This document summarizes several connective tissue diseases including polymyositis, systemic necrotizing vasculitis, polymyalgia rheumatica, giant cell arteritis, ankylosing spondylitis, Reiter's syndrome, and Marfan syndrome. For each disease, the document describes characteristics such as symptoms, risk factors, complications, diagnostic tests, and treatment options including drug and supportive therapies.

1. Connective Tissue Diseases
Medical Surgical Nursing

2. Connective Tissue Diseases
 Polymyositis
 Systemic Necrotizing Vasculitis
 Polymyalgia Rheumatica
 Giant Cell Arteritis
 Ankylosing Spondylitis
 Reiter’s Syndrome
 Marfan Syndrome

3. Polymyositis

4. Polymyositis
 Diffused inflammation
 Skeletal muscle

5. Polymyositis
 Weakness
 Atrophy
 Rash (dermatomyositis)
 Women > Men
 30-60 yrs of age

6. Polymyositis
 Polyarthritis
 Polyarthralgia
 Rynaud’s phenomenon

7. Polymyositis
 Drug Therapy:
 Steroids (high-doses)
 Immunosuppressive agents
 Supportive Care
 Nutrition !!!

8. Polymyositis
 Blood tests: elevated levels creatinine kinase (CK)
and aldolase
 Electromyography: pattern of electrical activity can
confirm a muscle disease.
 MRI: assess inflammation over a large area of
muscle.
 Muscle biopsy

9. Systemic Necrotizing Vasculitis

10. Systemic Necrotizing Vasculitis
 Arteritis
 Ischemia

11. Systemic Necrotizing Vasculitis
 Types:
 Polyarteritis
 Hypersensitivity Vasculitis (allergic)
 Drugs
 Infections
 Tumors
 Takayasu’s Arteritis (aortic arch syndrome)
 Pulseless disease
 Women in 20’s
 Japanese (most often)
 Cerebral ischemia (visual changes, syncope, vertigo)

12. Systemic Necrotizing Vasculitis
 Drug Therapy:
 Steroid (Prednisone)
 Immunosuppressive Agents

13. Polymyalgia Rheumatica

14. Polymyalgia Rheumatica
 Clinical syndrome
 Women > 50 yrs of age
 Joints:
 Neck
 Shoulder
 Hip

15. Polymyalgia Rheumatica (PMR)
 Stiffness (worse in morning)
 Weakness
 Aching proximal musculature (pelvic & shoulder
girdles)
 Low grade fever
 Arthralgia
 Fatigue
 Wt. loss
 Drug Therapy:
 Corticosteroids
 Calcium
 Vitamin D

16. Giant Cell Arteritis

17. Giant Cell Arteritis
 Temporal arteritis
 Genetic predisposition (HLA-DRBI)

18. Giant Cell Arteritis
 Fatigue
 Fever
 Wt. loss
 Night sweats
 Erthyema
 Pain
 Swelling
 Tenderness
 Diplopia
 Amaurosis fugax (temp. vision loss 1 eye)

19. Giant Cell Arteritis
 Drug Therapy:
 Corticosteroids (high doses)
 Calcium
 Vitamin D

20. Giant Cell Arteritis
 Complications:
 Blindness
 Aortic aneurysm
 Stroke

21. Giant Cell Arteritis
 Blood test
 Biopsy
 MRA
 Doppler Ultrasound
 PET Scan

22. Ankylosing Spondylitis

23. Ankylosing Spondylitis
 Marie-Strumpell Disease
 Rheumatoid Spondylitis

24. Ankylosing Spondylitis
 Vertebral column
 Spinal deformities
 At any age
 Men < 40 years

25. Ankylosing Spondylitis
 Iritis
 Arthritis
 Arthralgia
 Malaise
 Wt. loss
 Respiratory compromise

26. Ankylosing Spondylitis
 Drug Therapy:
 Anti-inflammatory agents
 Heat application
 DMARD’s (methotrexate)
 Biological Response Modifiers (BRM’s)
 TNF Inhibitors (infliximab Remicade)
 sulfasalazine (Azulfidine)

27. Reiter’s Syndrome

28. Reiter’s Syndrome
 Associated with HLA-B27 antigen
 Affect both male & female

29. Reiter’s Syndrome
 Triad:
 Arthritis
 Conjunctivitis
 Urethritis

30. Reiter’s Syndrome
 Balanitis circinata
 Skin lesions
 Both significant 4 confirmation of diagnosis

31. Reiter’s Syndrome
 Drug Therapy:
 Antibiotics (minocycline, Augmentin)
 NSAID’s
 Physical therapy

32. Marfan Syndrome

33. Marfan Syndrome
 Autosomal dominant
 Defect in extracellular microfibrils

34. Marfan Syndrome
 Abnormalities:
 Skeletal
 Ocular
 Cardiopulmonary
 Central nervous system

35. Marfan Syndrome
 Excessively tall
 Elongated hands
 Elongated feet
 Chest sinks in or sticks out
 Flexible joints
 Flat feet
 Crowded teeth
 Stretch marks
 Scoliosis
 Subluxation of lens (bilateral)
 Mitral valve prolapse w/regurgitation
 Aortic aneurysm w/regurgitation

36. Marfan Syndrome
 Management:
 Palliative & Preventive
 Cardiovascular meds
 Orthopedic surgery

37. Marfan Syndrome
 Chest Xray
 ECG
 Echocardiogram
 TEE
 MRI
 CT Scan
 Slit lamp eye exam

38. Thank You….
Sharon King, RN