Reu 6 - Systemic Disorders Flashcards

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1
Q

What are the diagnostic criteria for Systemic Lupus Erythematosus?

A

There has to be 4 of the 11 diagnostic criteria.
1.Skin disorders (malar rash,discoid rash,photosensitivity,painless oral ulcer).2,Inflammatory disorder(Arthritis[non-erosive, 2 joints], Serositis[pleuritis, pericarditis], (+)ANA). 3. Organ system Disorder (Renal[proteinuria, cellular casts],Neuro[seizures, psychosis],Heme[hemolytic anemia, leukopenia, lymphopenia,thrombocytopenia],Immune[antiphsopholipid Ab(+), anti-dsDNA, anti-Smith, false (+) VDRL]).

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2
Q

What type of lesions do we see in Lupus nephritis?

A

[Wire lupus] Wire loop lesions.

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3
Q

What labs are ordered for Lupus?

A

(+)ANA (antinuclear antibodies), Anti-dsDNA Ab (more specific for lupus), Anti-Smith Ab, Anti-histone Ab (Shows up more on drug induced lupus).

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4
Q

What diseases can have (+) ANA?

A

SLE, Sjogren syndrome, Scleroderma, Polymyositis and dermatomyositis, Rheumatoid arthritis, Juvenile idiopathic arthritis, Mixed connective tissue disease.

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5
Q

What drugs are associated with drug-induced Lupus?

A

[SHIPP]

Sulfonamides, Hydralazine, Isoniazid, Phenytoin, Procainamide.

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6
Q

What are the treatments for Lupus?

A

Steroids, NSAIDs, Hydroxychloroquine, Cyclophosphamide.

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7
Q

What is Scleroderma?

A

AKA Progressive systemic sclerosis, it causes fibrosis and excess collagen deposition. Skin (tight, shiny (no wrinkles). There are two main types: Diffused Scleroderma, CREST syndrome.

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8
Q

Describe Diffuse Scleroderma.

A

Diffuse Scleroderma: Diffuse, widespread sclerosis, it progresses rapidly, involves the visceral organs. It has Anti-DNA topoisomerase I (Anti-Scl-70) antibodies

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9
Q

Describe CREST Scleroderma.

A

Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias. There is limited skin involvement, less visceral involvement, Progresses less rapidly. It has anti-centromere antibodies.

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10
Q

What are the symptoms of Sjogren Syndrome?

A

Dry eyes (dryness, conjunctivitis, sensation of sand in the eyes), Dry mouth (dysphagia, difficulty swallowing), Arthritis. Can cause Parotid enlargement, increased risk of cavities, Increased risk of B cell lymphoma, autoantibodies (Anti-SSA [Anti-Ro], Anti-SSB [Anti-La]), (+) rheumatoid factor., Women over the age of 40.

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11
Q

What are Sicca symptoms?

A

Dry eyes, Dry mouth, Nasal dryness, Vaginal dryness, Chronic bronchitis, Reflux esophagitis.

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12
Q

What are the symptoms of Duchenne muscular dystrophy?

A

X-linked frameshift mutation causing a deletion of the dystrophin gene. Accelerated muscle breakdown causing elevated CK. Presents very early in life, starting with weakness in pelvic girdle muscles, progressing superiorly. They get pseudohypertrophy of the calf muscles due to replacement of muscle with fibrofatty tissue. They use Gower’s maneuver to stand up.

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13
Q

What are the symptoms of Becker muscular dystrophy?

A

Caused by a mutated dystrophin gene, it is less severe than DMD. It has a later onset: adolescence, early adulthood.

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14
Q

What are the symptoms of Polymyalgia Rheumatica?

A

Pain and stiffness in the shoulders and hips, malaise, weight loss, fever, but no muscle weakness. Average age of onset is 70, women and associated with temporal arteritis.

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15
Q

What are the lab findings and treatment for Polymyalgia Rheumatica?

A

Lab is going to find elevated ESR and normal CK. Treatment is steroids.

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16
Q

What is the most likely cause of proximal muscle weakness versus distal weakness?

A

Proximal muscle weakness is caused by muscle problems while distal weakness is caused by neurologic problems.

17
Q

What is pathophysiology of Polymyositis? How do we diagnose it?

A

CD8 T cell-induced injury to the muscle fibers often involving: shoulders and pelvic girdle. We diagnose it with Muscle biopsy (inflammation). There is going to be elevated CK and aldolase. There is also (+) ANA and (+) anti-Jo-1 antibodies.

18
Q

What is Dermatomyositis?

A

It is muscle inflammation with skin involvement (malar rahs, purple helo on eyelids, Gotrin papules, Shawl sign (rashes over the shoulder), V sign (rashes on the front chest in form of V), mechanic’s hand).

19
Q

What is Fibromyalgia?

A

Excess muscular tenderness in 11 of 18 particular sites (trigger points, palpating it with a specified amount of pressure). Symptoms are chronic generalized pain, fatigue, sleep disturbances, HA, cognitive difficulty, mood disturbances. 30% will also have depression and/or anxiety.

20
Q

What is the treatment for Fibromyalgia?

A

Pharmacological:FDA-approved: pregabalin, milnacripan. Traditional: amitriptyline, low-dose analgesic, fluoxetine. Non-pharma: Reassurance that it is a real disease and that it is benign, exercise and stretching, sleep, relaxation techniques, stress reduction.

21
Q

RFF: Anti-Smith and anti-dsDNA antibodies.

A

Lupus.

22
Q

RFF: Anti-histone Ab.

A

Drug induced lupus.

23
Q

RFF: Anti-centromere Ab.

A

CREST Scleroderma.

24
Q

RFF: Anti-topoisomerase Ab.

A

Diffuse systemic scleroderma.

25
Q

RFF: Anti-Jo-1 Ab.

A

Polymiositis.

26
Q

RFF: Anti-Ro Ab.

A

Sjogren syndrome.

27
Q

RFF: Facial rash and Raynoud phenomenon in a young woman.

A

Lupus.

28
Q

RFF: Arthritis, dry mouth, dry eyes.

A

Sjogren syndrome.

29
Q

RFF: Most common cause of death in SLE.

A

Lupus nephropathy/nephritis.

30
Q

What are the symptoms of Systemic Lupus Erythematosus?

A

Nonspecific systemic symptoms (fatigue, weight loss, fever), lymphadenopathy, Splenomegaly, Libman-Sacks endocarditis (sterile vegetations), Raynoud phenomenon.