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Sjögren’s syndrome (SS) is a chronic autoimmune inflammatory disorder characterized by diminished lacrimal and salivary gland function with resultant dryness of the eyes and mouth [1,2].

Other organs are also affected. The clinical features of SS can be divided into the two broad categories of exocrine glandular features and extraglandular features [3].

SS occurs in a primary form not associated with other diseases and in a secondary form that complicates other rheumatic conditions. The most common disease associated with secondary SS is rheumatoid arthritis.

In primary or secondary SS, decreased exocrine gland function leads to the “sicca complex,” a combination of dry eyes (keratoconjunctivitis sicca [KCS]) and dry mouth (xerostomia) [1,2]. In addition, a wide variety of other disease manifestations can occur in SS. The clinical manifestations of SS are divided into the exocrine gland features and the extraglandular disease features [3].

A retrospective study of 80 patients with primary SS followed for a median of 7.5 years reported the following frequencies of clinical manifestations [3]:

●KCS and/or xerostomia occurred in all patients and were the only disease manifestation in 31 percent

●Extraglandular involvement occurred in 25 percent

●Non-Hodgkin lymphoma developed in 2.5 percent

A series of different classification criteria have been used to define and characterize SS. Most information cited in this topic review regarding SS has relied upon the 2002 American European Consensus Group (AECG) classification criteria [4,5]. However, in 2012, a different set of criteria was proposed by the American College of Rheumatology (ACR) that differed from the AECG criteria in several respects, including the exclusion of subjective ocular or oral dryness from the ACR criteria set and the lack of a distinction between primary and secondary SS [6]. Thus, differences in estimates of the frequency and severity of various clinical manifestations may be observed that depend upon the criteria used to identify the patients included in the analysis [6,7]. (See "Diagnosis and classification of Sjögren's syndrome", section on 'Classification criteria'.)

The clinical manifestations of exocrine gland disease in SS will be reviewed here. The extraglandular manifestations, the classification and diagnosis, and the treatment of both sicca and extraglandular manifestations are discussed separately. (See "Clinical manifestations of Sjögren's syndrome: Extraglandular disease" and "Diagnosis and classification of Sjögren's syndrome" and "Treatment of dry eye in Sjögren's syndrome" and "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome" and "Treatment of systemic and extraglandular manifestations of Sjögren’s syndrome".)




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Complications of Injecting Drug Use

  • Local problems—Abscess (Figures 240-2 
    Image not available.

    A 32-year-old woman with type 1 diabetes developed large abscesses all over her body secondary to injection of cocaine and heroin. Her back shows the large scars remaining after the healing of these abscesses. (Courtesy of ­Richard P. Usatine, MD.)

    and 240-3; Abscess), cellulitis, septic thrombophlebitis, local induration, necrotizing fasciitis, gas gangrene, pyomyositis, mycotic aneurysm, compartmental syndromes, and foreign bodies (e.g., broken needle parts) in local areas.2
    • IDUs are at higher risk of getting methicillin-resistant Staphylococcus aureus(MRSA) skin infections that the patient may think are spider bites (Figure 240-4).
    • Some IDUs give up trying to inject into their veins and put the cocaine directly into the skin. This causes local skin necrosis that produces round atrophic scars (Figure 240-5).
  • IDUs are at risk for contracting systemic infections, including HIV and hepatitis B or hepatitis C.
    • Injecting drug users are at risk of endocarditis, osteomyelitis (Figures 240-6and 240-7), and an abscess of the epidural region. These infections can lead to long hospitalizations for intravenous antibiotics. The endocarditis that occurs in IDUs involves the right-sided heart valves (see Chapter 50, Bacterial Endocarditis).2 They are also at risk of septic emboli to the lungs, group A β-hemolytic streptococcal septicemia, septic arthritis, and candidal and other fungal infections.


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A 45-year-old woman with long-standing rheumatoid arthritis complains of dry eyes and dry mouth. Bilateral enlargement of the parotids is noted on physical examination. The syndrome described here is best described as


    A. autoimmune.


    B. infectious.


    C. metabolic.


    D. metastatic.


    E. primary neoplastic.

 The answer is A. Sjögren syndrome is an autoimmune disorder characterized by keratoconjunctivitis sicca and xerostomia, due to lymphocytic infiltration and parenchymal destruction of the parotid and lacrimal glands in association with a connective tissue disorder, such as rheumatoid arthritis. Malignant lymphoma is a frequent complication. 

A 75-year-old triathlete complains of gradually worsening vision over the past year. It seems to be involving near and far vision. The patient has never required corrective lenses and has no significant medical history other than diet-controlled hypertension. He takes no regular medications. Physical examination is normal except for bilateral visual acuity of 20/100. There are no focal visual field defects and no redness of the eyes or eyelids. Which of the following is the most likely diagnosis?

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The correct answer is A. You answered A.

Age-related macular degeneration is a major cause of painless, gradual bilateral central visual loss. It occurs as nonexudative (dry) or exudative (wet) forms. Recent genetic data have shown an association with the alternative complement pathway gene for complement factor H. The mechanism link for that association is unknown. The nonexudative form is associated with retinal drusen that leads to retinal atrophy. Treatment with vitamin C, vitamin E, beta-carotene, and zinc may retard the visual loss. Exudative macular degeneration, which is less common, is caused by neovascular proliferation and leakage of choroidal blood vessels. Acute visual loss may occur because of bleeding. Exudative macular degeneration may be treated with intraocular injection of a vascular endothelial growth factor antagonist (bevacizumab or ranibizumab). Blepharitis is inflammation of the eyelids usually related to acne rosacea, seborrheic dermatitis, or staphylococcal infection. Diabetic retinopathy, now a leading cause of blindness in the United States, causes gradual bilateral visual loss in patients with long-standing diabetes. Retinal detachment is usually unilateral and causes visual loss and an afferent pupillary defect.


Mr. Jenson is a 40-year-old man with a congenital bicuspid aortic valve who you have been seeing for more than a decade. You obtain an echocardiogram every other year to follow the progression of his disease knowing that bicuspid valves often develop stenosis or regurgitation requiring replacement in middle age. Given his specific congenital abnormality, what other anatomic structure is important to follow on his biannual echocardiograms?

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The correct answer is A. You answered A.

The answer is A. (Chap. 282) Bicuspid aortic valve is among the most common of congenital heart cardiac abnormalities. Valvular function is often normal in early life and thus may escape detection. Due to abnormal flow dynamics through the bicuspid aortic valve, the valve leaflets can become rigid and fibrosed, leading to either stenosis or regurgitation. However, pathology in patients with bicuspid aortic valve is not limited to the valve alone. The ascending aorta is often dilated, misnamed “poststenotic” dilatation; this is due to histologic abnormalities of the aortic media and may result in aortic dissection. It is important to screen specifically for aortopathy because dissection is a common cause of sudden death in these patients.



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Update May 12, 2021