Autoimmune Rheum Disorders

Question 1

What pt population would you commonly see SLE?

Answer 1

Women childbearing age (20-30yo)

African, Caribbean descendant, or Hispanic (Mexican)

Question 2

What are pt w/ SLE at high risk of?

Answer 2

Premature atherosclerosis
Cardiovascular dz
Kidney disease

Question 3

Pathophysiology SLE

Answer 3

• Autoantibodies nuclear proteins
• Defective apoptosis –> illicit immune response
• Epigenetic changes (infection, sunlight, smoking) –> trigger immune response
• C4 and C1q deficiencies (low complement)
• 50% pt have growth factor antibodies (BlyS)
• Anything seen in lupus is a manifestation of immune response
• Double stranded DNA –> glomerulonephritits
• Immune complex –> cutaneous rash
• Against blood components –> anemia, neutropenia, thrombocytopenia
• Phospholipid –> thrombosis

Question 4

Most common presentation of Lupus

Answer 4

• Musculoskeletal complaint
• Joint pain
• Skin rash
• Anemia

Question 5

New SLE Sx

Answer 5

• Glomerulonephritis (edema face, brown urine, hematuria/protienuria, decrease urine output, HTN, nocturia, foamy urine)
• Hematologic disrdoers (Anemia, thrombocytopenia)
• Arthritis & Rash (butterfly rash, lupus profundus)
• Chest pain–pleurisy, pericarditis

Question 6

Pt has established SLE and becomes symptomatic. Generally, what can flares present as?

Answer 6

• worsening of previous sx
• emergence of new sx that they previously didnt have
• same dz with diff mechanism (ex. diff type of anemia)

-may also present w/ neuropsychiatric lupus (seizures, depresssion, mood changes)

Question 7

Can you solely diagnose Lupus based on clinical presentation?

Answer 7

NO!!

Dx is based on clinical presentation + lab abnormalities

Question 8

What labs are you looking for in a pt w/ lupus?

Answer 8

ANA positive ***
Anti-DS DNA

Anemia
Thrombocytopenia
Low complement

Question 9

How would you diagnose lupus nephritis?

Answer 9

Urine analysis

• Proteinuria >0.5g/d or 3+
• active urinary sediment (>5RBC/HCP or >5WBC/HFP)

Biopsy if renal failure suspected

Question 10

If you suspect neuropsychiatric dz in lupus pt, what imaging would you get?

Answer 10

MRI

Functional MRI

Question 11

What is the main tx of lupus? (acute lupus flares)

Answer 11

GLUCOCORTICOIDS

Prednisone is used to tx a lot of the sx (neprhitis, serositis, cutaneous lupus, hematologic, neuropsych sx)

Question 12

Generally, what can be given to a lupus pt to decrease their frequency of flares & improve their survival?

Answer 12

HYDROXYCHLOROQUINE (immunosuppressive)

Question 13

What can you can for a lupus pt w/ heart block?

Answer 13

IV Immunoglobulins (IVIG)

Question 14

Pt w/ lupus comes in w/ arthralgia and arthritis, what would you give them to manage their pain?

Answer 14

NSAID +/- Prednisone

Question 15

What glands in the body does sjogren syndrome target?

Answer 15

Exocrine glands

Question 16

What pt population is Sjogren syndrome commonly seen in?

Answer 16

Women (9x more than men)

4th-5th decade of life

Question 17

Pathogenesis of Sjogren Syndrome

Answer 17

• SLOWLY progressive autoimmune rheum disorder
• autoimmunity to epithelial tissues
• immune response directed to antigens (Fodrin, Ro, La)
• autoantibodies against –> Ro/SS-A and La/SS-B

Question 18

Presentation of Sjogren Sydnrome

Answer 18

• Xeriphthalmia (ocular dryness)
• Xerostomia (mouth dryness)
• high risk for corneal ulcer or perforation
• difficulty swallowing dry foods or speaking at length
• may experience oral burning
• suspecitable to new onset dental caries

***SWELLING OF PAROTID GLAND; BILATERAL

Question 19

How can you dx a pt you suspect has sjogren syndrome?

Answer 19

Schirmer’s Test –> eye tests to help dx keratoconjuncitivitis sicca

Mouth eval
Serum reaction w/ Ro/SS-A and/or La/SS-B autoantigen
Labial biopsy

Question 20

What is another name for Sjogren Syndrome?

Answer 20

Keratoconjunctivitis Sicca

mouth and eye dryness

Question 21

What is the number 1 thing you want to tx in sjogren is pt presents with it?

Answer 21

MALIGNANT LYMPHOMA –> ANTI-CD-20 + CHOP regimen

Question 22

Generally, how would you tx Sjogren?

Answer 22

Sx relief, no definitive tx

Dry eyes –> artificial tears
Corneal ulcer –> eye patch, boric acid ointment
Xerostomia–> water

stimulate secretions –> pilocarpine PO or cevimeline

Question 23

What drugs do you want to AVOID in Sjogren pt?

Answer 23

Diuretics
Antihypertensives
Anticholinergics
Antidepressants

Question 24

What is scleroderma described as?

Answer 24

Rare chronic & frequently progressive disorder characterized by diffuse fibrosis of skin & internal organs w/ significant disability disfigurement and mortality

Question 25

What pt population is scleroderma most commonly seen in

Answer 25

Women | Age 40-50

Question 26

Is scleroderma mostly limited or diffused? And what is the definition of each?

Answer 26

Mostly Limited Dz Limited dz = thickening of skin confined to face, neck & distal extremities Diffuse dz = widespread thickening of skin, including trunk w/ area of increased pigmentation & depigmentation

Question 27

What is CREST Syndrome?

Answer 27

aka scleroderma ``` Calcinosis cutis Raynaud Esophageal motility disorder Sclerodactyly Telangiectasis ```

Question 28

Typical presentation of scleroderma

Answer 28

- No facial expression - Sausage fingers - GI manifestation (angiodysplasia--watermelon skin like apperance on antrum of stomach) - Raynaud (initial presentation) - Polyarthralgia, weight loss & malaise - soft tissue swelling, puffy fingers, intense pruritis

Question 29

What lab findings are diagnostic for scleroderma?

Answer 29

``` Scleroderma antibody (anti-SCL-70) Anticentromere antibodies ``` Other - anti RNAP antibodies - antibodies to B23 assoc w/ pulm HTN - anti-RNA polymerase III antibodies

Question 30

Generally how would you tx scleroderma

Answer 30

Symptomatic & supportive -CCB!! long acting nifedipine, losartan, sildenafil

Question 31

What would you counsel a scleroderma pt when eating?

Answer 31

- take omeprazole (liquid or crushed) - eat small frequent meals & remain upright for least 2hr after eating - AVOID SUGARS at all cost (causes a lot of fermentation & peristalsis)