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IgG4-related prostatitis is
prostate The prostate is both an Male accessory gland, accessory gland of the male reproductive system and a muscle-driven mechanical switch between urination and ejaculation. It is found only in some mammals. It differs between species anatomically, ...
involvement in men with IgG4-related disease (IgG4-RD), which is an emerging fibroinflammatory disease entity which is characterised (i) by a tendency to mass forming lesions in multiple sites of the body and (ii) by usually a prompt response to
steroid A steroid is a biologically active organic compound with four rings arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes that alter membrane fluidity; and a ...
therapy. Men with IgG4-related prostatitis may also present with manifestations of IgG4-RD at other sites anywhere in the body; involvement of different areas of the body can occur either at the same time (synchronously) or at different periods of time (metachronously).


Presentation

Several case studies on IgG4-related prostatitis have been reported. Patients have been noted to commonly present with lower urinary tract symptoms such as
dysuria Dysuria refers to painful or uncomfortable urination. It is one of a constellation of ''irritative'' bladder symptoms (also sometimes referred to as lower urinary tract symptoms), which includes nocturia and urinary frequency. Diagnosis The clin ...
, pollakisuria, urinary urgency, and a feeling of incomplete emptying. The clinical presentation is similar to that in
benign prostatic hyperplasia Benign prostatic hyperplasia (BPH), also called prostate enlargement, is a noncancerous increase in size of the prostate gland. Symptoms may include frequent urination, trouble starting to urinate, weak stream, inability to urinate, or loss o ...
or chronic
prostatitis Prostatitis is an umbrella term for a variety of medical conditions that incorporate bacterial and non-bacterial origin illnesses in the pelvic region. In contrast with the plain meaning of the word (which means "inflammation of the prostate"), the ...
, although pain, as occurs in CP/CPPS, does not usually appear to be significant.


Histology

The diagnosis of IgG4-related prostatitis could be made from histological examination if
prostate biopsy Prostate biopsy is a procedure in which small hollow needle-core samples are removed from a man's prostate gland to be examined for the presence of prostate cancer. It is typically performed when the result from a PSA blood test is high. It may ...
or surgery has been performed. The hallmark histopathological features of established IgG4-related disease are
storiform A woven or storiform pattern is a histopathologic architectural pattern. The name "storiform" originates from Latin ''storea'' (''woven''), as storiform tissue tends to resemble woven fabric on microscopy. Storiform fibrosis is a histologic sign ...
fibrosis Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of perma ...
, a dense lymphoplasmacytic (
lymphocyte A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic ad ...
s and plasma cells) infiltrate rich in IgG4-positive 
plasma cell Plasma cells, also called plasma B cells or effector B cells, are white blood cells that originate in the lymphoid organs as B lymphocytes and secrete large quantities of proteins called antibodies in response to being presented specific substan ...
s, and obliterative 
phlebitis Phlebitis (or Venitis) is inflammation of a vein, usually in the legs. It most commonly occurs in superficial veins. Phlebitis often occurs in conjunction with thrombosis and is then called thrombophlebitis or superficial thrombophlebitis. Unlike ...
. However, identification depends on whether or not
urologist Urology (from Greek οὖρον ''ouron'' "urine" and ''-logia'' "study of"), also known as genitourinary surgery, is the branch of medicine that focuses on surgical and medical diseases of the urinary-tract system and the reproductive organ ...
s and
pathologist Pathology is the study of the causal, causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when us ...
s are aware of IgG4-related prostatitis/disease, as special immunostaining is required to identify the characteristic IgG4-positive plasma cells infiltration in prostatic tissue.


Diagnosis

Men with IgG4-related prostatitis may have similar findings to those that are frequently, but not always, seen in other organ manifestations of IgG4-related disease, such as elevated blood levels of
IgG4 Immunoglobulin G (Ig G) is a type of antibody. Representing approximately 75% of serum antibodies in humans, IgG is the most common type of antibody found in blood circulation. IgG molecules are created and released by plasma B cells. Each IgG a ...
,
IgE Immunoglobulin E (IgE) is a type of antibody (or immunoglobulin (Ig) " isotype") that has been found only in mammals. IgE is synthesised by plasma cells. Monomers of IgE consist of two heavy chains (ε chain) and two light chains, with the ε c ...
and
eosinophil Eosinophils, sometimes called eosinophiles or, less commonly, acidophils, are a variety of white blood cells (WBCs) and one of the immune system components responsible for combating multicellular parasites and certain infections in vertebrates. A ...
s. FDG-
PET A pet, or companion animal, is an animal kept primarily for a person's company or entertainment rather than as a working animal, livestock, or a laboratory animal. Popular pets are often considered to have attractive appearances, intelligence, ...
scans have been reported to be useful as a diagnostic modality for detecting IgG4-related prostatitis in men with IgG4-RD.


Treatment

IgG4-related disease IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and ...
responds well, and often dramatically, to
glucocorticoid Glucocorticoids (or, less commonly, glucocorticosteroids) are a class of corticosteroids, which are a class of steroid hormones. Glucocorticoids are corticosteroids that bind to the glucocorticoid receptor that is present in almost every vertebr ...
therapy, provided that advanced
fibrotic Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of perman ...
lesions have not resulted in irreversible damage, and this has included resolution of radiologic findings. Men given glucocorticoids to treat IgG4-related disease at other anatomical sites sometimes report relief of their lower urinary tract symptoms, suggesting that IgG4-related prostatitis may be underdiagnosed. Cases are however likely to get misdiagnosed as
benign prostatic hyperplasia Benign prostatic hyperplasia (BPH), also called prostate enlargement, is a noncancerous increase in size of the prostate gland. Symptoms may include frequent urination, trouble starting to urinate, weak stream, inability to urinate, or loss o ...
and to get treated alternatively with medications such as
alpha blocker Alpha (uppercase , lowercase ; grc, ἄλφα, ''álpha'', or ell, άλφα, álfa) is the first letter of the Greek alphabet. In the system of Greek numerals, it has a value of one. Alpha is derived from the Phoenician letter aleph , which ...
s. The
efficacy Efficacy is the ability to perform a task to a satisfactory or expected degree. The word comes from the same roots as ''effectiveness'', and it has often been used synonymously, although in pharmacology a pragmatic clinical trial#Efficacy versu ...
of
alpha blocker Alpha (uppercase , lowercase ; grc, ἄλφα, ''álpha'', or ell, άλφα, álfa) is the first letter of the Greek alphabet. In the system of Greek numerals, it has a value of one. Alpha is derived from the Phoenician letter aleph , which ...
s in IgG4-related prostatitis remains unclear.


See also

*
IgG4-related disease IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and ...


References


External links


Prostatitis - Causes, Symptoms, Diagnosis & Treatment
{{Medical resources , ICD10 = {{ICD10, N, 41, 8 IgG4-related disease Inflammatory prostate disorders Steroid-responsive inflammatory conditions