project: document title: collagen vascular disease ... · – disease process characterized by...

64
Project: Ghana Emergency Medicine Collaborative Document Title: Collagen Vascular Disease: Considerations for Emergent Management Author(s): Joseph Hartmann, D.O., 2012 License: Unless otherwise noted, this material is made available under the terms of the Creative Commons Attribution Share Alike-3.0 License: http://creativecommons.org/licenses/by-sa/3.0/ We have reviewed this material in accordance with U.S. Copyright Law and have tried to maximize your ability to use, share, and adapt it. These lectures have been modified in the process of making a publicly shareable version. The citation key on the following slide provides information about how you may share and adapt this material. Copyright holders of content included in this material should contact [email protected] with any questions, corrections, or clarification regarding the use of content. For more information about how to cite these materials visit http://open.umich.edu/privacy-and-terms-use. Any medical information in this material is intended to inform and educate and is not a tool for self-diagnosis or a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional. Please speak to your physician if you have questions about your medical condition. Viewer discretion is advised: Some medical content is graphic and may not be suitable for all viewers. 1

Upload: others

Post on 27-Apr-2020

1 views

Category:

Documents


0 download

TRANSCRIPT

Page 1: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Project: Ghana Emergency Medicine Collaborative Document Title: Collagen Vascular Disease: Considerations for Emergent Management Author(s): Joseph Hartmann, D.O., 2012 License: Unless otherwise noted, this material is made available under the terms of the Creative Commons Attribution Share Alike-3.0 License: http://creativecommons.org/licenses/by-sa/3.0/

We have reviewed this material in accordance with U.S. Copyright Law and have tried to maximize your ability to use, share, and adapt it. These lectures have been modified in the process of making a publicly shareable version. The citation key on the following slide provides information about how you may share and adapt this material. Copyright holders of content included in this material should contact [email protected] with any questions, corrections, or clarification regarding the use of content. For more information about how to cite these materials visit http://open.umich.edu/privacy-and-terms-use. Any medical information in this material is intended to inform and educate and is not a tool for self-diagnosis or a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional. Please speak to your physician if you have questions about your medical condition. Viewer discretion is advised: Some medical content is graphic and may not be suitable for all viewers.

1

Page 2: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Attribution Key

for more information see: http://open.umich.edu/wiki/AttributionPolicy

Use + Share + Adapt

Make Your Own Assessment

Creative Commons – Attribution License

Creative Commons – Attribution Share Alike License

Creative Commons – Attribution Noncommercial License

Creative Commons – Attribution Noncommercial Share Alike License

GNU – Free Documentation License

Creative Commons – Zero Waiver

Public Domain – Ineligible: Works that are ineligible for copyright protection in the U.S. (17 USC § 102(b)) *laws in your jurisdiction may differ

Public Domain – Expired: Works that are no longer protected due to an expired copyright term.

Public Domain – Government: Works that are produced by the U.S. Government. (17 USC § 105)

Public Domain – Self Dedicated: Works that a copyright holder has dedicated to the public domain.

Fair Use: Use of works that is determined to be Fair consistent with the U.S. Copyright Act. (17 USC § 107) *laws in your jurisdiction may differ Our determination DOES NOT mean that all uses of this 3rd-party content are Fair Uses and we DO NOT guarantee that your use of the content is Fair. To use this content you should do your own independent analysis to determine whether or not your use will be Fair.

{ Content the copyright holder, author, or law permits you to use, share and adapt. }

{ Content Open.Michigan believes can be used, shared, and adapted because it is ineligible for copyright. }

{ Content Open.Michigan has used under a Fair Use determination. }

2

Page 3: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Collagen Vascular Disease -Considerations for Emergent

Management

Joseph H. Hartmann, D.O.

3

Page 4: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Emergent Management ???

•  Chronic conditions with long-term management decisions made by others

but •  Because of their chronicity they present with

- flares of general disease process - end-organ involvement

•  Occasionally determine the initial diagnosis

4

Page 5: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Where are you taking me today?

•  Raynaud phenomenon •  Reactive arthritis (Reiter Syndrome) •  Rheumatoid arthritis •  Systemic sclerosis (Scleroderma) •  Systemic lupus erythematosus •  Polymyalgia rheumatica •  Polymyositis / Dermatomyositis •  Vasculitides

5

Page 6: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon

•  Definition: – Exaggerated vasospasm of digital / precapil-

lary arteries of fingers, toes, ears, nose, knees and nipples

–  Initiated by exposure to cold or emotional stress

6

Page 7: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon

•  Etiology: – Unknown – May be first precursor of future connective

tissue disorder •  Clinical Presentation:

– Triphasic – Typically begins in one finger then symmetric-

ally spreads to other fingers but usually spares the thumb

7

Page 8: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon

•  Triphasic progression – White – pallor

•  Lack of arterial flow due to vasospasm

– Blue •  Cyanosis from blood pooling

– Red •  Reactive hyperemia

–  Ischemic phases (white-blue) last 15-20 min 8

Page 9: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

National Heart, Lung, and Blood Institute, Wikimedia Commons

9

Page 10: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Intermedichbo, Wikimedia Commons

10

Page 11: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Niklas D, Wikimedia Commons

11

Page 12: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon

•  Relative temperature shifts may be provocative

•  General body chill can trigger

•  Fear / anxiety can trigger

12

Page 13: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon

•  Specific criteria – Symmetric episodic attacks – No evidence of peripheral vascular disease – No tissue gangrene, digital pitting, or tissue

injury – Negative nailfold capillary examination – Negative ANA, normal ESR

13

Page 14: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon

•  Nailfold capillary microscopy – – Place drop of immersion oil on periungual

area then examine with ophthalmoscope set at diopter 40 or a dissecting microscope.

– Should see regularly spaced capillary loops – Abnormal findings –

•  Enlarged or distorted capillary loops •  Relative paucity of loops

14

Page 15: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined Source Undetermined

15

Page 16: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined 16

Page 17: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined 17

Page 18: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon

•  Treatment – Avoidance strategies

•  Cold, nicotine, sympathomimetics (decongestants, diet pills, herbs containing ephedra)

– Drug therapy •  Ca channel blockers, direct vasodilators (NTG,

hydralazine, minoxidil), sympatholytics (methyldopa, reserpine, prazosin), prostaglandins, anticoagulation / antithrombotic tx (aspirin, dipyrid-amole, heparin, LMWH)

•  sympathectomy 18

Page 19: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Raynaud Phenomenon •  Severe ischemia

– Warm patient (body and digits) – Analgesics – Antiplatelet tx – aspirin – Vasodilator tx

•  Nifedipine extended release 30-60 mg daily •  Amlodipine 5-10 mg daily

– Topical NTG – Heparin / LMWH 24-72 hrs – Temporary chemical sympathectomy

•  Digital or regional block – lidocaine/bupivicaine

–  IV prostaglandin administration 19

Page 20: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Reactive Arthritis (Reiter Syndrome)

•  Definition: – Arthritis following a preceding infection without

intra-articular presence of the pathogen i.e. not a septic joint

•  Etiology: – Seronegative (rheumatoid factor negative) – Spondyloarthropathy (very likely HLA-B27 pos) – Follows a GU or GI infection

20

Page 21: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Reactive Arthritis

•  Clinical Presentation: – Male 15 – 35 y/o – Asymmetric, oligoarthritis (2-4 joints) – Usually involves lower extremeties and sacroiliac

joints – Skin lesions resembling pustular psoriasis on

palms and soles – keratoderma blennorrhagicum –  lesions on glans penis – balanitis circinata

21

Page 22: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Centers for Disease Control and Prevention, Wikimedia Commons 22

Page 23: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined Source Undetermined

23

Page 24: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Reactive Arthritis

•  Classic triad of Reiter syndrome – Nongonococcal urethritis (Chlamydia) – Conjunctivitis / anterior uveitis – Arthritis

•  Following GI infection with Shigella, Sal-monella, Campylobacter, Yersinia, Clost-ridium difficile (?)

•  Infection precedes arthritis by 2 - 6 wk

24

Page 25: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Reactive Arthritis

•  Treatment: – NSAIDs

•  Naproxen 500 mg TID •  Indomethacin 50 mg TID

–  Intra-articular glucocorticoids – Expect resolution 3 – 12 months

25

Page 26: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Rheumatoid Arthritis

•  Definition: – Chronic, symmetric polyarticular synovial joint

disease •  Nonarticular & Systemic Manifestations

– HEENT •  Episcleritis

–  Painless injection of episcleral vessels (self-limiting)

•  Scleritis – Dark red / purple discoloration with marked ocular

tenderness –  Potential for visual impairment and scleral rupture 26

Page 27: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

Source Undetermined 27

Page 28: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

Source Undetermined 28

Page 29: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Rheumatoid Arthritis

•  Cricoarytenoid joint – Dysphonia, hoarseness, stridor –  If fix in closed position could require emergent

tracheostomy •  Ligamentous destruction of transverse

ligament of C-2 with potential for cord compression

29

Page 30: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Rheumatoid Arthritis

•  Pulmonary – Pleural effusion * –  Interstitial fibrosis – Pulmonary nodules

•  Cardiac – Pericarditis * – Pericardial effusion 10% – Myocarditis – CAD – sudden death, MI *

30

Page 31: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Rheumatoid Arthritis

•  Renal – Focal glomerulonephropathy – Drug toxicity from treatment *

•  Vasculitis – Distal infarcts, ulcerations, gangrene

•  CNS – Generally spared

31

Page 32: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Systemic Sclerosis - Scleroderma

•  Definition: – Disease process characterized by progressive

fibrosis, vascular abnormalities and inflamma-tory processes that can be manifested quite locally or diffusely systematically with organ system involvement

•  Etiology: – Poorly understood

32

Page 33: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Systemic Sclerosis

•  Clinical Presentation: – Often initial signs are a thickening, hardening

of the skin, usually fingers, hands and face and Raynaud phenomenon

– Female > Male – African-Americans tend to have worse

prognosis due to greater likelihood of having a more severe diffuse form

33

Page 34: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Systemic Sclerosis

•  Skin – Sclerodactyly – Telangiectasias – Digital ulcers – Calcinosis – Raynaud phenomenon

34

Page 35: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

In scleroderma, the abnormal build-up of fibrous tissue in the skin can cause the skin to tighten so severely that the fingers curl and lose their mobility.

35

Page 36: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Telangiectasia

Dilation of small vessels and capillaries cause flat red marks to appear on the skin

Kerry J, Flickr

36

Page 37: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Jmh649, Wikimedia Commons

37

Page 38: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

38

Page 39: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

39

Page 40: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Systemic Sclerosis •  Pulmonary

–  Most common cause of death –  Interstitial lung disease

•  Alveolitis leading to eventual fibrosis

–  Pulmonary vascular disease •  Pulmonary hypertension

•  Cardiac –  Pericarditis w/wo effusion –  Myocardial fibrosis

•  Resultant ventricular dysfunction with diminished cardiac output

–  Dysrhythmias •  Fibrosis of conduction system resulting in sudden death

40

Page 41: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Systemic Sclerosis •  Renal

– Renal crisis •  Acute onset of renal failure •  Proteinuria and microscopic hematuria •  Abrupt onset of hypertensive emergency

•  Gastrointestinal – Hypomobility

•  Esophageal dysmotility / GERD •  Pseudo-obstruction, constipation •  Teleangectasias with bleeding •  Pneumatosis intestinalis

41

Page 42: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Lipothymia, Wikimedia Commons

42

Page 43: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

Source Undetermined

43

Page 44: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Systemic Sclerosis •  Treatment

– Generally immunosuppressive therapy – Based on specific organ system involved – Emergently

•  Pulmonary decompensation •  Cardiac – effusion, failure, dysrhythmia •  Renal crisis

–  ACE inhibitor is first line antihypertensive agent – Captopril – Captopril + Ca channel blocker –  Angiotensin receptor blocker for those who can not tolerate

ACE inhibitor

44

Page 45: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Systemic Lupus Erythematosus

•  Definition: – Chronic autoimmune disease characterized by

presence of autoantibodies with multi-organ system involvement

•  Etiology: – Genetic predisposition and nebulous factors

combine to alter immune cell function resulting in production of autoantigens and thereby auto-antibodies with systemic consequences.

45

Page 46: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

SLE •  Clinical Presentation:

– Female > Male 10 : 1 – Typical presentation 21 – 45 yrs of age – African American > Caucasians – Constitutional

•  Wt loss, fever, myalgias, arthralgias •  Fatigue often the most debilitating

– Skin •  Butterfly malar rash – may be fleeting •  Oral and nasal ulcerations

46

Page 47: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

Source Undetermined

Source Undetermined

47

Page 48: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

SLE •  Pulmonary

– Pleurisy, effusion, interstitial lung disease, pulmonary hypertension

– “lupus lung” – alveolar hemorrhage •  Cardiac

– Pericarditis *, effusion –  Increased risk for CAD

•  Renal – Lupus nephritis

•  Elevated creatinine, proteinuria, hypertension 48

Page 49: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

SLE

•  Neurologic – Cognitive defects, cephalgia, seizures,

peripheral neuropathies (stocking / glove), psychosis, stroke (antiphospholipid antibody syndrome)

•  Musculoskeletal – Arthritis, atrophy, tendon rupture

49

Page 50: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

SLE •  Treatment:

–  Immunosuppression •  Glucocorticoids •  Methotrexate, cyclophosphamide, azathioprine,

mycophenolate, rituximab – Causes of death

•  Early deaths – first few years –  Active lupus (cardiac, renal, CNS dz) –  Infection due to immunosuppression

•  Late deaths – Chronic effects of lupus (ESRD, CAD) –  Infection, malignancy

50

Page 51: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyalgia Rheumatica

•  Definition: – Rheumatic condition frequently associated

with giant cell (temporal) arteritis •  Etiology:

– Genetic predisposition – Relatively common – 50% of pts with GCA will develop PMR – 15% of pts with PMR will develop GCA

51

Page 52: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyalgia Rheumatica

•  Clinical Presentation: – Age 50 or older at onset – Bilateral aching and morning stiffness which

lasts 30 min or more for 1 month or more involving at least 2 of 3 areas

•  Neck or torso •  Shoulders or proximal arms •  Hips or proximal thighs

– ESR = 40 mm/hr or greater

52

Page 53: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyalgia rheumatica

Original Image: Twisp, Wikimedia Commons Modified Image Lena Carleton, University of Michigan 53

Page 54: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

Giant Cell Arteritis

54

Page 55: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyalgia Rheumatica

•  treatment: – Prednisone 10 – 20 mg / day

•  (compared to 60 mg / day dose for GCA)

– Rapid response is characteristic (often after first dose)

– Relapse is commonly seen requiring increase in prednisone

55

Page 56: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyositis / Dermatomyositis

•  Definition: –  Idiopathic inflammatory myopathies

•  Etiology: – Genetic component with presumed environ-

mental triggers – Peak incidence between 40 – 50 yrs of age – Female : Male 2 : 1

56

Page 57: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyositis / Dermatomyositis •  Clinical Presentation:

– Muscle weakness •  Onset is insidious •  Gradually worsening over months •  Typically symmetric and proximal

– Myalgias / muscle tenderness occurs in 25-50% but is mild compared to PMR or fibromyalgia

– Dermatologic findings in DM •  Gottron’s sign

–  Erythematous, often scaly exanthem occurring symmetrically over MCP and IP joints and / or over extensor surfaces of elbows and knees resembling psoriasis

57

Page 58: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Madhero88, Wikimedia Commons

Madhero88, Wikimedia Commons

58

Page 59: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyositis / Dermatomyositis – Heliotrope rash

•  Violaceous eruption on upper eyelids, often with swelling

– Shawl sign and V sign •  Diffuse flat erythematous lesion over chest and

shoulders (shawl sign) or over anterior neck and chest (V sign)

– Erythroderma •  Extensive areas of skin redness (malar, forehead)

– Mechanic’s hands (DM or PM) •  Rough, cracking skin at tips and lateral aspects of

fingers with irregular dirty appearing lines 59

Page 60: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Madhero, Wikimedia Commons

60

Page 61: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Madhero88, Wikimedia Commons

61

Page 62: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Source Undetermined

Source Undetermined 62

Page 63: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Polymyositis / Dermatomyositis •  Diagnostic testing

– Elevated CK, LDH, aldolase, aminotransferases •  Increased incidence of malignancy

associated with dermatomyositis •  Treatment:

– Glucocorticoid regimen •  initiate with high doses for several months to

establish disease control •  Slow taper to lowest effective dose for 9 – 12 months

– Glucocorticoid-sparing regimen •  Azathoprine / methotrexate 63

Page 64: Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by progressive fibrosis, vascular abnormalities and inflamma-tory processes that can be

Vasculitides •  Large vessel

– Takayasu arteritis – Giant cell (temporal) arteritis

•  Medium vessel – Polyarteritis nodosa – Kawasaki disease

•  Small vessel – Churg-Strauss arteritis – Wegener’s granulomatosis – Henoch-Schonlein purpura 64