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Connective Tissue Disorders Flashcards

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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

Study These Flashcards
A

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

25
Treating Keratoconjunctivities (KCS)
Replacing or supplementing deficient tears
26
Immunosuppressive Treatment of Systemic Sjogren's
Hydroxychloroquine | Methotrexate
27
Define Scleroderma
Thickened, hardened skin
28
Spectrum of Scleroderma
Limited anatomic extent affecting only the skin & adjacent tissues Associated with systemic involvement
29
Pathophysiology of Systemic Sclerosis
Vascular damage Immune activation Excessive synthesis of extracellular matrix with deposition of structurally normal collagen results
30
Vascular Damage in Systemic Sclerosis
Vasoconstriction Endothelia's are fibrinogenic Sera found to be cytotoxic to endothelial cells
31
Immune Activation in Systemic Sclerosis
Activation of endothelial cells Up regulation of adhesion molecules Leukocyte migration out of the vasculature
32
Diagnosis of Systemic Sclerosis
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
Specific Organ Manifestations Treatment Directed at
``` Cutaneous manifestations Raynaud's phenomenon Musculoskeletal manifestations GI manifestations Pulmonary manifestations Cardiac manifestations Renal manifestations ED & dyspaurenia ```
34
Cutaneous Manifestations of Systemic Scleroderma
``` Edema & erythema of skin Pruritus Digital ulcers Pitting at the finger tips Telangiectasia ```
35
Treatment for Cutaneous Manifestations
``` Emollient creams: soften skin & decrease pruritus Antihistamines & doxepin: pruritus UVA therapy Highly potent topical steroids Methotrexate ```
36
Define Raynaud's Phenomenon
Exaggerated vascular response to cold temperature or emotional stress
37
Manifestation of Raynaud's Phenomenon
Sharply demarcated color changes of the skin of the digits due to abnormal vasoconstriction of digital arteries & cutaneous arterioles
38
In Systemic Sclerosis, Raynaud Phenomenon can Lead to
Ulcerations Digital infarctions Gangrene
39
Treatment of Raynaud's Phenomenon
``` Prevention CCBs: nifedipine or amlodipine Alpha blockers Low dose ASA ED pills?? Treat associated complications ```
40
Musculoskeletal Manifestations of Systemic Sclerosis
Erosive, inflammatory arthritis similar to rheumatoid arthritis
41
Treatment of Musculoskeletal Manifestations of Systemic Sclerosis
Methotrexate
42
GI Manifestations of Systemic Sclerosis
GI tract involved in all smooth muscle areas | GERD
43
Treatment of GI Manifestations in Systemic Sclerosis
Preventative measures PPIs Promotility agent: erythromycin
44
Pulmonary Manifestations of Systemic Sclerosis
Lung fibrosis Pulmonary HTN Increased risk of lung cancer CT: ground glass appearance
45
Treatment of Pulmonary Manifestations of Systemic Steroids
Cyclophosphamide | Steroids
46
Cardiac Manifestations of Systemic Sclerosis
Conduction abnormalities or dysrhythmias
47
Treatment of Cardiac Manifestations of Systemic Sclerosis
Anti-arrhythmics | Cardiac pacing
48
Renal Manifestations of Systemic Sclerosis
Acute renal crisis
49
Treatment of Renal Manifestations of Systemic Sclerosis
ACE inhibitors
50
Define CREST
``` Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangectiasia ```
51
Pathophysiology of CREST
Production of anti-centromere & anti-nuclear antibodies | Development of pulmonary HTN & CHF
52
Define Polymyositis/Dermatomyositis
Inflammatory muscle disease that can occur with or without a cutaneous eruption
53
Clinical Manifestations of Polymyositis/Dermatomyositis
Proximal muscle weakness Rash over PIP & MCP (Gottron's sign) Heliotrope over eyelids Shawl Sign
54
Presentation of Proximal Muscle Weakness in Polymyositis/Dermatomyositis
Insidious onset Symmetric Mild myalgias
55
Diagnosis of Polymyositis/Dermatomyositis
``` Clinically Med Rec Muscle enzyme evaluation: CK, LDH, AST, ALT Elevated ANA Muscle biopsy ```
56
Pathogenesis of Dermatomyositis
Humorally-mediated (B-cells) Cellular infiltrate focused around blood vessels Abnormal muscle fibers grouped in one portion of fascicle
57
Pathogenesis of Polymyositis
Cellular infiltrate within the fascicle | Abnormal muscle cells scattered throughout the fascicle
58
Treatment of Cutaneous Manifestations of Polymyositis/Dermatomyositis
Mild to moderate topical steroid therapies | Hydroxychloroquine or methotrexate
59
Treatment of Muscular Manifestations of Polymyositis/Dermatomyositis
High-dose systemic steroids PT program Immunosuppressant medication: methotrexate, azathioprine

Rheumatology (8 decks)

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