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Collectively, the collagen vascular diseases, including polymyositis, dermatomyositis, scleroderma, and systemic lupus erythematosus, may all affect the salivary glands, although Sjogren disease and sarcoidosis are most commonly responsible (Kessler and Bhatt 2018). Sjogren disease‐related sialadenitis is predominantly seen in females and most commonly in postmenopausal women (50–70 years of age). A juvenile subtype is seen in men younger than 20 years of age that typically resolves at puberty. Sjogren disease‐related sialadenitis is classified into two types. Sjogren type 1 disease (Mikulicz disease or sicca syndrome without a connective disorder) refers to autoimmune sialadenitis without a systemic collagen vascular disorder. These patients demonstrate xerostomia and are incorporated into the IgG4 spectrum of disease. Sjogren type 2 disease refers to autoimmune inflammation of the salivary glands with a systemic autoimmune diagnosis (rheumatoid arthritis > systemic lupus erythematosus > scleroderma).

Systemic lupus erythematosus is most frequently seen in fourth and fifth‐decade women. Any of the salivary glands may become involved, and a slowly enlarging gland is the presentation. The diagnosis is made by identification of the underlying systemic disorder, and salivary chemistry levels will reveal sodium and chloride ion levels that are elevated to two three times normal levels (Miloro and Goldberg 2002).

Sarcoid‐related sialadenitis is seen in 10–30% of patients with sarcoidosis and patients typically present with painless bilateral parotid swelling. The treatment of autoimmune sialadenitis involves treatment of the responsible systemic disease. The reader is referred to Chapter 6 in which autoimmune sialadenitis is more granularly reviewed and illustrated.

Immunoglobulin G4‐related disease (IgG4‐RD) is a condition characterized by an immune‐mediated fibroinflammatory pathologic process with a tendency to form tumefactive lesions in organs, the most common of which are the pancreas and salivary glands (Lang et al. 2016)(see Chapter 6). Clinical and serologic findings include swollen organs and elevated serum IgG4. Common histologic findings in tissues include fibrosis with a storiform pattern, a diffuse lymphoplasmacytic infiltrate, obliterative phlebitis, abundance of IgG4 plasma cells, and mild to moderate tissue eosinophilia (Puxeddu et al. 2018). The increase of IgG4 seems to be a reactive phenomenon rather than the primary etiology of the disease. Involvement of the salivary glands is seen in 27–53% of patients with IgG4‐related disease (Puxeddu et al. 2018). Unlike classic autoimmune diseases such as systemic lupus erythematosus and Sjögren syndrome that affect mainly females, IgG4‐related disease occurs in a subacute form in most patients without the rapid onset of general symptoms such as fever. Mikulicz disease affecting the lacrimal and parotid glands and Kuttner tumor affecting the submandibular glands are two examples of IgG4‐related disease of the salivary gland.