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Derm Rheum > Scleroderma/MCTD/Sjogren - Postlethwaite/Gupta > Flashcards

Scleroderma/MCTD/Sjogren - Postlethwaite/Gupta Flashcards

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Dermatology Board Prep flashcards
1
Q

Another name for systemic sclerosis?

A

Scleroderma…were off to a good start here

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2
Q

Pathophysiologic quartet of Systemic Sclerosis?

A
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3
Q

Autoimmunity and vasculopathy precede the onset and contribute to the progression of ____?

A

Fibrosis

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4
Q

Epidemiology of Scleroderma.

Classic presentation is in?

A

Middle aged females (30-50 years old)

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5
Q

Pathogenesis of Scleroderma. What are some important cells involved with scleroderma?

A

Fibroblasts, Endothelial cells, B cells, T cells, Monocytes

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6
Q

In Scleroderma, endothelial cell dysfunction causes what 3 features?

A

leads to:

  • inflammation (increased adhesion molecules)
  • Vasoconstriction (increased endothelin and decreased NO)
  • Secretion of growth factors (TGF-beta and PDGF)
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7
Q

Fibroblasts role in scleroderma?

A

Collagen deposition

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8
Q

Vasculature/microvasculature involvement in scleroderma?

A

Raynaud’s phenomenon in fingers/toes

Telangiectasis of the vasa vasorum

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9
Q

Types of Raynaud’s? Which one is associated with scleroderma

A

Type I and Type II

Type 1 = Primary Raynaud’s (no other disease)

Type II = occurs with another condition such as Scleroderma, SLE, RA, polymyositis, MCTD (mixed connective tissue disorder)

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10
Q

What are we looking at here?

What does (a) show?

What about (b)?

What is the significance of (b)?

A

These are nailfold capillary patterns under microscope

(a) is showing a normal nailfold patter; however, it could also be a patient with primary raynauds
(b) shows dilitation of nailfold capillaries and is associated with developing a CT disease (such as Systemic sclerosis)

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11
Q

In scleroderma patient, longstanding raynaud’s can progress to?

A

development of digital ulcers

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12
Q

2 main types of Systemic Sclerosis?

A

Limited and Difffuse

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13
Q

Skin/organ involvement in Limited SS?

A

Elbows to periphery. Knees to periphery, Head. with late visceral involvement

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14
Q

Skin/organ involvement in Difuse SS?

A

Skin involvemement is diffuse with early visceral involvement

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15
Q

Systemic Sclerosis famous mnemonic (hint not colgate)? Associated with what type (limited/diffuse)

A

CREST syndrome - Limited

  • Calcinosis/anti-Centromere antibodies
  • Raynaud’s phenomenon
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasis of the skin
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16
Q

Common organs involved in Diffuse Scleroderma?

A
  • Vessels (Raynaud phenomen)
  • GI tract (esophagel dysmotility and reflux)
  • Lungs (interstitial fibrosis and pulmonary HTN)
  • Kidneys (scleroderma renal crisis)
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17
Q

What is this? What disease is it associated with?

A

Gastric Antral Vascular Ectasia

= dilatated arteries that are prominent in stomach

“Watermelon appearance”

Diffuse Scleroderma

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18
Q

What 4 antibodies are associated with scleroderma?

A

Anti-Scl70 (topoisomerase 1), Anti centromere, Anti PM/Scl, Anti U1-RNP

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19
Q

Diffuse Scleroderma associated with what antibody?

A

Anti-Scl 70 (topoisomerase 1)

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20
Q

Antibodies associated with Limited Scleroderma?

A

Anti-centromere antibodies

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21
Q

What is a life threatening condition that occurs in roughly 5-10% of scleroderma patients?

A

Scleroderma renal crisis (SRC)

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22
Q

Scleroderma Renal Crisis risk factors?

A

Early diffuse skin disease

use of corticosteroids

anti-RNA polymerase III antibodies

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23
Q

What key pharmacologic intervention is crucial in SRC (scleroderma renal crisis)?

A

ACE-inhibitors. can cantrol and possibly reverse the disease. Dr. P says that even if patient goes into renal failure, still keep them on ACE-I, just put them on dialysis.

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24
Q

Raynaud’s + what GI prob = likely Scleroderma?

A

difficulty swallowing (dysphagia)

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25
Q

Mortality in Scleroderma is now most likely associated with?

A

Interstitial lung disease and PAH (60% of deaths in SSc). Renal crisis used to be a big mortality cause

26
Q

If scleroderma patient begins developing signs of right heart failure, what the balls is going on?

A

Patient has likely developed PAH (pulmonary arterial hypertension) = Not good good

27
Q

“En coup de sabre” deals with what type of scleroderma?

A

Localized scleroderma

28
Q

Localized scleroderma is:

Systemic or nonsystemic?

Primarily in children or adults?

How many major types?

Name these types

A

Nonsytemic, children, 5 major types

  1. Plaque morphea = MOST COMMON, isolated circular patch of thickened skin
  2. Generalized morphea - multiple lesions, estensive areas of skin
  3. Keloid morphea = nodular, like keloids
  4. Bullous morphea = Rare, subepithelial bullae
  5. Linear scleroderma = linear streak that crosses dermatomes and is associated with atrophy of muscle, underlying bone, and rarely the brain called “en coup de sabre”
29
Q

What is mixed connective tissue disease?

A

Autoimmune mediated tissue damage with mixed features of SLE, Systemic sclerosis, and polymyositis

30
Q

MCTD is most commonly associated with what antibody?

A

U1-RNP

31
Q

What is seen in nearly all patients with MCTD (mixed connective tissue disease)?

A

Raynaud’s. If they dont have raynauds, you should reconsider your diagnosis

32
Q

25 % of MCTD patients develop what?

A

Renal involvement - usually membranous glomerulonephritis. Proliferative glomerulonephritis is uncommon in MCTD

33
Q

Most common cause of death in MCTD?

A

Pulmonary HTN

34
Q

Rare finding in MCTD?

A

Serious CNS involvement. Pt may have trigeminal neuropathy and sensorineural hearing loss

35
Q

Common early finding in MCTD?

A

Inflammatory arthritic manifestations:

Fingers puffy, fusiform PIP swelling, Periungual infarcts

36
Q

Path and Scleroderma. Scleroderma biopsy will have what characteristic feature?

A

Morphea = tissue biopsy almost looks linear due to excessive deposition of collagen and deep fibrosis

37
Q

Path and Scleroderma. What features are different b/t early and late cutaneous systemic biopsys

A

In late forms, will see:

  • loss of dermal papillaes
  • hyperkeratosis (no nuclei present)
  • loss of hair follicles and glands (being pushed out by excess collagen and fibrosis)
38
Q

What are calcareous lesions? What disease are they associated with?

A

Scleroderma. Related to tumoral calcinosis - large painful masses in the periarucular soft tissue composed of calcium hydroxyapatite. better not be on the test

39
Q

Lupus nephritis vs Scleroderma renal crisis. Which glomerulus is which?

A

Top = Lupus nephritis. Glomeruli are enlarged (due to mesangial proliferation) and hypercellular

Bottom = SRC. Glomeruli are shrunken and lack inflammatory cells

40
Q

Path. What is structurally different b/t primary raynauds and secondary raynauds?

A

Primary raynauds (i.e. raynauds not attributed with any another disorder) = NO structural abnormalities.

Secondary raynauds (like due to Scleroderma) shows intimal thickening and medial hypertrophy

41
Q

Definition of Sjogren’s?

A

Autoimmune destruction of minor salivary glands and lacrimal glands

42
Q

List 3 clinical hallmarks of Sjogrens?

A
  • Keratoconjunctivitis sicca (dry eyes)
  • Xerostomia (dry mouth)
  • Parotid gland swelling
43
Q

List extraglandular manifestations of Sjogrens?

A
  • Fatigue
  • Raynaud’s
  • Polyarthralgia/arthritis
  • Interstitial lung disease
  • Neuropathy
  • Purpura
44
Q

Sjogren Epidemiology

Race? Age involvement? Age of onset?

90% of Sjogren’s patients are ?

Prevalence?

A
  • Affects all races and all ages, but onset is greatest in middle age
  • 90% are females
  • Prevalence may be 0.4-0.8%
45
Q

A patient with Sjogren’s has a 44x increased risk of developing? How to monitor?

A

Non-Hodgkin’s Lymphoma, check for any signs of lymphadenopathy or splenomegaly, symptoms of weight loss

46
Q

Sjogren’s and the kidney. What important disorder? What others?

A

Type 1 Renal Tubular Acidosis = Know this

Also: Interstitial nephritis, glomerulonephritis, and nephrogenic diabetic insipidus

47
Q

1/3 of Sjogren’s patients have what GI symptom?

A

Dysphagia (difficulty swallowing)

48
Q

If Sjogren patient has anti-aquaporin-4 antibody, what dx is this associated with?

A

Neuromyelitis optica

49
Q

Sjogren Syndrome antibodies to know? Which is most specific?

A

ANA (85%)

Anti-SSA/Ro (33%)

Anti-SSB/La (33%) = Most specific

50
Q

Sjogren’s. anti-SSA and anti-SSB are associated with what manifestations?

A

Extraglandular manifestations (e.g. neuropathies)

51
Q

75-95% of Sjogren’s patients will have what lab finding (not ANA, SSA, or SSB)?

A

+ Rheumatoid factor

52
Q

Common ocular manifestation seen in Sjogrens?

A

Keratoconjunctivitis sicca (keratitis caused by decreased lacrimal secretions)

53
Q

What test(s) are commonly done on eye of Sjogren’s patients?

A
  1. Exam of eye after instilling rose bengal dye to highlight epithelial lesions
  2. Schirmer’s test, measures how far tears fall down on paper hanging from eye
54
Q

Oral manifestations of Sjogren’s?

A
  • Xerostomia (severe mouth dryness)
  • Multiple dental carries
  • Unilateral parotid and/or submandibular salivary gland enlargement (50% of patients)
  • Dysphagia
  • Suck at the cracker challenge
55
Q

2 types of Sjogren’s? youve got this. i promise

A

Primary and Secondary

56
Q

Which type of Sjogren’s is considered a chronic autoimmune inflammatory disorder?

A

Primary

57
Q

Which type of Sjogren’s is associated with possible malignant transformation?

A

Primary

58
Q

Which type of Sjogren’s is a complication of another autoimmune connective tissue disease?

A

Secondary

59
Q

Which type of Sjogren’s is commonly seen in RA and SLE?

A

Secondary

60
Q

Unilateral parotid gland selling in Sjogren’s. Is this a good thing? How do you find out?

A

No, likely malignant. Need to get parotid gland biopsy

61
Q

Path and Sjogren’s. Microscopic findings that are seen with Sjogren’s?

A

Lots of lymphocytes, interstitial fibrosis, acinar atrophy of a minor salivary gland

62
Q

Sjogren patient biopsy shows polytypic lymphocytes. Is this good or bad? Explain why

A

Polytypic is good. Means multiple cell lines. However, if monoclonality develops (presence of abundant B cells) that is not good. Means patient has likely progressed to full blown lymphoma = not good good

Derm Rheum (39 decks)

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