sjogren's syndrome

Question 1

What is Sjögren’s Syndrome?

Answer 1

Sjögren’s Syndrome is an autoimmune condition that affects the exocrine glands, characterized by lymphocytic infiltrates.

Question 2

What is the prevalence of Sjögren’s Syndrome in females?

Answer 2

The prevalence of Sjögren’s Syndrome is higher in females (9:1 ratio), and it mainly occurs in middle-aged women.

Question 3

Can Sjögren’s Syndrome be secondary to other autoimmune conditions?

Answer 3

Yes, Sjögren’s Syndrome can be a primary condition or can occur secondary to other autoimmune conditions such as rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE).

Question 4

What is the pathophysiology of Sjögren’s Syndrome?

Answer 4

Sjögren’s Syndrome is caused by lymphocyte-mediated autoimmune destruction (type IV hypersensitivity) of minor salivary and lacrimal glands, leading to atrophy and fibrosis. Patients present with dry eyes and mouth.

Question 5

What are the common symptoms of Sjögren’s Syndrome?

Answer 5

Common symptoms include reduced tear secretion (keratoconjunctivitis sicca), dryness of the mouth (xerostomia), vaginal dryness, and extraglandular features such as arthritis and cutaneous vasculitis.

Question 6

What signs might be observed in Sjögren’s Syndrome?

Answer 6

Signs of Sjögren’s Syndrome include bilateral parotid gland enlargement, dental caries, oral candidiasis, and a fissured tongue.

Question 7

How is ocular dryness assessed in Sjögren’s Syndrome?

Answer 7

Ocular dryness is assessed using the Schirmer’s test, which demonstrates reduced tear production. A positive result is wetting of <5 mm on a strip of filter paper on the lower eyelid.

Question 8

What blood markers are usually raised in Sjögren’s Syndrome?

Answer 8

Markers of inflammation are usually raised in Sjögren’s Syndrome. Most patients are ANA positive, and rheumatoid factor is positive in 90% of cases. Anti-Ro and anti-La autoantibodies are present in 40–90% of patients.

Question 9

What diagnostic procedure confirms Sjögren’s Syndrome?

Answer 9

Salivary gland biopsy, showing histology with lymphocytic infiltrates, can confirm the diagnosis of Sjögren’s Syndrome.

Question 10

How is Sjögren’s Syndrome managed for symptom control?

Answer 10

Symptom control involves tear and salivary replacement for dryness, along with analgesia and exercise for pain and fatigue. Hydroxychloroquine may help with arthralgia and fatigue.

Question 11

What complications are associated with Sjögren’s Syndrome?

Answer 11

Sjögren’s Syndrome increases the risk of lymphoma, particularly B-cell lymphoma, in affected glands and organs. Peripheral neuropathy and interstitial lung disease can also occur, but organ complications are less common.

Question 12

In what context would immunosuppression be used in Sjögren’s Syndrome management?

Answer 12

Immunosuppression would usually only be used in the context of organ involvement, such as interstitial lung disease.

Question 13

What surveillance is recommended for Sjögren’s Syndrome complications?

Answer 13

Sjögren’s Syndrome is less commonly associated with organ complications than other connective tissue diseases, and surveillance is not performed routinely. Patients with new or changing lymphadenopathy should be investigated promptly.

Question 14

What is Sjögren’s syndrome?

Answer 14

Sjögren’s syndrome is an autoimmune condition that affects the exocrine glands, particularly the lacrimal and salivary glands, leading to symptoms such as dry mouth, eyes, and vagina.

Question 15

What are the sicca symptoms associated with Sjögren’s syndrome?

Answer 15

Sicca symptoms include dry eyes and dry mouth.

Question 16

What is the difference between Primary and Secondary Sjögren’s?

Answer 16

Primary Sjögren’s occurs in isolation, while Secondary Sjögren’s is associated with other diseases such as systemic lupus erythematosus and rheumatoid arthritis.

Question 17

Which antibodies are associated with Sjögren’s syndrome?

Answer 17

Antibodies associated with Sjögren’s include Anti-SS-A (anti-Ro) and Anti-SS-B (anti-La) antibodies.

Question 18

Describe the Schirmer test and its significance.

Answer 18

The Schirmer test involves placing filter paper under the lower eyelid, measuring the distance moisture travels along it after 5 minutes. In a healthy adult, 15mm is expected, and less than 10mm is significant.

Question 19

How is Sjögren’s syndrome diagnosed?

Answer 19

Diagnosis is typically based on clinical features and the presence of antibodies. Salivary gland biopsy may be used for confirmation but is not usually necessary.

Question 20

What are the management options for Sjögren’s syndrome?

Answer 20

Management involves rheumatology and ophthalmology. Treatment options include artificial tears, artificial saliva, vaginal lubricants, oral pilocarpine, and hydroxychloroquine for joint pain.

Question 21

How does Pilocarpine work in the management of Sjögren’s syndrome?

Answer 21

Pilocarpine stimulates muscarinic receptors, activating parasympathetic nerves and promoting salivary and lacrimal gland secretion.

Question 22

What complications can arise from exocrine gland dysfunction in Sjögren’s syndrome?

Answer 22

Complications include eye problems like keratoconjunctivitis sicca and corneal ulcers, oral problems such as dental cavities and candida infections, and vaginal problems like candida infection and sexual dysfunction.

Question 23

What other organ complications can occur in Sjögren’s syndrome?

Answer 23

Sjögren’s syndrome can rarely affect other organs, leading to complications such as pneumonia, bronchiectasis, non-Hodgkin’s lymphoma, peripheral neuropathy, vasculitis, and renal impairment.

Question 24

What symptoms does Patricia present with?

Answer 24

Patricia presents with fatigue, dry skin, red eyes, recurrent sensation of sand in her eyes, dry mouth, and cold-induced white and painful fingertips.

Question 25

What signs were observed on Patricia’s examination?

Answer 25

Signs include tooth decay and purpura on both lower extremities. Sialometry detected salivary hypofunction.

Question 26

What is the likely diagnosis for Patricia?

Answer 26

The likely diagnosis for Patricia is Sjogren syndrome.

Question 27

What are the target glands in Sjogren syndrome?

Answer 27

Sjogren syndrome primarily affects exocrine glands, especially salivary and lacrimal glands.

Question 28

How is Sjogren syndrome classified based on occurrence?

Answer 28

Sjogren syndrome can be classified as primary (sicca syndrome) when occurring alone or secondary when accompanied by other autoimmune diseases like lupus, rheumatoid arthritis, or scleroderma.

Question 29

What are the potential causes of Sjogren syndrome?

Answer 29

The exact cause of Sjogren syndrome is unknown, but genetic and environmental factors are believed to be involved.

Question 30

What triggers the immune response in Sjogren syndrome?

Answer 30

Nuclear components that leak out of dead or damaged cells in the body are perceived as antigens by helper T-cells in Sjogren syndrome.

Question 31

Which antibodies are formed in Sjogren syndrome?

Answer 31

Anti-SSA/RO and anti-SSB/LA antibodies, formed against ribonucleoproteins SS-A and SS-B, are produced in Sjogren syndrome.

Question 32

How do T-cells and antibodies contribute to gland damage in Sjogren syndrome?

Answer 32

Activated T-cells and antibodies enter circulation and reach exocrine glands, leading to lymphocytic infiltration and damage. Cytokines secreted by T-cells recruit more immune cells, resulting in fibrous tissue production and gland damage.

Question 33

What is the end result of the immune response in Sjogren syndrome?

Answer 33

The end result is a loss of secretory cells in the glands due to the damage caused by lymphocytic infiltration and fibrous tissue replacement.

Question 34

What are the glandular manifestations of Sjogren syndrome?

Answer 34

Glandular manifestations include decreased tear secretion leading to dry eyes, blurry vision, irritation, keratoconjunctivitis, diffuse fibrosis and painless enlargement of salivary glands causing xerostomia, mucosal atrophy, fissuring of the tongue, and dental issues.

Question 35

What are the potential complications of parotid gland involvement in Sjogren syndrome?

Answer 35

Parotid gland fibrosis can lead to bilateral parotid enlargement, potentially compressing nearby nerves and causing pain.

Question 36

How does Sjogren syndrome affect the nose and airways?

Answer 36

Sjogren syndrome causes dryness in the nose and airways, leading to ulceration and bleeding. If it affects the larynx, it can result in difficulty speaking (dysarthria).

Question 37

What extraglandular manifestations can occur in Sjogren syndrome?

Answer 37

Extraglandular manifestations include systemic symptoms such as fever, fatigue, myalgia, unintentional weight loss, and lymphadenopathy. Joint involvement can lead to arthralgia or inflammatory arthritis. Vascular conditions like purpura and Raynaud’s phenomenon may occur. Lung problems include chronic cough and interstitial lung disease. Renal involvement can cause interstitial nephritis and defects in tubular function. Autoimmune thyroiditis, hypergammaglobulinemia, monoclonal gammopathies, and cryoglobulinemia can also be associated. Over time, Sjogren syndrome can lead to lymphomas, particularly mucosa-associated lymphoid tissue (MALT) lymphoma.

Question 38

What is Raynaud’s phenomenon, and how is it related to Sjogren syndrome?

Answer 38

Raynaud’s phenomenon in Sjogren syndrome involves arterial spasms reducing blood flow to the fingers, causing them to turn white, then blue, often with numbness or pain, and finally turning red as blood flow returns.

Question 39

What is the potential renal involvement in Sjogren syndrome?

Answer 39

Renal involvement in Sjogren syndrome can cause interstitial nephritis and defects in tubular function, leading to an increase in creatinine levels.

Question 40

How can Sjogren syndrome be associated with lymphomas?

Answer 40

Over time, Sjogren syndrome can increase the risk of lymphomas, particularly mucosa-associated lymphoid tissue (MALT) lymphoma, which is typically associated with chronic inflammation.

Question 41

What type of lymphoma can be associated with Sjogren syndrome?

Answer 41

One type of lymphoma associated with Sjogren syndrome is mucosa-associated lymphoid tissue (MALT) lymphoma, which may present as nodular parotid enlargement instead of the diffuse enlargement normally seen in Sjogren syndrome.

Question 42

What are the criteria for diagnosing Sjogren syndrome?

Answer 42

Diagnosing Sjogren syndrome is based on symptoms persisting for more than three months, occurring daily, and relating to salivary gland, mouth, and eye involvement.

Question 43

What tests can identify salivary gland hypofunction in Sjogren syndrome?

Answer 43

Tests for salivary gland hypofunction include quantitative salivary gland scintigraphy (technetium excretion radionuclide scanning), whole sialometry, and imaging (ultrasound or MRI) to identify gland enlargement. Labial salivary gland biopsy is a confirmatory test, typically showing focal lymphocytic sialadenitis.

Question 44

How is eye involvement tested in Sjogren syndrome?

Answer 44

Eye involvement is tested using the Schirmer test for reflex tear production, ocular surface staining with fluorescein for signs of keratoconjunctivitis, and tear break-up time to assess the drying rate of tears over the eyes.

Question 45

What blood tests are relevant for diagnosing Sjogren syndrome?

Answer 45

Blood tests for Sjogren syndrome may show a low white blood cell count, elevated globulins, and an elevated ESR. Autoantibodies associated with primary Sjogren syndrome include anti-SSA/RO and anti-SSB/LA, while secondary disease may present with additional antibodies such as ANA and rheumatoid factor.

Question 46

Why might a pulmonary function test be necessary in Sjogren syndrome?

Answer 46

A pulmonary function test may be necessary to assess lung function, as lung involvement is common in Sjogren syndrome.

Question 47

What is the first-line treatment for individuals with mild Sjogren syndrome without other organ involvement?

Answer 47

Secretagogues, such as muscarinic agonists like pilocarpine and cevimeline, are the first-line treatment for individuals with mild Sjogren syndrome without other organ involvement.

Question 48

What are some symptoms that secretagogues can help relieve in Sjogren syndrome?

Answer 48

Secretagogues can help relieve symptoms such as dryness of the eyes and mouth by increasing the production of saliva and tears.

Question 49

What is the treatment approach for moderate to severe Sjogren syndrome with major salivary gland enlargement or involvement of other organs?

Answer 49

Individuals with moderate to severe Sjogren syndrome, with major salivary gland enlargement or involvement of other organs, generally require systemic medical therapy. Major salivary glandular enlargement due to inflammation can be treated with glucocorticoids. Disease-modifying anti-rheumatic drugs (DMARDs) are used for those with involvement of other organs.

Question 50

What are some non-biological DMARDs used in the treatment of Sjogren syndrome?

Answer 50

Non-biological DMARDs used in the treatment of Sjogren syndrome include hydroxychloroquine and methotrexate.

Question 51

What are some examples of biological DMARDs used for severe Sjogren syndrome?

Answer 51

Examples of biological DMARDs used for severe Sjogren syndrome include the alkylating agent cyclophosphamide and the anti-CD20 antibody rituximab, which targets B cells.

Question 52

What are some potential side effects of glucocorticoids used in Sjogren syndrome?

Answer 52

Glucocorticoids, used for treating major salivary glandular enlargement, can have potential side effects, including immunosuppression and other systemic effects.

Question 53

Why are pulmonary function tests recommended in Sjogren syndrome?

Answer 53

Pulmonary function tests are recommended in Sjogren syndrome to assess lung function, as lung involvement is common in this condition.

Question 54

What symptoms did Patricia present with that are suggestive of Sjogren syndrome?

Answer 54

Patricia presented with symptoms of fatigue, dry skin, dry mouth, red eyes, and Raynaud’s phenomenon.

Question 55

Why are these symptoms highly suggestive of Sjogren syndrome?

Answer 55

These symptoms are highly suggestive of Sjogren syndrome, especially since they persisted for more than three months. Signs of tooth decay and purpura on both lower extremities further support the diagnosis. Sialometry detected salivary hypofunction.

Question 56

What is the next step in the diagnosis of Sjogren syndrome for Patricia?

Answer 56

The next step in the diagnosis for Patricia would be blood work to look for anti-SSA/RO and anti-SSB/LA antibodies. Additionally, checking for signs of corneal ulceration and evaluating involvement of the lungs and other organs would be important.

Question 57

Why is blood work important in the diagnosis of Sjogren syndrome?

Answer 57

Blood work is crucial in the diagnosis of Sjogren syndrome as it helps identify specific autoantibodies associated with the condition, such as anti-SSA/RO and anti-SSB/LA antibodies. It also aids in assessing organ involvement, including the lungs.

Question 58

What other tests might be conducted to assess organ involvement in Sjogren syndrome?

Answer 58

Other tests that might be conducted to assess organ involvement in Sjogren syndrome include checking for signs of corneal ulceration and performing additional assessments for lung and systemic involvement.