005. Autoimmune connective tissue disease, polymyositis and dermatomysositits

Question 1

What are autoimmune connective tissue diseases?

Provide some examples

Answer 1

Overlapping connective tissue diseases that often require immunosuppresive therapies. Consider as a differential in unwell patients with multi-organ involvement

SLE
Sjorgens
Systemic scelrosis

Question 2

What are the features of systemic sclerosis?

What antibodies are associated with systemic sclerosis?

Answer 2

Scleroderma (skin fibrosis)

Internal organ fibrosis

microvascular abnormalities

90% ANA positive, 30-40% have anticentromere antibodies

Question 3

What are the two types of skin disease in systemic sclerosis?

Answer 3

Limited- Involves the face, hands and feet, CREST syndrome, pulmonary hypertesnion. High levels of anticentromere antibodies.

Diffuse- Can involve the whole body, need to control BP very well

Question 4

What is the management for systemic sclerosis?

Answer 4

Currently no cure, IV cyclophosphamide is used for organ involvement or progressive skin disease

Treat with ACE-I or ARB to decrease risk of renal crisis

Question 5

What is relapsing polychondritis?

Answer 5

Rare condition with cartilage inflammation and destruction

Floppy ears , affects nasal septum, tracheobronchial tree and joints

Question 6

What are polychondritis’ associations

What are the treatments?

Answer 6

Aortic valve disease, polyarthritis, vasculitis

Steriods, DMARDS, CPAP/ for airway involvement tracheostomy

Question 7

What is polymysositis and dermatomysositis?

Answer 7

Rare conditions charecterized by insidious onset of progressive symmetrical proximal muscle weakness.

Muscle weakness may cause dysphagia, dysphonia or respiratory weakness

Question 8

What are the signs of dermatomyositis?

Answer 8

Myositis (muscle inflammation)

MAcular rash

Lilaic-purple rash on eyelids

Roughened red papules n the knuckles (gottron’s)

Question 9

What are the extra-muscular signs of the mysoitis’

Answer 9

Fever, 
arthralgia, 
Raynaud's, 
interstitial lung fibrosis, 
myocardial invovlement

Question 10

What are your differentials for polymyosistis and dermatomysositis

Answer 10

HIV

Drugs reaction (peniclamine, satins, chloroquine)

Carcinomatous myositis

Muscular dystropy

Endocrine/metabolic myopathy

Rhabdomyolysis

Question 11

What is the management of dermatomyositis and polymyositis?

Answer 11

Start pred,

immunosuppresive

cytotoxics

Hydroxychloroquine/topical tacrolimus for skin diseases