Sjogren

Which of the following is a key histological finding in a patient with Sjogren Sx?

• Immuniflorescent staining depicting linear IgG pattern along the basement membrane under the basal skin layer

• Synovial fluid revealing needle-shaped crystals with negative biorefringence under polarized light

• Dense lymphocytic infiltration of salivary gland tissue

• Skin biopsy depicting peripheral palisading of basal cell nodules

Answer explanation

[Choice A]: Bullous pemphigoid is an autoimmune skin condition that is histologically characterized by detachment of the basal cell layer of skin from its basement membrane underneath.

[Choice B]: Gout is characterized by needle-shaped crystals that are negatively birefringent under polarized light.

[Choice D]: Basal cell carcinoma is histologically characterized by peripheral palisading nodules of basal skin cells.

Which of the following is assocaited with Sjogren sx?

• Reye Sx

• Primary biliary cirrhosis

• Hepatic steatosis

• Primary sclerosing cholangitis

Answer explanation

Sjögren syndrome is associated with primary biliary cirrhosis (PBC). This is an autoimmune disease that attacks the lobular bile ducts of the liver. When these bile ducts are destroyed, bile can accumulate and damage the liver. Notably, both diseases tend to occur in middle-aged females.

[Choice A]: Reye Syndrome is a rare, fatal hepatic encephalopathy that affects children who are given aspirin (acetylsalicylic acid).

[Choice C]: Hepatic steatosis, or “fatty liver disease,” is the early, reversible stage of alcoholic liver disease characterized by fat accumulation in the liver.

[Choice D]: Primary sclerosing cholangitis is a condition characterized by scarring of the bile ducts from chronic inflammation, leading to the narrowing (or obstruction) of these ducts, which can cause bile to accumulate in the liver.

Key Takeaway:

• Sjögren syndrome is associated with primary biliary cirrhosis.

What is the purpose of the Schirmer test?

• To assess if the salivary glands produce adequate saliva to keep the mouth moist

• To determine whether the eyes create tears adequately to prevent them from drying

• To measure the sharpness and clarity of the eyes when looking at distant or near objects

• To quantify the concentration of chloride ions in a pilocarpine-induced sweat test

Answer explanation

The Schirmer test assesses whether the eyes produce tears sufficiently to keep them moist and keep them from drying.

[Choice A]: This describes sialometry, a noninvasive diagnostic test for Sjögren syndrome that evaluates how much saliva is being produced in the mouth.

[Choice C]: The sharpness & clarity of the eyes in near and distant vision are measured with the visual acuity test. In Sjögren syndrome, the eyes become dry; however, their acuity should remain intact, especially if dryness is addressed early.

[Choice D]: This depicts a chloride sweat test, which aids in diagnosing cystic fibrosis.

Which of the following is/are used to distinguish age-related sicca syndrome from Sjogren Sx?

• Results of serological antibody tests

• Age of onset

• Histological findings from biopsy

• All of the above

Answer explanation

Age-related sicca syndrome is often confused with Sjögren syndrome; thus, differentiating the two clinically is critical. Antibody test results, age of onset, and biopsy findings are all factors that can be used to distinguish age-related sicca syndrome from Sjögren syndrome.

[Choice A]: Patients with age-related sicca syndrome will typically have normal antibody test results. This is in contrast to patients with Sjögren syndrome as they will test positive for specific autoantibodies such as anti-Ro (SSA) and/or anti-La (SSB).

[Choice B]: Age of onset is a distinguishing factor. Age-related sicca syndrome will typically affect older patients (70 to 80-year-olds) whereas Sjögren syndrome will affect patients in their 40s.

[Choice C]: A patient with age-related sicca syndrome will most likely have normal biopsy results of salivary gland tissue. Patients with Sjögren syndrome will have characteristic lymphocytic infiltration on salivary gland tissue biopsy.

Key Takeaway:

• Age-related sicca syndrome differs from Sjögren syndrome in that it has normal antibody test results, affects older individuals, and has a normal salivary gland tissue biopsy.

Which pair of autoantiboides most support the diagnosis of Sjogren Sx?

• anti-Sm, anti-sdDNA

• anti-Ro, anti-Sm

• anti-CCP, anti-dsDNA

• anti-La, anti-Ro

Answer explanation

Detection of the autoantibodies anti-La (SSB) and anti-Ro (SSA) supports a diagnosis of Sjögren syndrome. 

[Choice A]: Anti-Sm and anti-dsDNA autoantibodies are specific for systemic lupus erythematosus (SLE).

[Choice C]: Anti-CCP is used in the diagnosis of rheumatoid arthritis (RA).

Which of the following presetnations are consistent with Sjogren Sx?

• Xerosis, xanthoma, keratoconjunctivis, joing pain

• Xerosis, xerostomia, keratoconjunctivitis sicca, joint pain

• Xeroderma pigmentosum, xerostomia, xerosis, joint pain

• Xerostomia, xanthinuria, keratoconjunctivitis sicca, xerosis

Answer explanation

Sjögren syndrome presents with xerosis (dry skin), xerostomia (dry mouth), keratoconjunctivitis sicca (dry eyes), and joint pain. 

[Choice A]: Xanthomas are lipid deposits found in the skin of patients with hyperlipidemia. 

[Choice C]: Xeroderma pigmentosum is a hereditary condition characterized by dry skin, photosensitivity, and an increased risk of skin cancer due to defective DNA repair.

[Choice D]: Xanthinuria is a hereditary condition caused by a deficiency of the enzyme xanthine oxidase, which converts hypoxanthine and xanthine into uric acid in the final step of purine metabolism. Patients with this disease are at an increased risk of developing kidney stones.

Which of the following pharmacological agents is used to manage mild symptms of Sjogren Sx

• Antimuscarinics

• Sympathomimetics

• Anticholinergics

• Muscarinic agonists

Answer explanation

Patients with Sjögren syndrome typically have dry mouths. Muscarinic agonists, such as cevimeline and pilocarpine, are helpful in symptomatic management. Patients with severe symptoms will typically require further management with either immunosuppressants and/or glucocorticoids. 

[Choice A]: Antimuscarinics block muscarinic cholinergic receptors and interfere with parasympathetic nerve impulses (e.g., inhibition of secretions) which will only worsen the dry mouth.

[Choice B]: Sympathomimetics imitate the natural stimulation of the sympathetic nervous system and like antimuscarinics, have the opposite effects of muscarinic agonists.

[Choice C]: Anticholinergics inhibit the activity of acetylcholine in the central and peripheral nervous systems, leading to suppression of parasympathetic (“rest-and-digest”) functions, including salivation. Thus, anticholinergics will worsen dry mouth.

Key Takeaway:

• Mild symptoms of Sjögren syndrome can be managed with muscarinic agonists whereas severe symptoms may require the use of immunosuppressants and/or glucocorticoids

Which of the following complications is assocaited with Sjogren Sx?

• Decreased incidence of dental caries

• Achalasia

• MALT lymphoma

• Trachoma

Answer explanation

Patients with Sjögren syndrome have an increased risk of developing B-cell lymphoma.

[Choice A]: Sjögren syndrome increases the incidence of dental caries(cavities) due to dry mouth. 

[Choice B]: Sjögren syndrome patients may also suffer from difficulty chewingand swallowing due to a dry mouth. Achalasia presents similarly but is instead caused by damaged nerves in the esophagus, rendering it unable to transfer food properly from the mouth to the stomach. 

[Choice D]: Trachoma is a highly contagious eye disease due to infection by the bacterium Chlamydia trachomatis that can lead to irreversible vision loss if not treated early. 

Which of the following serological markers is most specific to support a diagnosis of Sjogren Sx?

• Rheumatoid factor

• Antinuclear antibody

• Decreased c-reactive protein

• Increased erythrocyte sedimentation rate

• Anti-La

Answer explanation

The two autoantibodies most specific for a diagnosis of Sjögren syndrome are anti-La (SSB) and anti-Ro (SS-A).

[Choices A, B, C, and D]: Non-specific serological markers for Sjögren syndrome can include positive rheumatoid factor (RF), positive antinuclear antibody (ANA), an elevated c-reactive protein (CRP), and an elevated erythrocyte sedimentation rate (ESR).