IgG4-related diseases and Miguritz disease

  Miguritz’s disease is an autoimmune disease involving salivary glands and lacrimal glands, characterized by lymphocytic infiltration and replacement of the alveoli, typically with chronic inflammation of the lacrimal and parotid glands with bilateral enlargement, mostly over 30 years of age, but also at any age, of unknown etiology, mostly with a slow onset, but also with acute onset. The initial onset is unilateral and the secondary onset is bilateral. It is not associated with pain or systemic reactions. The lacrimal gland is swollen, symmetrical, soft, mobile, elastic, and painless, sometimes accompanied by hepatosplenomegaly and generalized lymph node enlargement.  The parotid gland often starts to swell symmetrically at the same time or after a few weeks or years, sometimes invading the paracrine glands and glands, reducing salivary secretion and causing dryness of the oral, nasal and throat mucosa.  Clinical manifestations: dry mouth, dry eyes, salivary gland enlargement, arthrosis, etc. Salivary gland imaging, isotope scan, and biopsy pathology have diagnostic value. IgG4 is mostly elevated in laboratory tests, which is now considered a clinical manifestation of IgG4-associated disease.  IgG4-associated disease, also known as IgG4-associated multi-organ lymphoid hyperplasia syndrome, is an autoimmune disease characterized by elevated serum IgG4 levels (>135 mg/dl), IgG4-positive plasma cell infiltration and tissue fibrosis or sclerosis, and may involve multiple organs simultaneously. The most common ones are salivary glands, lacrimal gland involvement (Miguritz disease), pancreas involvement (autoimmune pancreatitis), mandibular glands, liver, lungs, kidneys and lymph nodes. Systemic manifestations may include fever, malaise, weight loss, enlargement of salivary glands and lacrimal glands, clinical manifestations of dry mouth and eyes, but the symptoms are often milder than those of dry syndrome, autoimmune pancreatitis, retroperitoneal fibrosis, sclerosing cholangitis, sclerosing meningitis, interstitial pneumonia, interstitial nephritis, inflammatory aneurysms, occlusive ductitis, etc. The involved lesions are often tumor-like or lymph node enlargement. The affected lesions are often tumor-like or lymph node enlargement, and should be differentiated from tumors and lymphomas.