Sjogren’s Flashcards

1
Q

Key Points:
•Lymphocytic attack on ? glands (? and ?)
•Classified as ? complex: dry ? and dry ?
•Symptoms are ? (primary) and ? (secondary).
•Secondary Sjögren’s is often associated with other ? conditions.
•Triggers include ? and ?.

A

Key Points:
•Lymphocytic attack on secretory glands (lacrimal and salivary)
•Classified as sicca complex: dry eyes and dry mouth
•Symptoms are glandular (primary) and extraglandular (secondary).
•Secondary Sjögren’s is often associated with other autoimmune conditions.
•Triggers include viral infections and hormone imbalances.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Etiology:
The etiology of Sjögren’s is largely unknown.

3 thoughts???

A

Etiology:
The etiology of Sjögren’s is largely unknown.

  1. •Genetics
    •Strongest association with HLA-DR region (HLA-DRB1)
    •Genes associated with innate immunity: IRF5 TNIP1 – affect cytokine production.
  2. •Hormones
  3. •Infections – viral
    •EBV and CMV- often raised antibodies to these
    •Coxsackievirus
    •Retroviruses – HIV and HTLV-1 can sometimes cause Sjögren-like symptoms of diffuse infiltrative lymphocytosis in salivary glands.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Clinical Features:

Exocrine gland features: 6

A

Clinical Features:
Exocrine gland features:

  1. •Keratoconjunctivitis sicca (dry eyes)
  2. •Xerostomia (dry mouth)
  3. •Salivary gland enlargement (parotid, submandibular…)
  4. •Lacrimal gland enlargement
  5. •Nonspecific cough, recurrent rhinitis/sinusitis
  6. •Vaginal dryness
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q
Extra-glandular Symptoms (usually from other conditions)
•Skin: 3
•Musculoskeletal: 2
•Thyroid: 2
•Pulmonary: 1
•Cardiac: 1
•GI: 5
•Liver: 2
•GU: 4
•Neuro: 3
•Lymphatic: 2
•General: 3
A

Extra-glandular Symptoms (usually from other conditions)

  • Skin: Raynaud’s, vasculitis, photosensitivity
  • Musculoskeletal: arthralgias (usually RA picture), myalgias
  • Thyroid: Hashimoto’s, hypothyroidism
  • Pulmonary: interstitial lung disease
  • Cardiac: pericarditis
  • GI: dysphagia, dyspepsia, epigastric pain, nausea, gastritis
  • Liver: primary biliary cirrhosis, chronic active hepatitis.
  • GU: interstitial nephritis, dysuria, nocturia, urgency.
  • Neuro: Peripheral neuropathy, ataxia, CN neuropathy
  • Lymphatic: non-hodgkins lymphoma, B-cell lymphoma
  • General: Fatigue, depression, anxiety
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Diagnostic Testing:
Serologic testing:

  • Antinuclear antibodies - ?
  • Anti-SSA/Ro and anti-SSB/La- ?
  • Rheumatoid factor – often ?
A
Diagnostic Testing: 
Serologic testing:
•Antinuclear antibodies - elevated
•Anti-SSA/Ro and anti-SSB/La- elevated
•Rheumatoid factor – often elevated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Follow-Up Testing:
All patients with Sjögren’s should be tested for additional autoimmunity

  • Other antibodies – 6
  • CBC – 3
  • Chem panel – 2
  • inflamation labs 2
  • Urinalysis – 2
A

Follow-Up Testing:
All patients with Sjögren’s should be tested for additional autoimmunity
•Other antibodies – anti-dsDNA, anti-centromere, anti-topoisomerase, anti-TPO, anti-TG, anti-CCP
•CBC – leukopenia, thrombocytopenia, anemia
•Chem panel – creatinine, liver enzyme tests
•ESR/CRP
•Urinalysis – proteinuria/hematuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
Dietary Modifications:
•Modifications for dysbiosis: 2
•Anti-inflammatory diet – 4
•Elimination of ?, specially ?
•Short-term ?
A

Dietary Modifications:
•Modifications for dysbiosis: low carbohydrate, low sugar
•Anti-inflammatory diet – reduction in red meat, dairy, sugar, carbohydrates.
•Elimination of food allergens specially Gluten
•Short-term fasting

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Hormones: elevated or decreased

A. Prolactin – ? in Sjögren's
B. Estrogen (Estradiol) – ? in Sjögren's
C. Cortisol – usually ?
D. Testosterone – usually ?
E. DHEA – usually ?
F. Thyroid hormone – usually ?
A

Hormones:

A. Prolactin – elevated in Sjögren’s

  1. •Check for hypothyroidism which may influence hyperprolactinemia
  2. •Vitex astus-cagnus – 200mg BID12 in dry herb equivalent (DHE)
  3. •Bromocriptine – 2.5mg qd - standard medication to reduce prolactin

B. Estrogen (Estradiol) – elevated in Sjögren’s

  1. •Reduce alcohol consumption
  2. •Reduce obesity
  3. •Indole-3-carbinol – 325mg per day13

C. Cortisol – usually decreased

  1. •Cortef – 20mg in am, 5mg late morning, 5mg in mid-afternoon
  2. •Pregnenolone – 10-15mg in the morning

D. Testosterone – usually decreased

  1. DHEA – 200mg/day16
  2. Transdermal testosterone (Androgel) – 5-10mg/day (women); 50 – 100mg/day (men)

E. DHEA – usually decreased

  1. •Anti-inflammatory, immunomodulatory and commonly deficiency
  2. •DHEA – 200mg/day

F. Thyroid hormone – usually decreased

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Viral Infections: 4

A

Viral Infections:
•EBV, CMV, HIV, HTLV-1 associated with Sjögren’s

  • Andrographis (Chuan Xin Lian) – SE with 30mg of andrographolides TID x 2-3 weeks
  • Monolaurin – Antiviral properties against CMV – 600mg TID
  • Reishi –(Ganoderma lucidum) – 2-6g of raw reishi or equivalent of concentrated extract.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Dysbiosis:
•Oral Candidiasis present in over ? of pts w/Sjögren’s
•Responses to bacterial DNA can trigger ? to human DNA

•Antimicrobial agents: 4

A

Dysbiosis:
•Oral Candidiasis present in over 1/3 of patients with Sjögren’s
•Responses to bacterial DNA can trigger cross-reactivity to human DNA

•Antimicrobial agents:

  1. •Berberine – 500mg BID for 3 – 6 months
  2. •Oregano Oil – emulsified and time released – 300mg BID
  3. •Artemisia annua – 100mg BID
  4. •Probiotics
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Vitamin D:
•Vitamin D status is ? associated w/inflammation in pts w/Sjogren’s s/d.
•Vitamin D supports normal ?, is ? and ?.
•There is not a lot of evidence that supplementation with Vitamin D promotes ? in flares or ? remission.

A

Vitamin D:
•Vitamin D status is inversely associated with inflammation in patients with Sjogren’s syndrome.29
•Vitamin D supports normal immune function, is anti-inflammatory and analgesic
•There is not a lot of evidence that supplementation with Vitamin D promotes reduction in flares or improves remission.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Additional Treatments: 3

A

Additional Treatments:

  1. •Essential Fatty Acids – 3 – 6g EPA+DHA per day
    •Well proven anti-inflammatory benefits in Sjögren’s
  2. •Green tea extract – 240-320mg of polyphenols30
    •Inhibits the expression of autoantigens in vitro
  3. •High Dose short-term oral vitamin A – 100,000IU per day for 2 weeks
    •Improved NK cell activity in those with Sjogren’s31
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Local Treatments:

  1. •Sip fluids with ? throughout the day
  2. •Chew ? –
  3. •? to help with salivary flow
  4. •If mouth sores present from dryness: 4?
A

Local Treatments:

  1. •Sip fluids with lemon throughout the day
  2. •Chew sugarless gum – promote saliva flow
  3. •Acupuncture to help with salivary flow
  4. •If mouth sores present from dryness: liquid folic acid, topical vitamin E and glutamine or chewable deglycyrrhizinated licorice.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly