Scleroderma Inflammatory Myositis

Question 1

• Connective tissue disease characterized by sclerodactyly (skin thickening and tightening (leather and hind bound)
• localized scleroderma: confined to the skin and subcutaneous tissue

Answer 1

Scleroderma

Question 2

• cold-related episodic vasospatic disorder leading to decreased blood flow in the digits
• structural damage to the microvasculature
• 2 color change: white (pallor)–> blue (cyanosis)—> red (hyperemia)
• can be normal in young women

Answer 2

Raynauds

Question 3

An autoimmune disease characterized by:

• Sclerodactyly
• raynauds phenomenon
• internal organ involvement (lungs, gastrointestinal tract, musculokeletal system and kidneys, +ANA)

Answer 3

Systemic sclerosis

Question 4

who does systemic sclerosis affect?

Answer 4

• twice as common in african americans
• african americans are more likely to have earlier onset of disease, diffuse cutaneous involvement and aggressive lung disease

Question 5

clinical presentation of systemic sclerosis?

Answer 5

• no two individuals with systemic sclerosis are 100% alike
• formal categorization scheme by degree of cutaneous involvement
• GI, Pulm, and/or renal manifestations maybe present and cause significant morbidity
• within each organ system, various manifestations may be present (and hard to remember!)
• manifestations may be attributed to either vasculopathy or fibrosis

Question 6

treatment of systemic sclerosis?

Answer 6

• targeted to disease manifestations
• monitor for new symptoms
• almost entirely focused on managing individual symptoms
• immunosuppressive therapy has limited role (only in cutaneous and pulmonary manifestations)

Question 7

• face and distal portion of the extremities (distal to elbows and knees)
• CREST (calcinosis, raynauds phenomenon, esophageal dysmotility, sclerodactyly, telangectasias)
• associated with a centromere antibody
• risk of pulmonary arterial hypertension

Answer 7

Limited cutaneous systemic sclerosis

Question 8

• Distal and proximal body regions (e.g, neck, arms, trunk and thigh)
• associated with Scl 70 antibody
• risk of interstitial lung disease

Answer 8

Diffuse cutaneous systemic sclerosis

Question 9

• No skin thickening
• all the same systemic effects, skin is spared

Answer 9

sclerosis sine scleroderma

Question 10

treatment of raynauds phenomenon?

Answer 10

first line therapy

• dihydropyridine calcium channel blockers- nifedipine, amlodipine

Second line

• other vasodilators
• phosphodiesterase 5 inhibitors (2nd line sildenafil)

Question 11

treatment of acute digital ischemia

Answer 11

• Intravenous prostaglandins
• endothelin-I receptor antagonists

Question 12

• Myositis: skeletal muscle inflammation
• fiver major idopathic forms: polymyositis, dermatomyositis, anti-synthetase, necrotizing, inclusion body myositis

Answer 12

Inflammatory myositis

Question 13

• painless symetric proximal muscle weakness (shoulders and thighs)
• fatigue, low appetite, arthralgias, weight loss over weeks to months
• dysphagia due to oropharyngeal muscle weakness
• dyspnea due to respiratory muscle weakness, interstitial lung disease

Answer 13

Dermatomyositis, and polymyositis

Question 14

what can be seen with dermatomyositis?

Answer 14

helitrope rash
gottron’s papules
gottron’s sign
V sign
shawl sign

Question 15

• presence of antibody directed against tRNA
• most typical Jo-1
• • ANA antibodies
• syndrome includes inflammatory myopathy, interstitial lung disease, “mechanic hands”, inflammatory arthritis, and raynaud phenomenon

Answer 15

Anti-Synthetase syndrome

Question 16

• Painful symmetric muscle weakness
• fatigue, low appetite, arthralgias, weight loss over weeks to months
• extensive necrosis on biopsy
• typically with +anti-hmg-coA or SRP autoantibodies
• history of statin use or red rice yeast supplement

Answer 16

Immune-mediated necrotizing myositis

Question 17

Slowly progressive muscle weakness over several years

• asymmetric, painless
• proximal and distal (wrist and finger flexors, quadriceps)
• oropharyngeal weakness leading to dysphagia
• abscence of cutaneous or pulmonary manifestations
• may also cause neuropathy- sensory deficits including numbness and tingling

Answer 17

inclusion body myositis

Question 18

lab results for myositis?

Answer 18

• increased ESR and CRP
• Increased serum creatinine kinase (2 to 100x in dermato/polymyositis; normal to < 10x in inclusion body myositis)
• increased muscle and tissue enzymes (AST, ALT, aldolase, and LDH

Question 19

work up for myositis?

Answer 19

• electromyography- can help determine to the location of muscle involvement and detect abnormalities indicative of inflammatory myopathy
• nerve conduction study- may demonstrate neuropathy in inclusion body myositis
• MRI- can show areas of edema, or alternative causes (abscess, DVT, trauma, etc.)

Question 20

muscle biopsy of dermato/polymyositis would show?

Answer 20

nonspecific signs of myopathy (degeneration, regeneration, atrophy) and an inflammatory infiltrate

Question 21

Muscle biopsy of inclusion body myositis may show?

Answer 21

• inflammatory infiltrate
• rimemd vacuoles
• triangular or angulated cells with fiber type grouping (due to denervation)

Question 22

• treatment of dermato/polymyositis/necrotizing myositis?

Answer 22

• high dose corticosteroids
• methotrexate, azathioprine, and mycophenolate
• IV immunoglobulin (IVIG) for refractory
• hydroxychloroquine or IVIG for skin disease in dermatomyositis

Question 23

treatment of inclusion body myositis?

Answer 23

• no curative treatment
• physical therapy and nutritional support

Question 24

• swollen hands, raynaud’s phenomenon, arthritis, and mysositis
• ribonucleoprotein (+RNP) antibody

Answer 24

Mixed connective tissue disease

Question 25

Diagnosis of mixed connective tissue disease?

Answer 25

The presence of mixed features of connective tissue diseases (at least 1 feature in 2 or 3 diseases is required

• lupus: arthritis, malar rash, pericarditis or pleuritis, leukopenia or thrombocytopenia
• systemic sclerosis: sclerodactyly, pulmonary fibrosis, restrictive changes reduced diffusion capacity, esophageal dilation
• polymyositis: muscle weakness, elevated serum muscle enzymes, myositis on EMG

Question 26

Prognosis and management of MCTD

Answer 26

• Outcomes are highly dependent on manifestations, same drugs as used in other connective tissue diseases
• some individuals may develop more signs and symptoms specific for one of the other connective tissue disease over time

Question 27

• Diagnosed in individuals who have few clinical features seen in the connective tissue diseases
• most have non-organ threatening clinical features (dry eyes and mouth, alopecia, rash, arthralgias, arthritis (non-erosive) oral ulcers, raynauds etc)
• do not fit entirely into a specific CTD

Answer 27

Undifferntiated connective tissue disease

Question 28

treatment of UCTD?

Answer 28

• often treated with NSAIDs and mild immunomodulatory drugs (e.g, hydroxychloroquine)
• usually better prognosis
• 20% have symptoms subside/minimize over time
• 30% develop new signs and symptoms over time, leading to a diagnosis of a more specific connective tissue disease within 3 to 5 years
• usually better prognosis

Question 29

MCTD vs UCTD?

Answer 29

MCTD

• raynauds, arthralgias, puffy edema, myositis
• +RNP
• mix of lupus, myositis and scleroderma
• treat based on symptoms as you would other CTD manifestations

UCTD

• no set criteria
• mix of + ana or typical CTD features
• good prognosis
• use NSAIDs +/- HCQ