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Rheumatology > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

Connective Tissue Disorders

A 
Sjogren's syndrome
Scleroderma
Raynaud's phenomenon
Myositis/Dermatomyositis
CREST syndrome
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2
Q

Define Sjogren’s Syndrome (SS)

A

Chronic inflammatory disorder characterized by diminished lacrimal & salivary gland function

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3
Q

Primary Sjogren’s Syndrome

A

Not associated with other disease

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4
Q

Secondary Sjogren’s Syndrome

A

Complication of other rheumatic conditions (RA, scleroderma, lupus)

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5
Q

Pathogenesis of Sjogren’s Syndrome

A

Lymphocytic infiltration into lacrimal & salivary glands, where lymphocytes are not normal found
Impairs function

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6
Q

Symptoms of Mild Sjogren’s Syndrome

A 
Dry eyes
Dry mouth
Fatigue
Myalgias
Cognitive dysfunction
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7
Q

Symptoms of Severe Sjogren’s Syndrome

A

Florid salivary gland enlargement
Adenopathy
Antibodies to Ro/SSA & La/SSB antigens
Cryoglobulinemia
Hypocomplementemia
Propensity to develop non-Hodgkin’s lymphoma

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8
Q

Extraglandular Involvement in Sjogren’s Syndrome

A 
Vaginal dryness
Dyspareunia
Interstitial nephritis
Recurrent thirties & sinusitis
Bronchial dryness
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9
Q

Define Karatoconjunctivitis Sicca

A

Deficiency in tear production leading to symptoms of dry eyes which usually presents insidiously overall several years

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10
Q

Complications of Karatoconjunctivitis Sicca

A

Corneal ulceration

Infection of the eyelids

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11
Q

Clinical Manifestations of Sjogren’s Syndrome

A

Karatoconjuncitivitis sicca
Xerostomia
Salivary gland enlargement

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12
Q

Complications of Xerostomia

A 
Dental caries
Oral candidiasis
Laryngotracheal reflux
Chronic esophagitis
Weight loss
Nocturia
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13
Q

Salivary Gland Enlargement in Sjogren’s Syndrome

A

Firm, diffuse, & non-tender

Occur in parotid & submandibular glands

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14
Q

Criteria for Diagnosis of Sjogren’s Syndrome

A

Ocular symptoms of inadequate tear production
Ocular signs of corneal damage
Oral symptoms of decreased saliva production
Salivary gland histopathology demonstrating foci of lymphocytes
Tests indicating impaired salivary gland function
Presence of autoantibodies (Anti-Ro/SSA & Anti-La/SSB)

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15
Q

Exclusion Criteria for Sjogren’s Syndrome

A 
Prior head/neck irradiation
Infection with hepatitis C
AIDS
Lymphoma
Sarcoidosis
Graft-vs-host disease
Recent use of anti-cholinergic meds
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16
Q

Primary Sjogren’s Syndrome Diagnosis Criteria

A

Positive salivary gland biopsy or anti-Ro/SSA and/or anti-La/SSB & satifies 4/6 criteria
Satisfies 3/4 objective items (ocular signs, biopsy, salivary gland involvement, autoantibodies)

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17
Q

Secondary Sjogren’s Syndrome Diagnosis Criteria

A

CTD present
One item indicative of ocular or oral dryness
2/3 objective items

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18
Q

Diagnosing of Sjogren’s Syndrome

A

Thorough H&P
Schirmer test
Rose bengal test
Labs: CBC w/diff, CMP, ESR, UA

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19
Q

Specialized Lab Testing for Sjogren’s Syndrome

A 
ANA
RF
Antibodies to Ro/SSA, La/SSB, Sm, & RNP antigens
Antibody to anti-centromere B or topoisomerase
ANCA (vasculitis)
Serum C3 & C4
Antibodies to dsDNA
Serum cryoglobulins
SPE
Ig levels
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20
Q

Diagnosis of Sjogren’s Syndrome

A

Salivary gland biopsy
Antibodies to Ro/SSA
Antibodies to La/SSB

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21
Q

Treatment of Sjogren’s Syndrome

A

Topical therapy for dry eyes & dry mouth

Treatment of systemic manifestations

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22
Q

3 Aspects of Treating Sicca Symptoms

A

General principles
Xerostomia
Karatoconjunctivities

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23
Q

General Treatment Principles of Sicca Symptoms

A 
Avoidance of irritants
Avoidance of exacerbating drugs
Avoidance of low humidity atmospheres
Consider candidiasis if  a painful mouth
Consider blepharitis if adequate hydration
Excellent oral hygiene
Topical moisturizers & lozenges
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24
Q

Treating Xerostomia

A

Stimulation of existing salivary flow: sugarless candy, dried fruit slices, citrus flavored tablets, gum
Muscarinic agonists
Artificial saliva

25
Q

Treating Keratoconjunctivities (KCS)

A

Replacing or supplementing deficient tears

26
Q

Immunosuppressive Treatment of Systemic Sjogren’s

A

Hydroxychloroquine

Methotrexate

27
Q

Define Scleroderma

A

Thickened, hardened skin

28
Q

Spectrum of Scleroderma

A

Limited anatomic extent affecting only the skin & adjacent tissues
Associated with systemic involvement

29
Q

Pathophysiology of Systemic Sclerosis

A

Vascular damage
Immune activation
Excessive synthesis of extracellular matrix with deposition of structurally normal collagen results

30
Q

Vascular Damage in Systemic Sclerosis

A

Vasoconstriction
Endothelia’s are fibrinogenic
Sera found to be cytotoxic to endothelial cells

31
Q

Immune Activation in Systemic Sclerosis

A

Activation of endothelial cells
Up regulation of adhesion molecules
Leukocyte migration out of the vasculature

32
Q

Diagnosis of Systemic Sclerosis

A

Skin induration
Heartburn or dysphagia
Acute onset HTN & renal insufficiency
Dyspnea on exertion associated with interstitial pulmonary changes
Pulmonary HTN
Diarrhea with malabsorption
Facial, lip, or hand telangiectasis
Digital infarctions and/or digital tip pitting
Heart issues

33
Q

Specific Organ Manifestations Treatment Directed at

A 
Cutaneous manifestations
Raynaud's phenomenon
Musculoskeletal manifestations
GI manifestations
Pulmonary manifestations
Cardiac manifestations
Renal manifestations
ED & dyspaurenia
34
Q

Cutaneous Manifestations of Systemic Scleroderma

A 
Edema & erythema of skin
Pruritus
Digital ulcers
Pitting at the finger tips
Telangiectasia
35
Q

Treatment for Cutaneous Manifestations

A 
Emollient creams: soften skin & decrease pruritus
Antihistamines & doxepin: pruritus
UVA therapy
Highly potent topical steroids
Methotrexate
36
Q

Define Raynaud’s Phenomenon

A

Exaggerated vascular response to cold temperature or emotional stress

37
Q

Manifestation of Raynaud’s Phenomenon

A

Sharply demarcated color changes of the skin of the digits due to abnormal vasoconstriction of digital arteries & cutaneous arterioles

38
Q

In Systemic Sclerosis, Raynaud Phenomenon can Lead to

A

Ulcerations
Digital infarctions
Gangrene

39
Q

Treatment of Raynaud’s Phenomenon

A 
Prevention
CCBs: nifedipine or amlodipine
Alpha blockers
Low dose ASA
ED pills??
Treat associated complications
40
Q

Musculoskeletal Manifestations of Systemic Sclerosis

A

Erosive, inflammatory arthritis similar to rheumatoid arthritis

41
Q

Treatment of Musculoskeletal Manifestations of Systemic Sclerosis

A

Methotrexate

42
Q

GI Manifestations of Systemic Sclerosis

A

GI tract involved in all smooth muscle areas

GERD

43
Q

Treatment of GI Manifestations in Systemic Sclerosis

A

Preventative measures
PPIs
Promotility agent: erythromycin

44
Q

Pulmonary Manifestations of Systemic Sclerosis

A

Lung fibrosis
Pulmonary HTN
Increased risk of lung cancer
CT: ground glass appearance

45
Q

Treatment of Pulmonary Manifestations of Systemic Steroids

A

Cyclophosphamide

Steroids

46
Q

Cardiac Manifestations of Systemic Sclerosis

A

Conduction abnormalities or dysrhythmias

47
Q

Treatment of Cardiac Manifestations of Systemic Sclerosis

A

Anti-arrhythmics

Cardiac pacing

48
Q

Renal Manifestations of Systemic Sclerosis

A

Acute renal crisis

49
Q

Treatment of Renal Manifestations of Systemic Sclerosis

A

ACE inhibitors

50
Q

Define CREST

A 
Calcinosis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telangectiasia
51
Q

Pathophysiology of CREST

A

Production of anti-centromere & anti-nuclear antibodies

Development of pulmonary HTN & CHF

52
Q

Define Polymyositis/Dermatomyositis

A

Inflammatory muscle disease that can occur with or without a cutaneous eruption

53
Q

Clinical Manifestations of Polymyositis/Dermatomyositis

A

Proximal muscle weakness
Rash over PIP & MCP (Gottron’s sign)
Heliotrope over eyelids
Shawl Sign

54
Q

Presentation of Proximal Muscle Weakness in Polymyositis/Dermatomyositis

A

Insidious onset
Symmetric
Mild myalgias

55
Q

Diagnosis of Polymyositis/Dermatomyositis

A 
Clinically
Med Rec
Muscle enzyme evaluation: CK, LDH, AST, ALT
Elevated ANA
Muscle biopsy
56
Q

Pathogenesis of Dermatomyositis

A

Humorally-mediated (B-cells)
Cellular infiltrate focused around blood vessels
Abnormal muscle fibers grouped in one portion of fascicle

57
Q

Pathogenesis of Polymyositis

A

Cellular infiltrate within the fascicle

Abnormal muscle cells scattered throughout the fascicle

58
Q

Treatment of Cutaneous Manifestations of Polymyositis/Dermatomyositis

A

Mild to moderate topical steroid therapies

Hydroxychloroquine or methotrexate

59
Q

Treatment of Muscular Manifestations of Polymyositis/Dermatomyositis

A

High-dose systemic steroids
PT program
Immunosuppressant medication: methotrexate, azathioprine

Rheumatology (8 decks)

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