Project: Ghana Emergency Medicine Collaborative
Document Title: Collagen Vascular Disease: Considerations for Emergent
Man...
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Collagen Vascular Disease Considerations for Emergent
Management
Joseph H. Hartmann, D.O.

3
Emergent Management ???
• 

Chronic conditions with long-term
management decisions made by others

but
• 

• 

Because of ...
Where are you taking me today?
• 
• 
• 
• 
• 
• 
• 
• 

Raynaud phenomenon
Reactive arthritis (Reiter Syndrome)
Rheumatoid...
Raynaud Phenomenon
•  Definition:
–  Exaggerated vasospasm of digital / precapillary arteries of fingers, toes, ears, nose...
Raynaud Phenomenon
•  Etiology:
–  Unknown
–  May be first precursor of future connective
tissue disorder

•  Clinical Pre...
Raynaud Phenomenon
•  Triphasic progression
–  White – pallor
•  Lack of arterial flow due to vasospasm

–  Blue
•  Cyanos...
National Heart, Lung, and Blood Institute, Wikimedia Commons

9
Intermedichbo, Wikimedia Commons

10
Niklas D, Wikimedia Commons

11
Raynaud Phenomenon
•  Relative temperature shifts may be
provocative
•  General body chill can trigger
•  Fear / anxiety c...
Raynaud Phenomenon
•  Specific criteria
–  Symmetric episodic attacks
–  No evidence of peripheral vascular disease
–  No ...
Raynaud Phenomenon
•  Nailfold capillary microscopy –
–  Place drop of immersion oil on periungual
area then examine with ...
Source Undetermined

Source Undetermined

15
Source Undetermined

16
Source Undetermined

17
Raynaud Phenomenon
•  Treatment
–  Avoidance strategies
•  Cold, nicotine, sympathomimetics (decongestants,
diet pills, he...
Raynaud Phenomenon
•  Severe ischemia
–  Warm patient (body and digits)
–  Analgesics
–  Antiplatelet tx – aspirin
–  Vaso...
Reactive Arthritis (Reiter Syndrome)
•  Definition:
–  Arthritis following a preceding infection without
intra-articular p...
Reactive Arthritis
•  Clinical Presentation:
–  Male 15 – 35 y/o
–  Asymmetric, oligoarthritis (2-4 joints)
–  Usually inv...
Centers for Disease Control and Prevention, Wikimedia Commons

22
Source Undetermined

Source Undetermined

23
Reactive Arthritis
•  Classic triad of Reiter syndrome
–  Nongonococcal urethritis (Chlamydia)
–  Conjunctivitis / anterio...
Reactive Arthritis
•  Treatment:
–  NSAIDs
•  Naproxen 500 mg TID
•  Indomethacin 50 mg TID

–  Intra-articular glucocorti...
Rheumatoid Arthritis
•  Definition:
–  Chronic, symmetric polyarticular synovial joint
disease

•  Nonarticular & Systemic...
Source Undetermined

Source Undetermined

27
Source Undetermined

Source Undetermined

28
Rheumatoid Arthritis
•  Cricoarytenoid joint
–  Dysphonia, hoarseness, stridor
–  If fix in closed position could require ...
Rheumatoid Arthritis
•  Pulmonary
–  Pleural effusion *
–  Interstitial fibrosis
–  Pulmonary nodules

•  Cardiac
–  Peric...
Rheumatoid Arthritis
•  Renal
–  Focal glomerulonephropathy
–  Drug toxicity from treatment *

•  Vasculitis
–  Distal inf...
Systemic Sclerosis - Scleroderma
•  Definition:
–  Disease process characterized by progressive
fibrosis, vascular abnorma...
Systemic Sclerosis
•  Clinical Presentation:
–  Often initial signs are a thickening, hardening
of the skin, usually finge...
Systemic Sclerosis
•  Skin
–  Sclerodactyly
–  Telangiectasias
–  Digital ulcers
–  Calcinosis
–  Raynaud phenomenon

34
In scleroderma, the abnormal build-up of fibrous tissue in the skin can
cause the skin to tighten so severely that the fin...
Telangiectasia

Kerry J, Flickr

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

36
Jmh649, Wikimedia Commons

37
Source Undetermined

38
Source Undetermined

39
Systemic Sclerosis
•  Pulmonary
–  Most common cause of death
–  Interstitial lung disease
•  Alveolitis leading to eventu...
Systemic Sclerosis
•  Renal
–  Renal crisis
•  Acute onset of renal failure
•  Proteinuria and microscopic hematuria
•  Ab...
Lipothymia, Wikimedia Commons

42
Source Undetermined
Source Undetermined

43
Systemic Sclerosis
•  Treatment
–  Generally immunosuppressive therapy
–  Based on specific organ system involved
–  Emerg...
Systemic Lupus Erythematosus
•  Definition:
–  Chronic autoimmune disease characterized by
presence of autoantibodies with...
SLE
•  Clinical Presentation:
–  Female > Male 10 : 1
–  Typical presentation 21 – 45 yrs of age
–  African American > Cau...
Source Undetermined

Source Undetermined

47
Source Undetermined
SLE
•  Pulmonary
–  Pleurisy, effusion, interstitial lung disease,
pulmonary hypertension
–  “lupus lung” – alveolar hemor...
SLE
•  Neurologic
–  Cognitive defects, cephalgia, seizures,
peripheral neuropathies (stocking / glove),
psychosis, stroke...
SLE
•  Treatment:
–  Immunosuppression
•  Glucocorticoids
•  Methotrexate, cyclophosphamide, azathioprine,
mycophenolate, ...
Polymyalgia Rheumatica
•  Definition:
–  Rheumatic condition frequently associated
with giant cell (temporal) arteritis

•...
Polymyalgia Rheumatica
•  Clinical Presentation:
–  Age 50 or older at onset
–  Bilateral aching and morning stiffness whi...
Polymyalgia rheumatica

Original Image:
Modified Image

Twisp, Wikimedia Commons
Lena Carleton, University of Michigan

53
Giant Cell Arteritis

Source Undetermined

54
Polymyalgia Rheumatica
•  treatment:
–  Prednisone 10 – 20 mg / day
•  (compared to 60 mg / day dose for GCA)

–  Rapid re...
Polymyositis / Dermatomyositis
•  Definition:
–  Idiopathic inflammatory myopathies

•  Etiology:
–  Genetic component wit...
Polymyositis / Dermatomyositis
•  Clinical Presentation:
–  Muscle weakness
•  Onset is insidious
•  Gradually worsening o...
Madhero88, Wikimedia Commons

Madhero88, Wikimedia Commons

58
Polymyositis / Dermatomyositis
–  Heliotrope rash
•  Violaceous eruption on upper eyelids, often with
swelling

–  Shawl s...
Madhero, Wikimedia Commons

60
Madhero88, Wikimedia Commons

61
Source Undetermined

Source Undetermined

62
Polymyositis / Dermatomyositis
•  Diagnostic testing
–  Elevated CK, LDH, aldolase, aminotransferases

•  Increased incide...
Vasculitides
•  Large vessel
–  Takayasu arteritis
–  Giant cell (temporal) arteritis

•  Medium vessel
–  Polyarteritis n...
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GEMC: Collagen Vascular Disease: Considerations for Emergent Management: Resident Training

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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/.

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GEMC: Collagen Vascular Disease: Considerations for Emergent Management: Resident Training

  1. 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. 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. 3. Collagen Vascular Disease Considerations for Emergent Management Joseph H. Hartmann, D.O. 3
  4. 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. 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. 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. 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. 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. 9. National Heart, Lung, and Blood Institute, Wikimedia Commons 9
  10. 10. Intermedichbo, Wikimedia Commons 10
  11. 11. Niklas D, Wikimedia Commons 11
  12. 12. Raynaud Phenomenon •  Relative temperature shifts may be provocative •  General body chill can trigger •  Fear / anxiety can trigger 12
  13. 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. 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. 15. Source Undetermined Source Undetermined 15
  16. 16. Source Undetermined 16
  17. 17. Source Undetermined 17
  18. 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. 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. 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. 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. 22. Centers for Disease Control and Prevention, Wikimedia Commons 22
  23. 23. Source Undetermined Source Undetermined 23
  24. 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. 25. Reactive Arthritis •  Treatment: –  NSAIDs •  Naproxen 500 mg TID •  Indomethacin 50 mg TID –  Intra-articular glucocorticoids –  Expect resolution 3 – 12 months 25
  26. 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. 27. Source Undetermined Source Undetermined 27
  28. 28. Source Undetermined Source Undetermined 28
  29. 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. 30. Rheumatoid Arthritis •  Pulmonary –  Pleural effusion * –  Interstitial fibrosis –  Pulmonary nodules •  Cardiac –  Pericarditis * –  Pericardial effusion 10% –  Myocarditis –  CAD – sudden death, MI * 30
  31. 31. Rheumatoid Arthritis •  Renal –  Focal glomerulonephropathy –  Drug toxicity from treatment * •  Vasculitis –  Distal infarcts, ulcerations, gangrene •  CNS –  Generally spared 31
  32. 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. 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. 34. Systemic Sclerosis •  Skin –  Sclerodactyly –  Telangiectasias –  Digital ulcers –  Calcinosis –  Raynaud phenomenon 34
  35. 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. 36. Telangiectasia Kerry J, Flickr Dilation of small vessels and capillaries cause flat red marks to appear on the skin 36
  37. 37. Jmh649, Wikimedia Commons 37
  38. 38. Source Undetermined 38
  39. 39. Source Undetermined 39
  40. 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. 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. 42. Lipothymia, Wikimedia Commons 42
  43. 43. Source Undetermined Source Undetermined 43
  44. 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. 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. 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. 47. Source Undetermined Source Undetermined 47 Source Undetermined
  48. 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. 49. SLE •  Neurologic –  Cognitive defects, cephalgia, seizures, peripheral neuropathies (stocking / glove), psychosis, stroke (antiphospholipid antibody syndrome) •  Musculoskeletal –  Arthritis, atrophy, tendon rupture 49
  50. 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. 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. 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. 53. Polymyalgia rheumatica Original Image: Modified Image Twisp, Wikimedia Commons Lena Carleton, University of Michigan 53
  54. 54. Giant Cell Arteritis Source Undetermined 54
  55. 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. 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. 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. 58. Madhero88, Wikimedia Commons Madhero88, Wikimedia Commons 58
  59. 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. 60. Madhero, Wikimedia Commons 60
  61. 61. Madhero88, Wikimedia Commons 61
  62. 62. Source Undetermined Source Undetermined 62
  63. 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. 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
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