What are IgG4 diseases?
Abstract. IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis.
Is IgG4 disease curable?
The short-term clinical, morphological, and functional outcomes of most IgG4-RD patients treated with steroid therapy are good, but the long-term outcomes are less clear due to several unknown factors such as relapse, developed fibrosis, and associated malignancy.
Is IgG4 life threatening?
IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening. Mod Rheumatol. 2019 Mar;29(2):240-250.
What causes IgG4 disease?
The cause of IgG4-related disease is unknown. It may be an autoimmune disorder: antinuclear antibodies and autoantibodies to pancreatic antigens have been found in autoimmune pancreatitis.
What are the symptoms of IgG4?
Symptoms of IgG4-RD include diffuse pain of joints, tendons, with associated fatigue, especially when several systems/organs are already affected. There is no fever, night sweats, or – generally – weight loss. In most cases, tissue biopsy is the gold diagnostic standard.
What is the treatment for IgG4?
Treatment for IgG4-RSD traditionally has involved prednisone, a steroid medication. Prednisone has proven very effective in treating this condition but also leads to side effects such as weight gain, osteoporosis, high blood pressure and diabetes.
How do you cure IgG4?
How is IgG4 diagnosed?
Histopathology Histopathologic assessment of the affected organs remains the gold standard for the diagnosis of IgG4-RD. In most cases the biopsy is the best way to exclude the mimickers of IgG4-RD including malignancy or infection.
What is normal range for IgG4?
The normal concentration of serum IgG4 in children varies with age. The reference interval of IgG4 in adults have been recommended as 0.052–1.250 g/L with the upper limit being 0.86–1.35 g/L [2, 11]. An elevated IgG4 level means that the concentration is higher than 1.35 g/L [12-14].
What is a IgG4 blood test?
Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibroinflammatory disorder that can result in lesions in almost any organ, can involve multiple organ systems, and can lead to organ failure.
What is IgG4-related disease?
“IgG4- related disease: an orphan disease with many faces”. Orphanet Journal of Rare Diseases. 9: 110. doi: 10.1186/s13023-014-0110-z. PMC 4223520.
Is idiopathic cervical fibrosis an IgG4-related Sclerosing Disease?
“Idiopathic cervical fibrosis–a new member of IgG4-related sclerosing diseases: report of 4 cases, 1 complicated by composite lymphoma”. The American Journal of Surgical Pathology. 34 (11): 1678–1685. doi: 10.1097/PAS.0b013e3181f12c85. PMID 20871392. S2CID 364195.
What does IgG4-ROD stand for?
(T2-weighted MRI) IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease, which is characterised by infiltration of lymphocytes and plasma cells and subsequent fibrosis in involved structures.
What causes elevated IgG4 levels in multiple sclerosis (MS)?
In approximately 51–70% of people with this disease, serum IgG4 concentrations are elevated during an acute phase. It is a relapsing-remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved.
