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IgG4-related disease

Table Of Contents

    • IgG4-related disease is a fibroinflammatory disease that tends to form mass-like lesions.

    • It can affect almost any organ system. However, it is not expected to involve the brain parenchyma, bone marrow, joints and intestinal mucosa.

    • The disease is most common in middle-aged and elderly men.

    Pathology

    • Basic pathological features in all organs affected by this disease;

    o Lymphoplasmocytic infiltration with a high percentage of IgG4 (+) plasma cells

    o Characteristic fibrosis called "Storiform" (basket weave-like)

    o Tendency to target blood vessels (obliterative phlebitis, especially in veins)

    o Inflammation that tends to surround the ducts of the involved gland (lacrimal, salivary and pancreatic)

    o Mild to moderate tissue eosinophilia


    Clinical Findings

    • Two common characteristics of IgG4 systemic disease;

    o Allergic disease: Most patients have atopy, eczema, asthma, sinusitis and eosinophilia.

    o Massive lesions mimicking malignancy: Patients may be mistakenly diagnosed with malignancy

    IgG4-related disease involvement

    Orbital and periorbital tissues:

    Eyelid and periorbital edema, orbital pseudotumor, dacryoadenitis,

     dacryocystitis

    Ear, nose and sinuses:.

    Allergic phenomena (bronchial asthma, allergic rhinitis, sinusitis), nasal obstruction, rhinorrhea, anosmia and sometimes bone destruction

    salivary glands:

    Submandibular/parotid growth

    Meninges :

    Headache, radiculopathy, cranial nerve palsy, dural thickening

    Hypothalamus and pituitary gland:

    Pituitary insufficiency, central diabetes insipidus, mass in the pituitary stalk

    Thyroid gland: Riedel's thyroiditis

    Lymph nodes: Diffuse/local lymphadenopathy

    Lungs: Cough, hemoptysis, dyspnea, pleural effusion, inflammatory

    pseudotumor, localized/diffuse interstitial pneumonia, central airway

    disease, pleuritis

    Aorta: Aortic dissection, periaortitis, inflammatory abdominal aorta

    aneurysm

    Retroperitoneum: Low back pain, pain in the lower abdomen, hydronephrosis

    Kidneys: Tubulointerstitial nephritis, membranous nephropathy, hypocomplementemia

    Pancreas: Type I autoimmune pancreatitis, mass appearance in the pancreas, obstructive jaundice

    Bile ducts and liver: Obstructive jaundice, abdominal pain, steatorrhea, weight loss, new-onset diabetes mellitus, findings mimicking primary sclerosing cholangitis

    Serological findings

    • The majority of patients have high serum IgG4 levels.

    • Serum IgG4 level does not correlate well with disease activity and treatment requirement.

    Treatment

    • Glucocorticoids are the first-line treatment option.

    • Rituximab is the second-line treatment option in patients with frequent relapses or steroid-resistant patients.

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