Scleroderma/MCTD/Sjogren - Postlethwaite/Gupta Flashcards by Jacob McMillan

Another name for systemic sclerosis?

Scleroderma…were off to a good start here

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Pathophysiologic quartet of Systemic Sclerosis?

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

Fibrosis

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Epidemiology of Scleroderma.

Classic presentation is in?

Middle aged females (30-50 years old)

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

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

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In Scleroderma, endothelial cell dysfunction causes what 3 features?

leads to:

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

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Fibroblasts role in scleroderma?

Collagen deposition

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Vasculature/microvasculature involvement in scleroderma?

Raynaud’s phenomenon in fingers/toes

Telangiectasis of the vasa vasorum

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

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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What are we looking at here?

What does (a) show?

What about (b)?

What is the significance of (b)?

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

development of digital ulcers

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2 main types of Systemic Sclerosis?

Limited and Difffuse

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Skin/organ involvement in Limited SS?

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

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Skin/organ involvement in Difuse SS?

Skin involvemement is diffuse with early visceral involvement

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

CREST syndrome - Limited

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

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Common organs involved in Diffuse Scleroderma?

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

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What is this? What disease is it associated with?

Gastric Antral Vascular Ectasia

= dilatated arteries that are prominent in stomach

“Watermelon appearance”

Diffuse Scleroderma

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What 4 antibodies are associated with scleroderma?

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

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Diffuse Scleroderma associated with what antibody?

Anti-Scl 70 (topoisomerase 1)

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Antibodies associated with Limited Scleroderma?

Anti-centromere antibodies

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

Scleroderma renal crisis (SRC)

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Scleroderma Renal Crisis risk factors?

Early diffuse skin disease

use of corticosteroids

anti-RNA polymerase III antibodies

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

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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Raynaud’s + what GI prob = likely Scleroderma?

difficulty swallowing (dysphagia)

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Mortality in Scleroderma is now most likely associated with?

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

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

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

"En coup de sabre" deals with what type of scleroderma?

Localized scleroderma

Localized scleroderma is: Systemic or nonsystemic? Primarily in children or adults? How many major types? Name these types

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"

What is mixed connective tissue disease?

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

MCTD is most commonly associated with what antibody?

U1-RNP

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

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

25 % of MCTD patients develop what?

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

Most common cause of death in MCTD?

Pulmonary HTN

Rare finding in MCTD?

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

Common early finding in MCTD?

Inflammatory arthritic manifestations: Fingers puffy, fusiform PIP swelling, Periungual infarcts

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

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

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

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)

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

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

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

Top = Lupus nephritis. Glomeruli are **enlarged** (due to mesangial proliferation) and **hypercellular** Bottom = SRC. Glomeruli are **shrunken** and **lack inflammatory cells**

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

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

Definition of Sjogren's?

Autoimmune destruction of minor salivary glands and lacrimal glands

List 3 clinical hallmarks of Sjogrens?

- Keratoconjunctivitis sicca (dry eyes) - Xerostomia (dry mouth) - Parotid gland swelling

List extraglandular manifestations of Sjogrens?

- Fatigue - Raynaud's - Polyarthralgia/arthritis - Interstitial lung disease - Neuropathy - Purpura

Sjogren Epidemiology Race? Age involvement? Age of onset? 90% of Sjogren's patients are ? Prevalence?

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

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

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

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

Type 1 Renal Tubular Acidosis = Know this Also: Interstitial nephritis, glomerulonephritis, and nephrogenic diabetic insipidus

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

Dysphagia (difficulty swallowing)

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

Neuromyelitis optica

Sjogren Syndrome antibodies to know? Which is most specific?

ANA (85%) Anti-SSA/Ro (33%) Anti-SSB/La (33%) = Most specific

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

Extraglandular manifestations (e.g. neuropathies)

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

+ Rheumatoid factor

Common ocular manifestation seen in Sjogrens?

Keratoconjunctivitis sicca (keratitis caused by decreased lacrimal secretions)

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

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

Oral manifestations of Sjogren's?

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

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

Primary and Secondary

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

Primary

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

Primary

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

Secondary

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

Secondary

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

No, likely malignant. Need to get parotid gland biopsy

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

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

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

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