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MSK II Exam 2 > Sjogren's Syndrome > Flashcards

Sjogren's Syndrome Flashcards

1
Q

Epidemiology

A 
Affects women > men (9:1)
Onset usually age 40-60
Affects ~1% of the population
Up to 30% of autoimmune rheumatic disease patients also have Sjogrens syndrome.
Symptoms may develop over 8-10 yrs.
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2
Q

Presentation

A

Dry eyes
- Gritty, sandy, worsens through day

Dry mouth

  • “Food sticking to roof of mouth”
  • Altered sense of taste
  • Difficulty wearing dentures
  • Needing liquids to swallow food
  • Dental caries

Parotitis

Dry mucosal surfaces (skin, bronchi, or vagina)

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3
Q

Sjogren’s syndrome is an autoimmune disorder with lymphocytic infiltration of the:

A – Endocrine glands
B – Exocrine glands
C – Adrenal glands
D – Hyaline membranes

A

B- exocrine glands

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4
Q

Etiology

A

Sjogren’s syndrome is an autoimmune disorder of the exocrine glands.

Most common presenting symptoms are dry eyes and dry mouth (sicca syndrome).

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5
Q

The inflammatory infiltrates in Sjogrens syndrome are characterized by:

A – NK cells
B – CD4+ T cells
C – Macrophages
D – Eosinophils

A

B - CD4+ T cells

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6
Q

Pathophysiology

A

Lymphocytic infiltration of exocrine glands

Helper T cells infiltrate gland.
B and T cells are activated.
Leads to autoantibodies, IL-2 release.
Glandular cells express MHC class II molecules & may act as antigen-presenting cell → cell apoptosis.
Metalloproteinases are secreted → block interaction of glandular cells & extracellular matrix, inhibiting gland function.

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7
Q

What’s going on here?

A

B cell hyperreactivity
Acetylcholine (muscarinic) receptor Ab
May interfere with neural stimulation of glandular secretion.

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8
Q

Pathogenesis

A

87% of patients with Sjogrens syndrome are positive for HLA-DR52.

Possible environmental triggers (viral?) in addition to genetic disposition.

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9
Q

Glands that may be Affected

A 
Lacrimal
Salivary
Parotid
Vulvovaginal
Prostate/urethral
Pancreas
Sweat glands
Glands in GI tract
Bronchopulmonary glands
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10
Q

Which of the following is most commonly associated with Sjogrens syndrome?

A – Anti-Ro/SSA, Anti-La/SSB
B – Antimitochondrial
C – Antihistone
D – Anti-Smith

A

A – Anti-Ro/SSA, Anti-La/SSB

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11
Q

Diagnosis

A

At least 4 of 6; must include + biopsy or Ab
Dry eyes x 3 mos., tear substitutes >3x/day
Dry mouth, swollen salivary glands, needing liquids to swallow
Eye signs: Schirmer test or + vital dye staining
Oral signs: Abnormal scintigraphy or sialography findings
+ Biopsy of minor salivary gland
+ Anti-Ro/SSA or anti-La/SSB

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12
Q

Diagnostic Tests

A

Schirmer test
(at least 10 mm moisture in 5 min.)

Sialogram of salivary glands

Lissamine green dye staining

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13
Q

Eye Irritation

A

↓ liquid in tears
Tear hyperosmolarity
→ Inflammatory cascade & immune activation of the conjunctiva

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14
Q

Additional Lab Findings

A 
↑ ESR
Anemia
Leukopenia
Eosinophilia
Hypergammaglobulinemia
\+ RF
\+ ANA
Antiphopholipid Ab
Cryoglobulins
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15
Q

Which of the following would mimic a diagnosis of Sjogrens syndrome?

A – Recent steroid use
B – Rheumatoid arthritis
C – History of head and neck irradiation
D – History of pancreatitis

A

History of head and neck irradiation

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16
Q

Diagnostic Exclusions

A 
Difficult to diagnose Sjogrens syndrome if:
Past head and neck irradiation
Hepatitis C infection
AIDS
Prior lymphoma
Sarcoidosis
Graft vs. host disease
Taking anticholinergic drugs
IgG4-related disease
17
Q

Mucous Membrane Involvement

A

Atrophic changes in upper respiratory tract
→ Nasal dryness, recurrent infection, hoarseness
Atrophic changes in vulvovaginal area
→ Pruritus, vaginitis
Dryness of anorectal area
→ Pruritus, inflammation

18
Q

GI Involvement

A 
Dysphagia due to dry pharynx & esophagus
Acute or chronic pancreatitis
Malabsorption
Digestive symptoms due to achlorhydria
Delayed gastric emptying
19
Q

Extraglandular Involvement

A

Periepithelial Infiltration

  • Interstitial nephritis/RTA
  • Liver disease
  • Interstitial lung disease

Extraepithelial Extraglandular

  • B-cell hyperreactivity
  • Hypergammaglobulinemia
  • Immune complex formation
  • —- Glomerulonephritis, purpura, peripheral neuropathy
20
Q

Lung Involvement

A

Airway hyperreactivity

  • Cough
  • Bronchiolitis
  • Obstructive pattern on spirometry

Interstitial lung disease

  • Several types of interstitial pneumonitis
  • Restrictive pattern on spirometry
21
Q

Kidney Involvement

A 
Tubulointerstitial nephritis
- Mildly increased serum creatinine
- Distal (type 1) renal tubular acidosis
↓ potassium, urine pH > 5.5, urine anion gap
- Hypokalemia
- Fanconi syndrome/proximal (type 2) RTA
- Nephrogenic diabetes insipidus

Renal calculi

Interstitial cystitis

22
Q

Liver Involvement

A

Autoimmune liver disease

Primary biliary cirrhosis

  • Immune-mediated destruction of small bile ducts
  • Cholestatic pattern on LFT’s
    • Antimitochondrial Ab
    • Antibody to carbonic anhydrase 11

Autoimmune hepatitis

  • Hepatocellular pattern on LFT’s
    • Anti-smooth muscle Ab or + ANA
23
Q

Neuro Involvement

A 
Peripheral neuropathy
- Including mononeuritis multiplex
Myelitis
Cognitive dysfunction
Cranial neuropathies
Neuromyelitis optica (can mimic MS)
24
Q

Cardiac Involvement

A

Pericarditis
Pulmonary hypertension
Autonomic dysfunction
- Associated with M3 muscarinic receptor Ab

25
Q

Skin Involvement

A 
Dryness, pruritus
↓ Sweating
Erythema annulare (Asian pts.)
Cutaneous vasculitis (purpura)
Raynaud phenomenon
Dry hair, alopecia
26
Q

Other Symptoms

A

Fatigue

Arthritis occurs in 42%.
- Nonerosive, mild

27
Q

Tx of Sicca Symptoms

A

Hydration

Symptomatic treatment

Ocular

  • Artificial tears
  • Punctal plugs
  • Topical cyclosporine

Oral

  • Artificial saliva
  • Sugar-free lozenges
  • Pilocarpine, cevimeline
28
Q

Sjogrens Treatment

A

Arthritis

  • NSAID’s
  • Hydroxychloroquine
  • Methotrexate
  • Short-term, low-dose corticosteroids

Fatigue
- Exercise

29
Q

Biological Therapies

A

TNF-α inhibitors are not used to treat sicca symptoms!

For systemic symptoms – begin with standard DMARDs or high-dose corticosteroids.

If no improvement, consider rituximab for:

  • Cryoglobulinemia/vasculitis
  • Renal disease
  • Inflammatory arthritis
  • Pulmonary disease
  • Peripheral neuropathy, especially mononeuritis
30
Q 
Patients with Sjogrens syndrome have a highly increased risk of developing:
A – Non-Hodgkin lymphoma
B – Follicular thyroid cancer
C – Renal cell carcinoma
D – Colon cancer
E – Coronary artery disease
A

Non-Hodgkin lymphoma

31
Q

Complications

A

Lymphoma occurs in 4.3% of Sjogrens patients (increased 18x above normal).
Most commonly MALT lymphoma or other B-cell lymphomas.

32
Q

Anti-Ro/SSA is associated with which complication in babies born to women with Sjogrens syndrome?

A – Neonatal thyroiditis
B – Neonatal hypoglycemia
C – Developmental dysplasia of the hip
D – Neonatal heart block

A

Neonatal heart block

33
Q

Other Complications

A

Parotid infections
- Staphylococcal, streptococcal, pneumococcal
Parotid tumors
Antiphopholipid Ab syndrome

Neonatal lupus with congenital heart block

34
Q

Prognosis

A

Symptoms do not tend to progressively worsen.
Mortality is similar to the general population.
Poor prognostic indicators at diagnosis:
- ↓ Complement levels
- Lymphocytopenia
- Cryoglobulinemia
…predictive of lymphoma, more severe disease, and increased mortality.

MSK II Exam 2 (16 decks)

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