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Rheumatology > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

Sjogren’s Syndrome (SS)

  1. Definition?
  2. Other symptoms? 3
  3. What is the primary form?
  4. What is the secondary form?
A

Definition
1. Chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function

  1. Dry eye, dry mouth, vaginitis
  2. What is the Primary form?
    Not associated with other diseases
  3. What is the Secondary form?
    Tends to complicate other rheumatic conditions (most common disease associated with secondary SS is RA)
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2
Q

What is Sicca complex?

A

dry eyes and dry mouth

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3
Q

Sjogren’s Syndrome

  1. Occurs in which age groups?
  2. Peak in what decade?
  3. Gender?
A
  1. Occurs in all age groups
  2. Peaks in 4th and 5th decades
  3. 9:1 female to male ratio
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4
Q

Sjogren’s Syndrome (SS)
1. Pathogenesis?

  1. Thought to share common pathogenetic features with what?
A
  1. Considered “lymphocytic infiltrative” rather than antibody mediated
    - Lymphocytic infiltration into lacrimal and salivary glands, where lymphocytes are not normally found, impairs function
  2. other autoimmune diseases such as SLE
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5
Q

Describe the milder type of SS? 5

Describe the more severe type of SS?
7

A

Mild end?
Moderate symptoms of
1. dry eyes,
2. dry mouth, and
3. maybe vague symptoms of fatigue, myalgias, and
4. cognitive dysfunction.
5. These patients tend to have a low titer of ANA

Severe end?

  1. Florid salivary gland enlargement,
  2. adenopathy,
  3. antibodies to Ro/SSA and La/SSB antigens,
  4. cryoglobulinemia,
  5. hypocomplementemia,
  6. a propensity to develop non-Hodgkin’s lymphoma, and other
  7. extraglandular disease manifestations
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6
Q

SS
1. What occurred IN ALL PATIENTS and were the only disease manifestation in 31 percent? 2

  1. What involvement occurred in 25 percent?
  2. What developed in 2.5 percent?
  3. Which glad will be most affected and also the target for biopsy of lymphocytes?
A
  1. Keratoconjunctivitis sicca and/or xerostomia
  2. Extraglandular
  3. Non-Hodgkin’s lymphoma
  4. Parotid
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7
Q

SS Extraglandular Involvement

4

A
  1. Vaginal dryness in women—dyspareunia
  2. Interstitial nephritis
  3. Involvement of the exocrine glands of the upper airways—can have recurrent rhinitis and sinusitis
  4. Bronchial dryness—lead to dry cough—-mistakenly treated for asthma or bronchitis
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8
Q

Sjogren’s Syndrome (SS)
Clinical Manifestations
1. Ocular symptoms?

  1. You may also hear the term “dysfunctional tear syndrome” to refer to the subtype of dry eye in SS. How does this present?
  2. Ask the following for ocular symtpoms? 3
A
  1. “keratoconjunctivitis sicca [KCS]”
  2. Deficiency in tear production leading to symptoms of dry eyes which usually presents insidiously over several years
    • “Do you often feel a gritty or sandy sensation in your eyes?”
    • “Have you had symptoms of dry eyes on a daily basis for more than three months?”
    • “How often do you use eye drops?”
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9
Q

SS: Complications of KCS include? 2

A
  1. Corneal ulceration

2. Infection of the eyelids

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10
Q

SS: Clinical Manifestations: Oral symptoms

Patient complains of mouth dryness
Ask the following? 2

A
  1. “Do you have to wake up at night to drink water because your mouth is so dry?”
  2. “Do you frequently drink liquids to help in swallowing dry foods?”
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11
Q

SS: Complications of xerostomia include:

8

A
  1. Dental caries**
  2. Oral candidiasis**
  3. Gingival recession
  4. Other types of oral infections, such as bacterial infections of Stensen’s duct
  5. Laryngotracheal reflux
  6. Chronic esophagitis
  7. Weight loss
  8. Nocturia
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12
Q

SS: What do the following complications lead to:

  1. Laryngotracheal reflux? 4
  2. Chronic esophagitis? 2
  3. Weight loss? 1
  4. Nocturia results why?
A
  1. which may lead to
    - frequent throat clearing,
    - cough,
    - substernal pain and
    - nocturnal awakening that simulates panic attacks
  2. due to
    - impaired clearance of acid and
    - lack of the buffering effects of saliva
  3. due to difficulty with chewing and process of swallowing.
  4. results from attempts on the part of the patients to counter dry mouth by drinking copious volumes of fluid
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13
Q

Laryngotracheal reflux in SS is the result of what? 3

A
  1. decreased salivary flow,
  2. absence of the normal gastric acid buffer, and
  3. reflux of gastric acid into the esophagus and trachea.
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14
Q

Sjogren’s Syndrome (SS)
Salivary Gland Enlargement (occurs in 30-50% of patients with SS at some point in the disease)
1. Glands look how?
2. Can occur in what glands? 2

A
  1. Glands are typically firm, diffuse, and nontender

2. Can occur in both parotid and submandibular glands

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15
Q

SS: Criteria for Dx?
6

(3 out of 4 = sensitivity of 80%)

A
  1. Ocular symptoms of inadequate tear production
  2. Ocular signs of corneal damage
  3. Oral symptoms of decreased saliva production
  4. Salivary gland histopathology demonstrating foci of lymphocytes
  5. Tests indicating impaired salivary gland function
  6. Presence of autoantibodies (anti-Ro/SSA and/or anti-La/SSB)
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16
Q

Exclusion Criteria for SS?

7

A
  1. Prior head & neck irradiation
  2. Infection w/ hep C
  3. AIDS
  4. Lymphoma
  5. Sarcoidosis
  6. Graft-versus-host disease
  7. Recent use of anti-cholinergic meds
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17
Q

What dx a pt as having primary SS?

6

A
  1. Patients have no associated CTD and
  2. no exclusionary diagnoses cited
  3. Patient has either a positive salivary gland biopsy or
  4. anti-Ro/SSA and/or
  5. anti-La/SSB and
  6. satisfies a total of four of the six items

The patient satisfies three of the four objective items (ocular signs, biopsy, salivary gland involvement, or autoantibodies)

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18
Q

A classification of secondary SS is made if a CTD is present and both of the following criteria are met. What are they?
4

A
  1. At least one item indicative of ocular or oral dryness is present
    Any two of the following three objective items are present:
  2. Ocular signs
  3. Postive salivary gland biopsy
  4. Abnormal tests of salivary gland function
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19
Q

Diagnosis of SS

  1. Mainly through?
  2. The following tests are recommended for use in clinical practice when SS is suspected? 3
A
  1. Med Hx and PE
    • Schirmer test
    • Rose Bengal test
    • Basic Lab Testing
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20
Q

Labs for SS? 4

A
  1. CBC w/ diff
  2. CMP
  3. Sed Rate
  4. UA (protein and sugar from leaky membrane)
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21
Q

Diagnosis of SS Specialized Laboratory Testing

11

A

REFER TO RHEUM

  1. Antinuclear antibodies
  2. RF
  3. Antibodies to the Ro/SSA, La/SSB, Sm, and RNP antigens
  4. Antibody to anti-centromere B or topoisomerase, in patients with nucleolar pattern of ANA and symptoms of overlap with systemic sclerosis
  5. ANCA, in patients with evidence of vasculitis
  6. Serum C3 and C4
  7. Antibodies to double-stranded DNA, in patients with proteinuria
  8. Serum cryoglobulins
  9. SPE
  10. Quantitative immunoglobulin levels (IgG, IgM, and IgA)
  11. Salivary gland Biopsy
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22
Q

Sjogren’s Syndrome (SS)
1. WHat antibodies are found in approximately 70 to 97 percent of patients with primary Sjögren’s syndrome?

  1. High titers of these antibodies are associated with a greater incidence of what?
  2. What antibodies are present in only 10 to 15 percent of patients with secondary Sjögren’s syndrome associated with rheumatoid arthritis?
  3. It is unusual to encounter sera that contain ________ activity without demonstrable antibodies to Ro/SSA in patients with SLE or Sjögren’s syndrome.
A
  1. Anti-Ro/SSA
    • extraglandular features,
    • especially purpura,
    • cutaneous vasculitis, and a
    • demyelinating syndrome resembling multiple sclerosis
  3. Anti-Ro/SSA
  4. anti-La/SSB
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23
Q

SS: Treatment

Therapy can be grouped: 2

A

a) topical therapy of dry eyes and dry mouth (i.e. therapy for “sicca symptoms”)
b) treatment of systemic manifestations of SS and recognition of therapies for other conditions that can exacerbate dryness complaints

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24
Q

SS: Treatment for Sicca Symptoms
General principles?
7

A
  1. Avoidance of irritants
  2. Avoidance of exacerbating drugs
  3. Avoidance of low humidity atmospheres
  4. A dry mouth is NOT a painful mouth (consider candidiasis)
  5. Irritated eyes despite sufficient hydration? (consider blepharitis)
  6. Excellent oral hygiene including brushing teeth, flossing, and dental prophylaxis is REALLY important
  7. Topical moisturizers and lozenges are great
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25
Q

Treatment for Sicca Symptoms
Xerostomia
In general how do we do that? 3

A
  1. Stimulation of existing salivary flow
  2. Muscarinic agonists (e.g.cevimeline—SE?)
  3. Artificial saliva
26
Q

SS: How can we stimulate exsiting salivary flow? 4

A
  1. Sugarless candies
  2. Dried fruit slices such as peaches or nectarines
  3. Citrus flavored sugarless tablets (e.g., “Salivasure”)
  4. Sugar-free chewing gums, containing various sweeteners such as aspartame, saccharin, and sorbitol can also be helpful.

Educate patient the too much sorbitol can cause diarrhea

27
Q

Treatment for Sicca Symptoms
Keratoconjunctivitis (KCS)
1. In general how should we treat?
2. How cna we accomplish this? 4

A
  1. Replacing or supplementing deficient tears
    • Replacement of the aqueous layer with hypromellose 0.3% or methylcellulose 0.3% (beneficial when the watery component is deficient, as is usual in SS)
  • Spreading agents (polyvinyl alcohol, polyethylene glycol, dextran [help the aqueous layer spread over the hydrophobic corneal and conjunctival epithelium])
  • Longer acting lubricant ointment at night
  • Topical Cyclosporine (Restasis)
28
Q

Treatment Systemic Sjogren’s

2

A

Immunosuppressive:

  1. Hydroxychloroquine
  2. Methotrexate
29
Q

In general how can scleroderma present?

A

Scleroderma may be a clinical feature of limited anatomic extent affecting only the skin and adjacent tissues OR it may be associated with systemic involvement.

30
Q
  1. When the characteristic skin disorder is associated with internal organ involvement, the disease is termed what?
  2. Disease with vascular, fibrotic, and immunologic manifestations and involves several systems including what? 5
A
  1. Systemic Sclerosis (SSc)
    • skin,
    • GI tract,
    • lungs,
    • heart, and
    • kidneys
31
Q

Systemic Sclerosis: Peak onset when?

A

30-50

32
Q

Scleroderma: Combination of what? 3

A
  1. Vascular damage
  2. Immune activation
  3. Excessive synthesis of extracellular matrix w/ deposition of structurally normal collagen results
33
Q
  1. Normal Endothelin cells do what?
  2. Sera from pts w/ SSc has been found to be what?
  3. How does immune activation contribute to the pathology of this? 2
A
  1. Endothelins cause vasoconstriction & are fibrinogenic
  2. cytotoxic to endothelial cells
  3. activation of endothelial cells results in
    - up regulation of adhesion molecules, &
    - leukocyte migration out of the vasculature
34
Q

Diagnosis
The diagnosis of systemic sclerosis and related disorders is based primarily upon the presence of characteristic clinical findings
-The combination of skin induration plus one or more of the following clinical features supports the diagnosis of systemic sclerosis? 7

A
  1. Heartburn and/or dysphagia of new onset
  2. Acute onset of hypertension and renal insufficiency
  3. Dyspnea on exertion associated with interstitial pulmonary changes on radiography
  4. Pulmonary hypertension
  5. Diarrhea with malabsorption
  6. Characteristic facial, lip, or hand telangiectasia
  7. Digital infarctions and or digital tip pitting
35
Q

***There is no treatment that comprehensively addresses Systemic Sclerosis… so treatment is directed at specific organ manifestations?
8

A
  1. Cutaneous manifestations
  2. Raynaud’s phenomenon
  3. Musculoskeletal manifestations
  4. GI manifestations
  5. Pulmonary manifestations
  6. Cardiac manifestations
  7. Renal manifestations
  8. Erectile dysfunction & dyspaurenia
36
Q
  1. Scelroderma: Skin involvement is characterized by variable?
  2. What are the earliest body parts involved? 3
  3. What may precede the skin induration?
A
  1. thickening and hardening of the skin
  2. The fingers, hands, and face are generally the earliest areas of the body involved.
  3. Edematous swelling and erythema of the skin may precede skin induration.
37
Q

Scleroderma: Other prominent skin manifestations include?

5

A
  1. ***Pruritus
  2. Edema
  3. Digital ulcers
  4. Pitting at the fingertips
  5. Telangiectasia
38
Q

Scleroderma: ***There is no treatment proven to reverse the skin thickening seen in scleroderma.
1. What can help with pruritus? 2. Options for other symptoms? 3

A
    • Emollient creams should be used liberally to soften the skin and decrease pruritus
    • Antihistamines and
    • doxepin (Sinequan) are also helpful at alleviating pruritus
  2. -UVA therapy shows some promise
    -Other options include highly potent topical steroids and methotrexate
    -The combination of high-dose systemic glucocorticoids and
    low dose methotrexate has also been used
39
Q
  1. ***Most common initial manifestation of scleroderma (generally precedes other manifestations, sometimes by years or even decades!)
  2. Also associated with what?
  3. What is it?
  4. Manifested clinically by what?
A
  1. Raynaud’s Phenomenon
  2. SLE
  3. An exaggerated vascular response to cold temperature or emotional stress.
  4. Manifested clinically by sharply demarcated color changes of the skin of the digits due to abnormal vasoconstriction of digital arteries and cutaneous arterioles
40
Q

When is Raynaud’s considered primary and when is it considered secondary?

A
  1. RP is considered primary if these symptoms occur alone without evidence of any associated disorder.
  2. Secondary RP refers to the presence of the disorder in association with a related illness, such as scleroderma.
41
Q

RP: A typical episode is characterized by?

6

A
  1. Sudden onset of cold fingers (or toes) in
  2. association with sharply demarcated color changes of skin pallor (white attack) and/or
  3. cyanotic skin (blue attack)
  4. With rewarming, the ischemic phase (white or blue attack) usually lasts for 15 to 20 minutes.
  5. The skin subsequently blushes upon recovery, thereby
  6. resulting in the erythema of reperfusion.
42
Q

Describe the 3 step process of RP?

A
  1. Cold Temperature or
    Emotional Stress

leads to

  1. White Attack (Pallor)
    OR
    Blue Attack (Cyanosis)
    (lasts 15-10 min)

leads to

  1. Erythema of Reperfusion
43
Q

Raynaud’s Phenomenon
1. Primary Raynaud phenomenon, which is commonly seen in the general population, occurs in otherwise healthy individuals, and almost always has what kind of prognosis?

  1. In patients with Systemic Sclerosis, Raynaud phenomenon (which would be termed secondary) can lead to? 3
A
  1. a benign course.

2.

  • Ulcerations
  • Digital Infarctions
  • Gangrene
44
Q

Raynaud’s Phenomenon
Treatment?
6

A
  1. Prevention (bundling up well against cold temps)
  2. Calcium channel blockers (meds such as nifedipine or amlodipine)- NO BB
  3. Alpha blockers may be given along with or instead of calcium channel blockers
  4. Low dosed ASA should be used
  5. Erectile dysfunction pills?
  6. Treat any associated complications
    - Debridement of ulcers
    - ABX for secondary infections
45
Q
  1. Musculoskeletal Manifestations
    of Systemic Sclerosis?
  2. Generally treated with?
A
  1. Erosive, inflammatory arthritis similar to rheumatoid arthritis can occur
  2. Generally treated with drugs such as methotrexate
46
Q

GI Manifestations of Systemic Sclerosis

  1. Which tissue areas?
  2. Most frequent clinical GI manifestation is?
  3. Treatment? 3
A
  1. GI tract involved in all smooth muscle areas
  2. Most frequent clinical manifestation is GERD
  3. Treatment
    - Preventive measures
    - Proton Pump Inhibitors
    - Promotility agent
47
Q

Pulmonary Manifestations of Systemic Sclerosis

  1. What manifestation occurss commonly?
  2. CT scan may show?
  3. Treatment?
  4. Pts may also suffer from?
  5. Increased risk of what?
  6. What are all these medications working on?
A
  1. Lung fibrosis occurs commonly
  2. CT scan may show a ground glass pattern
  3. Treatment
    - Cyclophosphamide
  4. Patients may also suffer from Pulmonary HTN
  5. Increased risk of lung cancer (5 fold)
  6. Endothelins
48
Q

Cardiac Manifestations of Systemic Sclerosis
1. Common manifestations? 2

  1. Treatment is with what? 2
A
  1. Patients can have
    - conduction abnormalities or
    - dysrhythmias
  2. appropriate
    - antiarrhythmics and
    - cardiac pacing, if needed
49
Q

Renal Manifestations of Systemic Sclerosis
Patients may have an acute renal crisis seen early in the disease (typically has been associated with use of moderate- to high-dose steroid therapy)

  1. Treatment?
  2. What should be avoided?
A
  1. ACE inhibition

2. Diuretics should be avoided!!!

50
Q

What is CREST syndrome?

A
  1. Calcinosis
  2. Raynaud’s phenomenon
  3. Esophageal dysmotility
  4. Sclerodactyly
  5. Telangiectasia
51
Q

PP of CREST:
1. Immune system produces what? 2

  1. Often used to refer to what?
  2. Patients often go on to develop what? 2
A
    • anti-centromere and
    • anti-nuclear antibodies
  2. to the limited cutaneous form of scleroderma
    • pulmonary hypertension and
    • CHF
52
Q

Polymyositis/Dermatomyositis

  1. Definition?
  2. With?
  3. Without?
A
  1. Inflammatory muscle disease that can occur with or without a cutaneous eruption
  2. With? ➔ termed Dermatomyositis
  3. Without? ➔ termed Polymyositis
53
Q
  1. Inflammatory damage of myocytes leads to what?

2. Three rashes are characteristic. What are they?

A
  1. proximal muscle weakness
    • Rash over PIP and MCP joints (Gottron’s sign)
  • Purple (heliotrope) eruption over the eyelids
  • Erythematous poikiloderma over upper anterior chest and around over the shoulders [Shawl Sign]
54
Q

What is Erythematous poikiloderma?

5

A
  1. pigmentation,
  2. telangiectasia,
  3. purpura,
  4. pruritus
  5. atrophy
55
Q

Polymyositis/Dermatomyositis

  1. What is the most common presenting feature?
  2. Describe its progression?
  3. Describe the distribution of weakness?
  4. Myalgias and muscle tenderness occur in 25 to 50 percent of cases. These symptoms tend to be what?
A
  1. Proximal muscle weakness is the most common presenting feature
  2. Onset is usually insidious, with gradual worsening over a period of several months before medical attention is sought
  3. Distribution of weakness is typically symmetric and proximal.
  4. mild, unlike the more prominent muscle pain that occurs in polymyalgia rheumatica and fibromyalgia.
56
Q

What is generally not seen in early cases, even in patients with marked weakness, but may occur in severe, long-standing disease?

A

Muscle atrophy

57
Q

What are the three signs for dermatomyositis?

A
  1. Gottron’s sign (over knuckles)
  2. Heliotrope eruption (around eyes)
  3. Shawl sign (around shoulders)
58
Q

Polymyositis/Dermatomyositis
Diagnosis is suggested clinically (especially with cutaneous manifestations)
Make sure to acquire excellent medication history

  1. Labs? 5
  2. What else may be helpful?
A
  1. Muscle enzyme eval:
    -CK,
    -LDH,
    -AST,
    -ALT
    -ANA elevated in up to 80% (specific autoantibodies)
    Almost all patients will have elevation of at least one and many will have elevations of all
  2. Muscle biopsy
59
Q 
Pathogenesis
DM:
1. Mediated how?
2. Cellular infiltrate focused where?
3. What would suggest microinfarction?

PM:

  1. Cellular infiltrates with what?
  2. Abnormal muscle cells scattered throughout the what?
A

DM:

  1. Humorally-mediated (antibodies- more complement driven)
  2. Cellular infiltrate focused around blood vessels
  3. Abnormal muscle fibers grouped in one portion of the fascicle suggesting microinfarction

PM:

  1. Cellular infiltrate within the fascicle
  2. Abnormal muscle cells scattered throughout the fascicle
60
Q

Polymyositis/Dermatomyositis
Treatment:
1. Cutaneous manifestations? 2

  1. Muscular manifestations? 3
A
  1. Cutaneous manifestations
    - Mild to Moderate topical steroid therapies
    - May require hydroxychloroquine or methotrexate
  2. Muscular manifestations
    - Initial treatment with high-dose systemic steroids (usually tapering will begin in a month)
  • Physical therapy program
  • Sometimes other immunosuppressant meds started including Methotrexate (high dose steriods first!!) and azathioprine
61
Q
  1. There is a wide spectrum of presentation for patients with Sjogren’s Syndrome… however, ***ALL patients with SS will have what? 2
  2. If it’s characteristic of Sjogren’s and you have a positive what, you have your diagnosis?
  3. If a patient has muscle weakness and myalgias and something’s not making sense, order what?
A
  1. KCS and xerostomia
  2. anti Ro-SSA
  3. muscle enzymes

Rheumatology (11 decks)

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