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Connective Tissue Diseases
Medical Surgical Nursing
Connective Tissue Diseases
 Polymyositis
 Systemic Necrotizing Vasculitis
 Polymyalgia Rheumatica
 Giant Cell Arteritis
 Ankylosing Spondylitis
 Reiter’s Syndrome
 Marfan Syndrome
Polymyositis
Polymyositis
 Diffused inflammation
 Skeletal muscle
Polymyositis
 Weakness
 Atrophy
 Rash (dermatomyositis)
 Women > Men
 30-60 yrs of age
Polymyositis
 Polyarthritis
 Polyarthralgia
 Rynaud’s phenomenon
Polymyositis
 Drug Therapy:
 Steroids (high-doses)
 Immunosuppressive agents
 Supportive Care
 Nutrition !!!
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
Systemic Necrotizing Vasculitis
Systemic Necrotizing Vasculitis
 Arteritis
 Ischemia
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)
Systemic Necrotizing Vasculitis
 Drug Therapy:
 Steroid (Prednisone)
 Immunosuppressive Agents
Polymyalgia Rheumatica
Polymyalgia Rheumatica
 Clinical syndrome
 Women > 50 yrs of age
 Joints:
 Neck
 Shoulder
 Hip
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
Giant Cell Arteritis
Giant Cell Arteritis
 Temporal arteritis
 Genetic predisposition (HLA-DRBI)
Giant Cell Arteritis
 Fatigue
 Fever
 Wt. loss
 Night sweats
 Erthyema
 Pain
 Swelling
 Tenderness
 Diplopia
 Amaurosis fugax (temp. vision loss 1 eye)
Giant Cell Arteritis
 Drug Therapy:
 Corticosteroids (high doses)
 Calcium
 Vitamin D
Giant Cell Arteritis
 Complications:
 Blindness
 Aortic aneurysm
 Stroke
Giant Cell Arteritis
 Blood test
 Biopsy
 MRA
 Doppler Ultrasound
 PET Scan
Ankylosing Spondylitis
Ankylosing Spondylitis
 Marie-Strumpell Disease
 Rheumatoid Spondylitis
Ankylosing Spondylitis
 Vertebral column
 Spinal deformities
 At any age
 Men < 40 years
Ankylosing Spondylitis
 Iritis
 Arthritis
 Arthralgia
 Malaise
 Wt. loss
 Respiratory compromise
Ankylosing Spondylitis
 Drug Therapy:
 Anti-inflammatory agents
 Heat application
 DMARD’s (methotrexate)
 Biological Response Modifiers (BRM’s)
 TNF Inhibitors (infliximab Remicade)
 sulfasalazine (Azulfidine)
Reiter’s Syndrome
Reiter’s Syndrome
 Associated with HLA-B27 antigen
 Affect both male & female
Reiter’s Syndrome
 Triad:
 Arthritis
 Conjunctivitis
 Urethritis
Reiter’s Syndrome
 Balanitis circinata
 Skin lesions
 Both significant 4 confirmation of diagnosis
Reiter’s Syndrome
 Drug Therapy:
 Antibiotics (minocycline, Augmentin)
 NSAID’s
 Physical therapy
Marfan Syndrome
Marfan Syndrome
 Autosomal dominant
 Defect in extracellular microfibrils
Marfan Syndrome
 Abnormalities:
 Skeletal
 Ocular
 Cardiopulmonary
 Central nervous system
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
Marfan Syndrome
 Management:
 Palliative & Preventive
 Cardiovascular meds
 Orthopedic surgery
Marfan Syndrome
 Chest Xray
 ECG
 Echocardiogram
 TEE
 MRI
 CT Scan
 Slit lamp eye exam
Thank You….
Sharon King, RN

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