Unit 3 - CVD Pt 3

Question 1

Dermatomyositis and Polymyositis

Answer 1

autoimmune disease

if it affects the muscles but not the skin, it is called poly

Question 2

Dermatomyositis comes in 2 flavors

Answer 2

1. Classical Dermatomyositis:
   Skin plus skeletal muscle (trunk, proximal limbs, diaphragm)
2. Amyopathic Dermatomyositis:
   Skin but no muscle involvement

Question 3

Classical Dermatomyositis def and ratio

Answer 3

Autoantibodies attack skin & skeletal muscles

Females: Males=2:1

Question 4

Dermatomyositis
clinical features (something characteristic)

Answer 4

Skin: Scaly, spotty rash
Trunk and Proximal Extremities are weak
Facial Edema and Periorbital Discoloration (Heliotrope** Rash -violet colored flowering plant)
Scaly nodules over dorsum of MCP/IP joints - Grotten’s Sign
Dysphagia
Pulmonary Failure

Question 5

Dermatomyositis age and diagnosis

Answer 5

20% > 40 years Old
Have an occult Malignancy
Esp. Lung cancer, stomach and pancreatic cancer, ovarian cancer, non-Hodgkin’s lymphoma
ESR/CRP is Elevated
CK (CK-MM) isoenzyme Aldolase (converts glucose to energy) is elevated
Found in skeletal muscles
Aspartate aminotransferase (AST) elevated
ANA elevated (30% of cases)

Question 6

mixed collagen vascular disease (MCVD)

Answer 6

undifferentiated connective tissue disorder with anti-U1-RNP Ab) and raynaud’s

evolves to become one of several other connective tissue disorders or an overlap syndrome

MCTD
Overlapping clinical features of systemic lupus erythematosus (SLE), scleroderma and dermatomyositis
Major diagnostic signs from these CVD’s
Raised levels of anti-U1-RNP Antibody
80% of the disease occurs in females

Question 7

Polyarteritis Nodosa

Answer 7

30% association with Hepatitis B or Hepatitis C
Pervasive Vasculitis (Multiple arteries are involved)
Affects small/medium arteries
Pulmonary vessels-spared
Thickening of the arterial wall

Question 8

Classical Polyarteritis Nodosa s/s

Answer 8

Constitutional Symptoms (fever, fatigue, weight loss, loss of appetite) 
Renal Failure, Heart Attack, Stroke
Hypertension
Neurological Signs
Headaches
Memory Loss
Dementia

Question 9

Pathology of Polyarteritis Nodosa

Answer 9

Inflammation of vessel wall
Nodule formation and occlusion
Necrosis of vessel wall with aneurysm

Question 10

Blood markers Polyarteritis Nodosa

Answer 10

Anti-neutrophil cytoplasmic antibodies (ANCAs)
Antinuclear Antibodies (ANA)

Question 11

Varients of Polyarteritis Nodosa

Answer 11

Focal (Area Specific)
Temporal Arteritis
Wegener’s Granulomatosis
Pulseless Disease

Question 12

Temporal arteritis

Answer 12

Also called Giant Cell Arteritis, most common form
Elderly Women > Men
Affects the Superficial Temporal & Intracranial Arteries

Question 13

s/s of Temporal arteritis

Answer 13

Headaches
Dementia
Visual Loss
Constitutional Symptoms
Redness/Pain-Involved Vessel
Elevated inflammatory markers (ESR & CRP)

Polymyalgia Rheumatica-Pain/stiffness upper back, shoulders, & neck
Some have discomfort in the UE’s, Hips, and LE’s

Question 14

Wegener’s Granulomatosis

Answer 14

Polyarteritis with lesions-affects primarily the upper respiratory system, lungs, mouth, pharynx, and kidneys

Polyarteritis-causes necrotic changes 
Respiratory System
Bleeding & Mucosal Loss
Upper respiratory infections
Pulmonary infections
Renal Polyarteritis with reduced blood flow
Kidneys-chronic renal failure                                                               
Elevated blood pressure
holes on palate
scarred kidneys

Question 15

Pulseless Disease (Takayasu’s Arteritis)

Answer 15

Affects Aortic Arch Branches
Onset < 30 Years of Age
Female (Asian) Heritage

Question 16

Other Arthropathies

Answer 16

Ankylosing Spondylitis (Marie-Strumpel)
Psoriatic Arthritis
Reiter’s Syndrome (Reactive Arthritis)-STD
Enteropathic Arthritis (Inflammatory Bowel Disease-Crohn’s Disease)

Question 17

Ankylosing Spondylitis: Marie-Strumpel Disease

ratio and blood marker

Answer 17

8:1 Males to Females
 Inflammatory, Progressive, Chronic
Affects: 
Lumbar Spine
SI Joint
Vertebral/Rib Junctions (Vertebrocostal Joints)
Cervical Spine

Blood markers
Positive for HLA B27 antigen*
Present in 90% of patients
*Protein found on the surface of white blood cells

Question 18

Psoriasis and Psoriatic Arthritis

Answer 18

Psoriasis-chronic, non-contagious autoimmune disease (males:females=1:1)
Affects the skin (100%)
-Causes red scaly plaques to appear on the skin
-Skin takes on a silvery-white appearance (frosty)

-chronic, non-contagious autoimmune disease
Affects the joints (10-30%)
-Pain and swelling

Question 19

Rieter’s Syndrome (Reactive Arthritis)

Answer 19

Autoimmune Disorder secondary to infection

STD-Chlamydia trachomatis
Most common STD in the US
Generally occurs in males

Question 20

Enteropathic Arthritis (Crohn’s Disease)

Answer 20

Crohn’s Disease: Inflammatory Autoimmune
Bowel Disease
May cause a Secondary Arthritis
Enteropathic arthritis

Question 21

Fibromyalgia

Answer 21

Muscular Aches and Pains
Widespread Stiffness
Soft Tissue Swelling
Diffuse Muscle Spasms
 Potential Tender Points-18
 Pain - 11 of 18 tender 		 	points*
Fibromyalgia Questionnaire*

Question 22

Chronic Fatigue Syndrome

Answer 22

Fatigue must be severe enough to decrease ability to participate in ordinary activities by at least 50%.
Must last 6 months or longer

No reliable blood tests or biomarkers
Elevated Alpha-Melanocyte Stimulating Hormone (MSH)

Question 23

Gout

Answer 23

Elevated Serum Uric Acid (from Purines)
95% of Cases are MEN
Urate Crystals Form in Joints
Tophi-Large Amounts of Uric Acid

Question 24

primary vs secondary gout

Answer 24

Primary: Inborn error of metabolism (Genetic)
Secondary: due to a diet or disease 
renal failure
 AML
leukemia