Connective tissue diseases

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