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Musculoskeletal - Pathology > Systemic Sclerosis > Flashcards

Systemic Sclerosis Flashcards

1
Q

Why do people die of systemic sclerosis?

A

Internal Organ involvment

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2
Q

The interplay of what 4 factors leads to systemic sclerosis?

A
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3
Q

What gender and ethnic group have a higher incidence of scleroderma?

A

African Americans and Women have increased incidence of scleroderma

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4
Q

How does the composition of inflammatory cells change over time in scleroderma?

A

• T cell infiltration may develop into a monocytic infiltration as a results of IFN-gamma release. Then over time the monocytes differentiate into more of a fibroblastic type of cell.

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5
Q

The figure here shows the involvment of different cell types in scleroderma.

A
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6
Q

What is indicated by the presence of papilledema?

A

• Very high blood pressure

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7
Q

What are the earliest pathologic changes in SSc and what causes these changes?

A

Changes in endothelial cell function with increased apoptosis, MHC class II (on APCs) and ICAM-1 (intracellular adhesion molecule) expression on endothelial cells.

• Increased endothelial activation will lead to platelet aggregation and digital artery occlusion. The occurance of this process on a chronic basis leads to vessel wall thickening/lumenal narrowing and telangiectasis of the vasa vasorum.

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8
Q

What 3 steps does Raynaud’s occur in?

A

Loss of blood ciculation = white, then deoxgenated blood = blood, then hyperemia as bloodflow returns causes release of vasodilatory mediators like Adenosine and NO.

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9
Q

What is shown here?
• is this a result of primary or secondary disease?

A

Capillary dropout from repeated ischemic injury is a feature of SECONDARY Raynaud’s not primary

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10
Q

What features of Scleroderma are shown here?
• what are some treatment options for these patients?

A

A. Sclerodactyly
B. Thicked Skin Overlying Chest
C. Inflammatory Signs of Early Disease
D. Vitiligo

Treatment options:
• Sildenafil (viagra - PDE5 inhibition)
• Losartin (ACE receptor blocker)
• CCB’s

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11
Q

What are some other locations this could show up?
• would you expect to happen if you pressed on it?

A

This is a Telangectasia of SSc, these are NON-blanching and typically are found on the face, tongue, and chest

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12
Q

What are the 2 types of scleroderma?
• what are their subtypes?
• How do they differ?

A

Localized scleroderma consists of 2 types of scleroderma:
• Morphea - skin lesion biopsy will look like normal scleroderma but only invovles the skin
• Linear Scleroderma - shows a single line of thickening deep into muscle

Systemic Scleroderma:
• Limited Scleroderma - mostly skin with limited invovlment of internal organs.

  • Diffusion Scleroderma - both skin and internal organs
  • Sine Scleroderma - only involves internal organs
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13
Q

How can you tell the difference between limited and diffuse scleroderma without looking at internal structures.

A

• Skin involvement pattens

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14
Q

What are some organs commonly involved in Systemic Scleroderma?
• How are they involved?

A

GI:
• Esophageal Dysmotility; GAVE (gastric vascular vascular ectasia) Pancreatic Insufficiency; Primary Biliary Cirrhosis; Giant Diverticuli with decreased intestinal mobility

Cardiopulmonary:
• EFFUSIONS (common 1/5); Cardiac fibrosis; Pulmonary Artery HTN or pumonary fibrosis => Cor Pulmonale; Raynaud’s

Glands:
• Salivary (Sjorgren’s), Pancreatic

Musculoskeletal:
• Osteoporosis, Arthritis, Myositis

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15
Q

What 4 antibody types are associated with scleroderma?

A
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16
Q

What Phenotype of Scleroderma is associated with Anti-topoisomerase antibodies?

A
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17
Q

What Phenotype of Scleroderma is associated with Anti-centromere antibodies?

A

Notice the SICCA syndrome association (dry eyes, mouth, etc.)

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18
Q

What Phenotype of Scleroderma is associated with Anti-PM/scl antibodies?

A
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19
Q

What Phenotype of Scleroderma is associated with Anti-U1-RNP antibodies?

A

MIXED disease

20
Q

_______________(a) was typically the cause of death in scleroderma before the advent of _______________(b)

A

Renal Crisis was typically the cause of death in scleroderma before the advent of Ace inhibitors

21
Q

What is scleroderma renal crisis (SRC)?
• how often does this occur?
• how do we control this potentially fatal condition?

A

Scleroderma Renal Crisis (SRC)
• Characterized by three main things: 1Abrupt increase in blood pressure associated with increase in plasma renin activity that may precipitate 2acute kidney damage resulting in a 3mild proteinuria with only a few cell or casts.

Controlling Scleroderma Renal Crisis:
• Early pharmacologic intervention with ACE inhibitors is crucial and may reverse the condition.

22
Q

What are 3 important risk factors for developing Scleroderma Renal Crisis?
• what are some other associations?

A

3 important risk factors:

Risk Factors:
• Early diffuse skin disease
• Use of Corticosteroids
• ***Presence of anti-RNA pols III abs***

Other Associations:
• New anemia (schistocytes), New cardiac events (hrt failure, pericardial effusion), presence of anti-RNA pols I, use of drugs like NSAIDs, cyclcosporin.

23
Q

How commonly is the GI tract involved in scleroderma?
• Where do these symptoms typically manisfest?
• Associated progosis with different locations of involvment?

A

GI tract invovlment is a UNIVERSAL feature of scleroderma. Most commonly the Upper GI tract is involved in the disease, but the lower tract may also be involved and is associated with a poor prognosis.

24
Q

What manifestations of scleroderma are shown here?

A

A. Esophageal dysmotility with congestion and Dilation
B. Watermelon Stomach due to vascular ectasias (pathologic dilation of BVs) in the stomach
C/D. Gastric Dysmotility with pseudoobstruction

25
Q

What are your chances of having pulmonary manifestations of scleroderma if you have the disease?

A

Pulmonary manifestations are likely and there are many different ways disease may manifest.

Commonly:
• Interstitial Lung Disease/Pulmonary Artery HTN
• Lung Cancer of any type
• Aspiration pneumonia
• Pleural Effusions.

26
Q

How might someone with scleroderma present whenever they become symptomatic with scleroderma pulmonary manifestations?
• what underlying factors may be contributing?
• When in the disease process does this happen?

A

Pulmonary involvment typically happens in later stages of diffuse scleroderma and may present as dyspena, fatigue, dry cough, and/or GERD with crackels and rhales on auscultation. Underlying causes are most commonly Interstitial Lung Disease and PAH.

• Other contributatory causes may be Chest Wall Restriction due to fibrosis of chest wall skin, infection, and use of MTX.

27
Q

What are some distinctive signs of pulumonary artery hypertension that you might see on a cardiopulmonary physical exam?

A
  • LOUD P2 sound with a fixed split
  • Parasternal Heaves (i think…)
28
Q

Is PAH a symptom or CREST, Diffuse Scleroderma, or both?

A

PAH is a symptoms of BOTH Limited Scleroderma (CREST) associated with anti-centromere antibodies, and diffuse SSc with antifibrillairn antibodies (Anti-U3RNP antibodies).

29
Q

Its important to diagnose PAH EARLY so that you can treat it. DON’T mistake it for a lack of physical fitness.

A
30
Q

Linear Scleroderma
• 5 subtypes?

A

5 subtypes:
• Plaque morphea
• generalized morphea
• Bullous morphea
• Linear Morphea
• Deep morphea

31
Q

What is plaque morphea?
• how common is this?

A

Plaque morphea is the most common subtype of localized scleroderma and is an isolated circular patch of thickened skin.

32
Q

What is generalized morphea?

A

MULTIPLE lesions involving extensive areas of the skin that occasionally merge to mimic changes seen in systemic sclerosis.

33
Q

What is keloid morphea?

A

Nodular form of localized scleroderma that resembles keloids

34
Q

What is bullous morphea?

A

Subepithelial form of localized scleroderma where bullae form under epithelium (RARE)

35
Q

What is Linear Scleroderma?

A

A form of localized scleroderma that forms a linear streak that crosses dermatomes and is associated with atrophy of muscle and infiltrates in the the underlying bone and rarely the brain.

AKA “en coup de sabre” to decribe the jagged lesions see at the dermal-epidermal junction

36
Q

What is mixed connective tissue disease (MCTD)?
• how rapidly does this disease onset?
• How does it typically onset?

A

Mixed Connective Tissue disease is a disease with features of lupus, scleroderma, and inflammatory myositis.

Onset of mixed connective tissue disease is slow and may take several years to develop. Typically this occurs with the aquisition of one disease (or feature of a disease) at a time.

37
Q

What antibody in scleroderma is associated with Mixed Connective tissue disease?

A

U1-RNP

38
Q

What key features should you look for in someone you suspect is presenting with MCTD?
• what organ system is involved in 25% of cases? Disease(s)?
• What typically kills these people?

A

RAYNAUD’s => if they don’t have this then reconsider your diagnosis.

In 1/4 of patients renal involvement is manifested as membranous glomerularnephritis. Proliferative GN is rare.

Patients with this disease typically die of PAH

39
Q

What key features should you look for in a skin lesion of scleroderma?

A

1Excessive Collagen Deposition, 2Deep Fibrosis and perivascular 3lymphohistiocytic infiltrates.

40
Q

What is shown here?
• key characterisitics?

A

this is normal skin, notice the fairly deep ridges at the dermal-epidermal junctionan the presence of blood vessels and glands in the dermis. There is also an absence of lymphocytes.

41
Q

What is shown here?
* Key features?

A

Skin seen in the early stages of scleroderma. There is some loss of the papillae at the dermal-epidermal junction. No blood vessels are apparent and we see a lymphocytic infiltrate (bottom right). With loss of follicles and glands.

42
Q

What is shown here?
• key features?

A

This is late stage scleroderma. Lymphocytic infiltrates have regressed and and rete pegs (papilla a dermal-epidermal junction) are flattened. There is also an absence of glands. Notably pigment has been lost from the dermal epidermal junction possibly causing a vitiligo type effect in this person (see earlier picture below for comparison to early stage disease image)

43
Q

What is shown here?
• where on the body of a scleroderma patient would this likely be found?

A

Calcareous Lesions - are related to tumoral calcinosis
• these are large painful masses in the periauricular soft tissue composed of hydroxyappetite

44
Q

What changes might you see in the joints of someone with scleroderma?

A
45
Q

What is shown in each of these images?

A
46
Q

These changes are associated with Raynaud’s Phenomenon. Would you expect them to be more associated with Primary or Secondary Raynauds?

A

These changes would be associated with SECONDARY Raynaud’s. Vascular damage does not occur in primary Raynaud’s.

Note: 1-2% of ppl. with primary Raynaud’s progress to SSc (may just be poorly diagnosed SSc)

Musculoskeletal - Pathology (17 decks)

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