head and neck (e.g., the salivary glands and orbits), where the gender distribution is approximately equal. The epidemiology of IgG4-RD remains poorly understood because of its recognition only recently as a multi-organ disease. However, IgG4-RD accounts for many conditions once regarded as disparate, single-organ disorders The purpose of our study is to evaluate the method of plasmablast measurement in peripheral blood of IgG4-RD patients, for diagnosis and follow-up on disease progression and response to treatment. This document will outline the collection, processing and testing procedures for measuring plasmablasts from IgG4-RD patients
IgG4-related disease (IgG4-RD) is an immune-mediated, fibro-inflammatory disease that leads
to tissue damage, organ dysfunction and, if untreated, to organ failure. The disease can
affect almost any anatomic location, but the sites involved most commonly are the pancreas,
salivary glands, orbital adnexa, lymph nodes, and retroperitoneum. IgG4-RD, typically
diagnosed among individuals who are middle-aged, is characterized by a male predominance
except with regard to organs of the head and neck (e.g., the salivary glands and orbits),
where the gender distribution is approximately equal. The epidemiology of IgG4-RD remains
poorly understood because of its recognition only recently as a multi-organ disease. However,
IgG4-RD accounts for many conditions once regarded as disparate, single-organ disorders.
The current gold standard for the diagnosis of IgG4-RD is the identification of
characteristic histology and immunohistochemistry features through biopsy. These pathology
features are consistent across the full range of organs affected by IgG4-RD. However,
histopathologic variation can occur according to the stage of the lesion; that is,
longstanding disease may be predominately fibrotic and a cellular. Confirming the diagnosis
of IgG4-RD in such cases can be difficult. Moreover, IgG4-RD organ pathology and IgG4-RD
mimickers, such as granulomatosis with polyangiitis (formerly Wegener's), sarcoidosis,
histiocytosis, and malignancies (e.g., lymphoma and adenocarcinoma of the pancreas), may
share similar features including an IgG4-positive plasma cell infiltrate. Reliance upon serum
IgG4 concentrations to diagnose IgG4-RD is similarly problematic because both the specificity
and positive predictive value of serum IgG4 concentrations are poor.
Plasmablasts, derived from the B-cell lineage and characterized as CD19lowCD20−CD38+CD27+,
comprise a stage intermediate between activated B-cells and plasma cells. Plasmablasts are
generally rare in the peripheral blood of healthy individuals, but expansions are observed
briefly during responses to infection or vaccination. In contrast, in the setting of
autoimmunity and persistent self-antigen(s), plasmablasts can circulate for prolonged
periods.
Circulating plasmablasts have been described previously in inflammatory bowel disease,
rheumatoid arthritis, systemic lupus erythematosus, and multiple myeloma. Recently, several
studies identified plasmablsts in IgG4-RD both as a diagnostic tool and as an indicator of
response to treatment
2. Aim
The purpose of our study is to evaluate the method of plasmablast measurement in peripheral
blood of IgG4-RD patients, for diagnosis and follow-up on disease progression and response to
treatment. This document will outline the collection, processing and testing procedures for
measuring plasmablasts from IgG4-RD patients.
3. Plasmablast source
Plasmablasts will be isolated from peripheral blood derived from patients and normal donors
who consent to participate in the study. Blood samples will be drawn by a physician or
accredited nurse, transferred to the research lab in order to separate PMBCs, which will be
stained for CD19lowCD20−CD38+CD27+ markers and measured by flow cytometry (FACS) located at
the hematology lab
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