This is a lecture by Dr. Joseph Hartmann from the Ghana Emergency Medicine Collaborative. To download the editable version (in PPT), to access additional learning modules, or to learn more about the project, see http://openmi.ch/em-gemc. 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/.

1. 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 open.michigan@umich.edu 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

2. Attribution Key
for more information see: http://open.umich.edu/wiki/AttributionPolicy
Use + Share + Adapt
{ Content the copyright holder, author, or law permits you to use, share and adapt. }
Public Domain – Government: Works that are produced by the U.S. Government. (17 USC § 105)
Public Domain – Expired: Works that are no longer protected due to an expired copyright term.
Public Domain – Self Dedicated: Works that a copyright holder has dedicated to the public domain.
Creative Commons – Zero Waiver
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
Make Your Own Assessment
{ Content Open.Michigan believes can be used, shared, and adapted because it is ineligible for copyright. }
Public Domain – Ineligible: Works that are ineligible for copyright protection in the U.S. (17 USC § 102(b)) *laws in
your jurisdiction may differ
{ Content Open.Michigan has used under a Fair Use determination. }
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
2
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.

3. Collagen Vascular Disease Considerations for Emergent
Management
Joseph H. Hartmann, D.O.
3

4. 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

5. 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

6. Raynaud Phenomenon
• Definition:
– Exaggerated vasospasm of digital / precapillary arteries of fingers, toes, ears, nose, knees
and nipples
– Initiated by exposure to cold or emotional
stress
6

7. Raynaud Phenomenon
• Etiology:
– Unknown
– May be first precursor of future connective
tissue disorder
• Clinical Presentation:
– Triphasic
– Typically begins in one finger then symmetrically spreads to other fingers but usually
spares the thumb
7

8. 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

9. National Heart, Lung, and Blood Institute, Wikimedia Commons
9

10. Intermedichbo, Wikimedia Commons
10

11. Niklas D, Wikimedia Commons
11

12. Raynaud Phenomenon
• Relative temperature shifts may be
provocative
• General body chill can trigger
• Fear / anxiety can trigger
12

13. 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

14. 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

15. Source Undetermined
Source Undetermined
15

16. Source Undetermined
16

17. Source Undetermined
17

18. 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, dipyridamole, heparin, LMWH)
• sympathectomy
18

19. 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

20. 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

21. 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

22. Centers for Disease Control and Prevention, Wikimedia Commons
22

23. Source Undetermined
Source Undetermined
23

24. Reactive Arthritis
• Classic triad of Reiter syndrome
– Nongonococcal urethritis (Chlamydia)
– Conjunctivitis / anterior uveitis
– Arthritis
• Following GI infection with Shigella, Salmonella, Campylobacter, Yersinia, Clostridium difficile (?)
• Infection precedes arthritis by 2 - 6 wk
24

25. Reactive Arthritis
• Treatment:
– NSAIDs
• Naproxen 500 mg TID
• Indomethacin 50 mg TID
– Intra-articular glucocorticoids
– Expect resolution 3 – 12 months
25

26. 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

27. Source Undetermined
Source Undetermined
27

28. Source Undetermined
Source Undetermined
28

29. 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

30. Rheumatoid Arthritis
• Pulmonary
– Pleural effusion *
– Interstitial fibrosis
– Pulmonary nodules
• Cardiac
– Pericarditis *
– Pericardial effusion 10%
– Myocarditis
– CAD – sudden death, MI *
30

31. Rheumatoid Arthritis
• Renal
– Focal glomerulonephropathy
– Drug toxicity from treatment *
• Vasculitis
– Distal infarcts, ulcerations, gangrene
• CNS
– Generally spared
31

32. Systemic Sclerosis - Scleroderma
• Definition:
– Disease process characterized by progressive
fibrosis, vascular abnormalities and inflammatory processes that can be manifested quite
locally or diffusely systematically with organ
system involvement
• Etiology:
– Poorly understood
32

33. 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

34. Systemic Sclerosis
• Skin
– Sclerodactyly
– Telangiectasias
– Digital ulcers
– Calcinosis
– Raynaud phenomenon
34

35. 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.
Source Undetermined
35

36. Telangiectasia
Kerry J, Flickr
Dilation of small vessels and capillaries cause flat
red marks to appear on the skin
36

37. Jmh649, Wikimedia Commons
37

38. Source Undetermined
38

39. Source Undetermined
39

40. 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

41. 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

42. Lipothymia, Wikimedia Commons
42

43. Source Undetermined
Source Undetermined
43

44. 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

45. 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 autoantibodies with systemic consequences.
45

46. 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

47. Source Undetermined
Source Undetermined
47
Source Undetermined

48. 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

49. SLE
• Neurologic
– Cognitive defects, cephalgia, seizures,
peripheral neuropathies (stocking / glove),
psychosis, stroke (antiphospholipid antibody
syndrome)
• Musculoskeletal
– Arthritis, atrophy, tendon rupture
49

50. 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

51. 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

52. 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

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

54. Giant Cell Arteritis
Source Undetermined
54

55. 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

56. Polymyositis / Dermatomyositis
• Definition:
– Idiopathic inflammatory myopathies
• Etiology:
– Genetic component with presumed environmental triggers
– Peak incidence between 40 – 50 yrs of age
– Female : Male
2:1
56

57. 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

58. Madhero88, Wikimedia Commons
Madhero88, Wikimedia Commons
58

59. 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

60. Madhero, Wikimedia Commons
60

61. Madhero88, Wikimedia Commons
61

62. Source Undetermined
Source Undetermined
62

63. 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

64. 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