68: Sjögren Syndrome Flashcards
1
Q
What is the primary characteristic of Sjögren syndrome?
A
Sjögren syndrome is characterized by chronic lymphocytic infiltration and destruction of exocrine glands and epithelia, leading to dry mouth and dry eyes.
2
Q
What are the common systemic manifestations associated with primary Sjögren syndrome (pSS)?
A
Common systemic manifestations of pSS include:
- Mild sicca symptoms
- Fatigue
- Arthralgias
- Severe systemic symptoms involving multiple organ systems.
3
Q
What is the estimated annual incidence rate of primary Sjögren syndrome (pSS)?
A
The estimated annual incidence rate of pSS is 3.9 to 5.3 per 100,000 individuals.
4
Q
What role does the Type 1 interferon (IFN) signaling pathway play in Sjögren syndrome?
A
The Type 1 interferon (IFN) signaling pathway plays a central role in Sjögren syndrome by contributing to apoptosis in epithelial cells of damaged salivary glands and activating autoreactive lymphocytes and autoantibodies, perpetuating chronic inflammation.
5
Q
What are some environmental factors implicated in the pathogenesis of Sjögren syndrome?
A
Environmental factors implicated in the pathogenesis of Sjögren syndrome include:
- Viruses: especially Epstein–Barr virus (EBV), Hepatitis C virus, and HIV.
- Chronic inflammation in salivary glands.
- Other viruses: cytomegalovirus, coxsackie A virus, and endogenous retroviruses.
6
Q
What is the gender distribution of Sjögren syndrome?
A
Sjögren syndrome has a strong predominance in females, with a female to male ratio of 9:1.
7
Q
What are some genetic factors associated with primary Sjögren syndrome (pSS)?
A
Genetic factors associated with pSS include:
- HLA DQA1*0501
- HLA DQB1*0201
- Susceptibility genes such as IRF5, STAT4, IL12A, BLK, and CXCR5.
8
Q
What role does the Type 1 interferon (IFN) signaling pathway play in the pathogenesis of primary Sjögren syndrome (pSS)?
A
The Type 1 IFN signaling pathway is central to the pathogenesis of pSS, contributing to increased apoptosis in epithelial cells of damaged salivary glands, activation of plasmacytoid dendritic cells (pDCs), autoreactive lymphocytes, and the production of autoantibodies, which perpetuate chronic inflammatory processes.
9
Q
How do genetic factors contribute to the susceptibility of primary Sjögren syndrome (pSS)?
A
Genetic factors such as specific HLA class genes (e.g., HLA DQA10501, HLA DQB10201) and susceptibility genes identified in genomewide association studies (GWAS) like IRF5, STAT4, and IL12A are associated with pSS. These genes are involved in immune regulation and the Type I IFN pathway, influencing the disease’s development.
10
Q
What environmental factors are implicated in the development of primary Sjögren syndrome (pSS)?
A
Environmental factors include viral infections, particularly Epstein–Barr virus (EBV), which replicates in salivary glands, and other viruses like Hepatitis C and HIV. These factors contribute to chronic inflammation in the salivary glands, which is associated with the disease’s pathogenesis.
11
Q
What is the significance of the ‘IFN signature’ in patients with primary Sjögren syndrome (pSS)?
A
The ‘IFN signature’ refers to the overexpression of IFN-stimulated genes and proteins in approximately 50% of pSS patients. This signature is confirmed in peripheral blood and/or salivary glands and indicates heightened Type I IFN activity, which is a key feature of the disease.
12
Q
How does the presence of anti-Ro/SSA and anti-La/SSB antibodies correlate with the risk of lymphoma in primary Sjögren syndrome (pSS)?
A
The presence of anti-Ro/SSA and anti-La/SSB antibodies in pSS patients correlates with an increased risk of developing lymphoma, with studies indicating that about 1 in 5 pSS patients may develop lymphoma, particularly in the context of B-cell activation and proliferation.
13
Q
What is the role of CD4+ T lymphocytes in the pathogenesis of primary Sjögren’s syndrome (pSS)?
A
CD4+ T lymphocytes are involved in the immune response by interacting with antigen-presenting cells, leading to the recruitment of inflammatory cells and contributing to the autoimmune process in pSS.
14
Q
What are the characteristic features of xerostomia in primary Sjögren’s syndrome?
A
Xerostomia, or dry mouth, is characterized by:
- Decreased saliva secretion
- Persistent dryness throughout the day and night
- Difficulty in chewing and swallowing dry foods
- Altered sense of taste and burning discomfort
- Physical examination may reveal a red and fissured tongue with atrophy of the filiform papillae.
15
Q
What are the common extraglandular manifestations associated with primary Sjögren’s syndrome?
A
Common extraglandular manifestations include:
- Vasculitis
- Peripheral neuropathy
- Hematologic abnormalities
- Lymphoma
- Cryoglobulinemia
- Primary biliary cirrhosis
- Raynaud phenomenon
- Fatigue and myalgia.
16
Q
What is the significance of anti-Ro/SSA and anti-La/SSB antibodies in primary Sjögren’s syndrome?
A
Anti-Ro/SSA and anti-La/SSB antibodies are hallmark autoantibodies in systemic autoimmune diseases, particularly in pSS. They are associated with:
- Targeting ribonucleoprotein antigens
- High prevalence in patients with pSS
- Potential to cause fetal heart block in newborns of affected women.
17
Q
What are the clinical findings associated with keratoconjunctivitis sicca in primary Sjögren’s syndrome?
A
Keratoconjunctivitis sicca is characterized by:
- Ocular dryness
- Burning and itching sensation in the eyes
- Lack of tear production
- Intolerance to contact lenses
- Corneal injection and mucous discharge in the lower fornix
- Enlarged lacrimal glands.
18
Q
What is the significance of rheumatoid factor positivity in a patient with pSS?
A
Rheumatoid factor positivity is a marker of systemic autoimmune response in pSS.
19
Q
What is the significance of ANA positivity in a patient with pSS?
A
ANA positivity is a marker of systemic autoimmune response in pSS.
20
Q
What is the significance of anti-Ro/SSA antibody positivity in a patient with pSS?
A
Anti-Ro/SSA antibody positivity is associated with extraglandular manifestations, vasculitis, and hematologic abnormalities in pSS.
21
Q
What is the significance of anti-La/SSB antibody positivity in a patient with pSS?
A
Anti-La/SSB antibody positivity is associated with systemic and hematologic alterations in pSS.
22
Q
What is the significance of anticentromere autoantibody positivity in a patient with pSS?
A
Anticentromere autoantibody positivity is associated with increased risk of Raynaud phenomenon, thyroid dysfunction, vasculitis, and peripheral neuropathy in pSS.
23
Q
What should be your primary concern with a patient with pSS who develops unilateral parotid gland enlargement?
A
Persistent unilateral parotid gland enlargement in pSS raises suspicion for lymphoma.
24
Q
What serological findings might you expect in a patient with pSS who has a history of peripheral neuropathy?
A
Serological findings may include anti-Ro/SSA and anti-La/SSB antibodies, rheumatoid factor, and ANA positivity.
25
What role do epithelial cells play in the pathogenesis of primary Sjögren's syndrome (pSS)?
Epithelial cells in pSS are actively involved in disease pathogenesis through mechanisms such as:
1. **Autoimmune Epithelitis**: They express HLA class II molecules and costimulator CD86, interacting with CD28 on T cells to recruit inflammatory cells.
2. **Environmental Damage**: Damage from environmental factors (e.g., viruses) leads to dysfunction and increased apoptosis of epithelial cells, activating Type I IFN production by plasmacytoid dendritic cells (pDCs).
3. **Antigen Presentation**: Apoptotic epithelial cells relocalize autoantigens (Ro/SSA and La/SSB) to the cell surface, presenting them to autoantibodies.
26
How do xerostomia and keratoconjunctivitis sicca manifest in patients with primary Sjögren's syndrome?
In primary Sjögren's syndrome, xerostomia and keratoconjunctivitis sicca present with the following features:
**Xerostomia (Dry Mouth)**:
- Principal symptom with decreased saliva secretion.
- Persistent dryness throughout the day and night, significantly affecting quality of life.
- Difficulty in chewing and swallowing dry foods.
- Altered taste sensation and burning discomfort from acidic or spicy foods.
- Physical examination may reveal a red and fissured tongue with atrophy of the filiform papillae.
**Keratoconjunctivitis Sicca (Dry Eyes)**:
- Characterized by burning and itching sensations in the eyes, often exacerbated by smoke.
- Lack of tear production and intolerance to contact lenses.
- Paradoxically, tear production during crying may remain unaffected.
- May present with corneal injection and mucous discharge in the lower fornix.
27
What are the implications of the presence of anti-Ro/SSA and anti-La/SSB antibodies in patients with primary Sjögren's syndrome?
The presence of anti-Ro/SSA and anti-La/SSB antibodies in patients with primary Sjögren's syndrome has several clinical implications:
1. **Autoimmune Response**: These antibodies are hallmarks of systemic autoimmune diseases and indicate an autoimmune response.
2. **Fetal Heart Block**: Women with these antibodies may give birth to newborns with fetal heart block.
3. **Association with Other Conditions**: Their presence is associated with other autoimmune diseases and can help distinguish primary Sjögren's syndrome from other causes of salivary or lacrimal gland dysfunction.
4. **Clinical Manifestations**: Patients may experience systemic symptoms, including hematologic and immunologic alterations, such as Raynaud phenomenon and peripheral neuropathy.
28
What are the common extraglandular manifestations associated with primary Sjögren's syndrome?
Common extraglandular manifestations associated with primary Sjögren's syndrome include:
| Manifestation | Description |
|-----------------------------------|-----------------------------------------------------------------------------|
| **Vasculitis** | Inflammation of blood vessels, leading to various systemic symptoms. |
| **Hematologic Abnormalities** | Includes conditions like anemia and leukopenia. |
| **Lymphoma** | Increased risk of developing lymphoma, particularly non-Hodgkin lymphoma. |
| **Peripheral Neuropathy** | Nerve damage leading to pain, weakness, or numbness in extremities. |
| **Raynaud Phenomenon** | Episodes of reduced blood flow to fingers and toes, causing color changes. |
| **Primary Biliary Cirrhosis** | Liver condition that may co-occur with Sjögren's syndrome.
29
What are the most common symptoms associated with Sjögren Syndrome?
The most common symptoms include:
- **Nonspecific pruritus**
- **Burning sensations**
- **Pin prick-like feeling**
30
What is the significance of hypergammaglobulinemic purpura in Sjögren Syndrome?
Hypergammaglobulinemic purpura is characterized by:
- **Purpuric macules**, which are very common in SS
- **Flat, nonpalpable, blanching purpura**
- Associated with **benign hypergammaglobulinemic purpura**
- Skin biopsies show **ruptured blood vessels with complement deposition**.
31
What are the characteristics of annular erythema associated with primary Sjögren Syndrome?
Characteristics of annular erythema include:
- Primarily seen in **Asian patients** with high levels of **anti-Ro/SSA** and **anti-La/SSB** antibodies
- Erythematous lesions with a **wide elevated border** and **central clearing**
- May start from a small indurate erythema and expand to an **annular form**
- Localized mainly on the **faces**, arms, trunk, and proximal thighs
- Triggers include **sunlight, cold exposure, mental stress, and pregnancy**.
32
What types of cutaneous vasculitis are associated with Sjögren Syndrome?
Types of cutaneous vasculitis include:
1. **Palpable purpura**
- Does not blanch on pressure
- Associated with dermal vasculitis and extravasation of red blood cells
- Commonly found on lower extremities and buttocks
- Important marker of more severe disease
2. **Urticarial vasculitis**
- Second most frequent form of CV in SS
- Pruritic wheals with erythema that last for more than 24 hours
- Resolves with hyperpigmentation
3. **Necrotizing vasculitis**
- Palpable purpuric lesions that ulcerate and resolve within 1 to 4 weeks
- Associated with arthritis, Raynaud phenomenon, and other systemic symptoms.
33
What is the relationship between cutaneous manifestations and systemic autoimmunity in Sjögren Syndrome?
Cutaneous manifestations in Sjögren Syndrome are associated with systemic autoimmunity, including:
- **Antinuclear antibodies**
- **Anti-Ro/SSA and anti-La/SSB antibodies**
- **Hypergammaglobulinemia**
- **Rheumatoid factor**
- Increased risk of **lymphoma development** and higher **mortality rates** in patients with cutaneous vasculitis.
34
What is the significance of recurrent Raynaud phenomenon in a patient with pSS?
Raynaud phenomenon can precede sicca symptoms by many years and is not accompanied by telangiectasias, unlike systemic sclerosis.
35
What is the likely diagnosis for a patient with pSS who has recurrent erythematous macules that do not blanch on pressure?
The patient likely has cutaneous vasculitis, which is an important marker of more severe disease and is associated with an increased risk of lymphoma development.
36
What condition should you suspect in a patient with pSS presenting with painful nodular eruptions on the anterior surface of the lower extremities?
Suspect erythema nodosum, which is rare in pSS and may raise suspicion for sarcoidosis.
37
What is the likely cause of recurrent epistaxis and hoarseness in a patient with pSS?
The likely cause is dryness of the upper respiratory tract, a common manifestation in pSS.
38
What is the underlying mechanism of hypergammaglobulinemic purpura in a patient with pSS?
Hypergammaglobulinemic purpura is associated with polyclonal hypergammaglobulinemia and rheumatoid factor positivity, with skin biopsies showing ruptured blood vessels and complement deposition.
39
What are the typical triggers for annular erythema in a patient with pSS?
Triggers for annular erythema include sunlight, cold exposure, mental stress, and pregnancy.
40
What is the underlying mechanism of hypergammaglobulinemic purpura in pSS?
Hypergammaglobulinemic purpura is associated with polyclonal hypergammaglobulinemia and rheumatoid factor positivity, with skin biopsies showing ruptured blood vessels and complement deposition.
41
What are the typical triggers for annular erythema in pSS?
Triggers for annular erythema include sunlight, cold exposure, mental stress, and pregnancy.
42
What is the likely cause of recurrent vaginal infections in pSS?
Recurrent vaginal infections in pSS are likely due to vaginal dryness.
43
What condition should you suspect with recurrent erythema multiforme-like lesions in pSS?
Suspect erythema perstans, which may occur in pSS.
44
What are the most common symptoms associated with Sjögren Syndrome?
The most common symptoms include nonspecific pruritus, burning sensations, and pin prick-like feeling.
45
How does hypergammaglobulinemic purpura present in Sjögren Syndrome?
It typically presents as purpuric macules, associated with benign hypergammaglobulinemic purpura, characterized by polyclonal hypergammaglobulinemia and rheumatoid factor positivity.
46
What are the characteristics of cutaneous vasculitis in Sjögren Syndrome?
Cutaneous vasculitis is characterized by palpable purpura, typically occurring on the lower extremities and buttocks, and indicates a higher risk of severe disease.
47
What triggers annular erythema associated with primary Sjögren Syndrome?
Triggers include sunlight exposure, cold exposure, mental stress, and pregnancy.
48
What are common cutaneous manifestations associated with Sjögren Syndrome?
Common manifestations include acrocyanosis, pernio-like eruptions, livedo, periungual hemorrhage, photosensitivity, indurated erythema, alopecia, vitiligo vulgaris, lichen planus, sarcoidosis, granuloma annulare, and psoriasis.
49
What are the musculoskeletal manifestations frequently observed in Sjögren Syndrome?
Musculoskeletal manifestations include symmetric nonerosive polyarthritis, arthralgias, and myalgias.
50
What are the common neurologic manifestations in patients with Sjögren Syndrome?
Common neurologic manifestations include peripheral axonal polyneuropathies, ganglionopathy, cranial neuropathies, and CNS manifestations.
51
What are the diagnostic criteria for primary Sjögren Syndrome (pSS)?
At least 4 criteria must be met, including evidence of lymphocytic infiltration or presence of autoantibodies.
52
What is the significance of lymphoproliferative disease in patients with Sjögren Syndrome?
There is an increased risk of developing lymphomas, with specific predictors including palpable purpura and low C4 serum levels.
53
What is the likely underlying mechanism for recurrent Bell palsy in pSS?
Bell palsy is due to lymphocytic infiltration affecting the facial nerve.
54
What cranial nerves are likely involved in recurrent diplopia in pSS?
Diplopia may involve the oculomotor, trochlear, or abducent nerves.
55
What is the significance of recurrent splenomegaly in pSS?
Splenomegaly is a significant prognostic factor for lymphoproliferative disease.
56
What is the significance of recurrent low C3 serum levels in pSS?
Low C3 serum levels are a significant prognostic factor for lymphoproliferative disease.
57
What is the significance of recurrent lymphopenia in pSS?
Lymphopenia is a significant prognostic factor for lymphoproliferative disease.
58
What is the significance of recurrent neutropenia in pSS?
Neutropenia is a significant prognostic factor for lymphoproliferative disease.
59
What diagnostic criteria confirm primary Sjögren syndrome (pSS) in a patient with xerostomia and keratoconjunctivitis sicca?
The revised American-European classification system requires at least 4 criteria, including evidence of lymphocytic infiltration or presence of specific autoantibodies.
60
What is the most likely cause of peripheral axonal polyneuropathies in pSS?
They are due to lymphocytic infiltration affecting the peripheral nervous system.
61
What is the likely underlying cause of recurrent miscarriages in pSS?
Recurrent miscarriages are likely due to placental insufficiency associated with the autoimmune background.
62
What maternal antibodies are responsible for neonatal lupus in pSS?
Maternal anti-Ro/SSA and anti-La/SSB antibodies are responsible.
63
What other cutaneous manifestations might be expected in a patient with photosensitivity and alopecia in pSS?
Other manifestations may include indurated erythema, vitiligo vulgaris, lichen planus, sarcoidosis, granuloma annulare, and psoriasis.
64
What other renal manifestations might be expected in a patient with interstitial nephritis in pSS?
Other renal manifestations include renal tubular acidosis and, rarely, glomerulonephritis.
65
What other pulmonary manifestations might be expected in a patient with interstitial pneumonitis in pSS?
Other pulmonary manifestations include dry cough and MALT lymphoma.
66
What is the likely cause of dysphagia in pSS?
Dysphagia is likely due to xerostomia and esophageal dysmotility.
67
What are the common musculoskeletal manifestations associated with Sjögren Syndrome?
Common manifestations include symmetric nonerosive polyarthritis, absence of rheumatoid factor, and absence of erosions on radiographs.
68
How does Sjögren Syndrome affect pregnancy outcomes?
It increases the risk of spontaneous abortions, preterm deliveries, low-body-weight infants, and congenital heart block.
69
What are the diagnostic criteria for primary Sjögren Syndrome (pSS)?
At least 4 criteria must be met, including subjective complaints of dry eyes or dry mouth.
70
What are the potential neurological manifestations of Sjögren Syndrome?
Potential manifestations include peripheral axonal polyneuropathies, ganglionopathy, cranial neuropathies, and CNS manifestations.
71
What is the significance of palpable purpura in patients with Sjögren Syndrome?
Palpable purpura is a significant predictor of non-Hodgkin lymphoma and lymphoproliferative diseases.
72
What are the criteria for diagnosing ocular symptoms in Sjögren Syndrome?
Criteria include persistent dry eyes for more than 3 months, recurrent sensation of sand in the eyes, and use of tear substitutes more than 3 times a day.
73
What is the significance of the Schirmer test in diagnosing Sjögren Syndrome?
A result of less than 5 mm of wetting in 5 minutes indicates dry eye, a symptom of Sjögren Syndrome.
74
What are the common laboratory findings associated with Sjögren Syndrome?
Common findings include increased inflammatory markers, anemia, hypoalbuminemia, and hyperglobulinemia.
75
What histopathological feature is most indicative of Sjögren Syndrome?
A biopsy showing a focal, periductal infiltrate composed of T and B lymphocytes.
76
What are the two patterns of primary Sjögren Syndrome (pSS) and their associated risks?
Type I is high-risk with low complement C4 levels and increased lymphoma risk. Type II is low-risk with a more benign course.
77
What are the main treatment strategies for managing dry mouth in Sjögren Syndrome?
Treatment is largely symptomatic, focusing on adequate hydration and reducing irritants.
78
What is the significance of recurrent low C4 serum levels in pSS?
Low C4 serum levels are a predictor of lymphoproliferative disease and increased mortality.
79
What is the significance of recurrent palpable purpura in pSS?
Palpable purpura is a predictor of lymphoproliferative disease and increased mortality.
80
What is the significance of recurrent cryoglobulins in pSS?
Cryoglobulins are associated with increased risk of parotid enlargement, vasculitis, and leukoplakia.
81
What is the significance of recurrent anemia in pSS?
Anemia is a common sign of chronic inflammation.
82
What is the significance of recurrent hypoalbuminemia in pSS?
Hypoalbuminemia is a common sign of chronic inflammation.
83
What is the significance of recurrent hyperglobulinemia in pSS?
Hyperglobulinemia is present in 80% of pSS patients and is associated with systemic inflammation.
84
What does a biopsy showing focal, periductal infiltrate composed of T and B lymphocytes indicate?
This histologic hallmark is indicative of Sjögren syndrome.
85
What does a positive Schirmer test indicate?
It indicates decreased tear production, consistent with keratoconjunctivitis sicca.
86
What are the key diagnostic criteria for ocular symptoms in Sjögren Syndrome according to the Revised International Classification?
Criteria include persistent dry eyes for more than 3 months, recurrent sensation of sand in the eyes, and use of tear substitutes more than 3 times a day.
87
What laboratory findings are commonly associated with chronic inflammation in Sjögren Syndrome?
Findings include anemia, hypoalbuminemia, hyperglobulinemia, and elevated inflammatory markers.
88
What is the significance of the focus scoring system in the histopathology of Sjögren Syndrome?
It evaluates lymphocytic infiltration in minor salivary glands, indicating the presence of Sjögren Syndrome.
89
What is a focus in the context of Sjögren Syndrome?
A focus is defined as a conglomeration of at least 50 lymphocytes per 4 mm of glandular tissue, indicating the presence of Sjögren Syndrome.
90
What are the two patterns of primary Sjögren Syndrome (pSS)?
1. **High-risk disease syndrome (Type I)**: ~20% of cases, low complement C4 levels, palpable purpura (early), significantly increased risk of lymphoproliferative disease and higher mortality rate.
2. **Low-risk (Type II)**: ~80% of cases, no predictors of high risk, no increased risk of death, more benign course dominated by sicca symptoms.
91
What nonpharmacologic strategies are recommended for managing dry mouth in Sjögren Syndrome?
- Adequate hydration
- Reduction of irritants such as coffee, alcohol, and nicotine.
92
What are the recommended nonpharmacologic measures for managing dry mouth in Sjögren Syndrome?
- Frequent sips of water to maintain moisture
- Use of sugar-free candies and chewing gum to increase saliva production
- Avoid sugar-containing foods to reduce the risk of dental caries and oral candidiasis
- Maintain meticulous dental hygiene to prevent or treat dental caries
- Use fluoride application for prevention
- Treat oral candidiasis with antifungal therapy and avoid wearing dentures at night.
93
What pharmacologic therapies are available for managing dry mouth in Sjögren Syndrome?
- **Secretagogue drugs**: Pilocarpine (5 mg 4 times/day), Cevimeline (30 mg 3 times/day).
- These drugs act on muscarinic receptors to increase exocrine gland secretion.
- Contraindications include narrow-angle glaucoma and uncontrolled asthma.
- Common side effects include excessive sweating, urinary frequency, and flushing.
94
What are the first-line nonpharmacologic measures for managing dry eyes in Sjögren Syndrome?
- Avoid dry, smoky, and windy environments
- Avoid contact lenses or choose those with higher water content
- Minimize medications that inhibit tear production (e.g., diuretics, β-blockers, tricyclic antidepressants, antihistamines)
- Use preservative-free tear substitutes for frequent use.
- Consider surgical options like punctal occlusion to block tear drainage for increased moisture.
95
What are the extraglandular manifestations associated with Sjögren Syndrome?
- Reduced tolerance to nonsteroidal antiinflammatory drugs
- Symptoms may include: arthralgia/arthritis, myalgia, fatigue, annular erythema, dryness of the eyes and mouth.
- Corticosteroids and immunosuppressive drugs may be used for visceral manifestations such as vasculitis, pneumonitis, and glomerulonephritis.
96
What is the role of rituximab in the treatment of Sjögren Syndrome?
- Rituximab is a monoclonal antibody against CD20 that has shown: improved stimulated salivary flows, better laboratory parameters of inflammation, subjective symptom relief.
- It also leads to reduced glandular infiltration and morphologic improvements in epithelial cells on salivary gland biopsies.
97
What nonpharmacologic measures can you recommend for a patient with pSS reporting burning sensations in the eyes?
Avoid dry, smoky, and windy environments, minimize medications that inhibit tear production, and use preservative-free tear substitutes.
98
What preventive measures can you recommend for a patient with pSS who has recurrent oral thrush?
Preventive measures include meticulous dental hygiene, avoiding sugar-containing foods, and not wearing dentures at night.
99
What nonpharmacologic measures can be taken to manage dry mouth in Sjögren Syndrome patients?
- Frequent sips of water to maintain moisture
- Mechanical stimulation of saliva secretion (sugar-free candies and chewing gum)
- Use of saliva substitutes (gels, oils, sprays)
- Avoidance of sugar-containing foods to reduce risk of dental caries
- Meticulous dental hygiene to prevent or treat dental caries
- Prevention of oral candidiasis with antifungal therapy and proper denture care.
100
What are the pharmacologic options for treating dry mouth in Sjögren Syndrome?
- **Secretagogue drugs**: Pilocarpine (5 mg 4 times/day), Cevimeline (30 mg 3 times/day).
- These drugs act on muscarinic receptors to increase exocrine gland secretion but are contraindicated in narrow-angle glaucoma and uncontrolled asthma.
- Common side effects include excessive sweating, urinary frequency, and flushing.
101
What are the first-line nonpharmacologic measures for managing dry eyes in Sjögren Syndrome?
- Avoidance of dry, smoky, and windy environments
- Avoiding contact lenses or opting for those higher in water content
- Minimizing medications that inhibit tear production (e.g., diuretics, β-blockers, tricyclic antidepressants, antihistamines)
- Use of preservative-free tear substitutes, especially for frequent use
- Surgical options like occlusion of the puncta to block tear drainage for increased moisture.
102
What are the treatment options for extraglandular manifestations in Sjögren Syndrome?
- **Antimalarial drugs** (e.g., hydroxychloroquine) for arthralgia/arthritis, myalgia, and fatigue
- **Corticosteroids and immunosuppressive drugs** for visceral manifestations such as vasculitis and pneumonitis
- **Watchful waiting** for low-grade B-cell lymphomas localized to exocrine glands
- **Rituximab** and cytotoxic regimens for higher-grade lymphomas.
103
What molecular target therapies are being explored for Sjögren Syndrome?
- **Rituximab** (monoclonal antibodies against CD20) has shown improved stimulated salivary flows and reduced glandular infiltration.
- **Epratuzumab** (monoclonal antibodies against CD22) is also being investigated.
- Other potential targets include cytokines (IL-6, BlyS), interferons, adhesion molecules, and chemokines.
- Gene therapy restricted to salivary and lacrimal glands may alter the immune response locally while avoiding systemic side effects.
