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Flashcards in Autoimmune Conditions Deck (33)
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Examples

RA, SLE, MS, celiac dx, Sjorgen's syndrome

1

common labs used in diagnosis

C-reactive protein, erythrocytes sedimentation rate, rheumatoid factor, anti nuclear antibodies

2

Immunocompromised pts are more at risk of:

1. TB and HepB: must test and treat prior to starting immune-suppressing agents
2. Viruses: if can be prevented by live vaccine; give prior to starting therapy
3. Skin cancer and lymphoma are normally suppressed by the immune system
4. Infections (monitor CBC and symptoms)

3

Vaccines

Live vaccines: yellow fever, measles, rubella, varicella, and zoster should not be given to those who are immunocompromised

Inactivated: influenza, Tdap, pneumococcal, (pts with asplenia should get meningococcal

4

RA

Chronic, symmetrical, systemic, and progressive

Symptoms: joint swelling, stiffness, pain, and eventually bone deformity

5

Diagnosis of RA

Criteria 1-4 must be present for >/= 6 weeks and 4 or more criteria must be present:
1. Morning stiffness around joints lasting >1hr
2. Soft tissue swelling in 3 or more joints
3. Sweeping of hand, foot, or wrist joints
4. Symmetric involvement
5. Rheumatoid nodules
6. Positive serum rheumatoid factor
7. Radio graphic erosions or osteopenia (hand or wrist joints)

6

Pharmacologic treatment

Goal is to have the patient on a DMARD within three months of diagnosis (two slows disease progression)

These are often bridged with NSAIDs or steroids short-term or long-term

Patients with milder symptoms may be able to live acceptably on non-biologic agents such as methotrexate, hydroxychloroquine, laflunomide sulfasalazine, and minocycline

Very severe patients may need anti-TNF agents (adalimumab, entanercept, golimumab, infliximab)

7

Methotrexate (Rheumatrex, Trexall)- DMARD

Low weekly doses are used for RA these smaller doses can be split into smaller doses taken over 12 to 36 hours but never dose daily for RA

Blackbox warning hepatitis, renal, pneumonitis, bone marrow suppression, mucositis, dermatologic, renal and lung toxicity

Contraindications: pregnancy and breast-feeding, alcoholism, chronic liver disease, blood dyscrasias, immunodeficiency syndrome

Side effects: nausea, vomiting, increased LFTs, stomatitis, alopecia, photosensitivity

Monitoring: SCr and LFTs every 2 to 4 weeks for three months

Notes: pregnancy category X, folic acid can be given to decrease the side effects associated commonly given 5 mg by mouth weekly on the day following methotrexate; may take up to 12 weeks to see full benefit can also give 1 to 4 mg per day

New formulation otrexup approved in 2013 for patients to self administer methotrexate SC once weekly via auto injector

absolutely do not take with alcohol, loops and methotrexate interact use caution when used together, AVOID METHOTREXATE AND CYCLOSPORINE TOGETHER

8

Hydroxychloroquine or Plaquenil-DMARDS (also used for malaria )

Take with food or milk

Side effects: nausea, diarrhea, rashes, pigmentation of skin and hair, weakness, vision changes (blurred vision, retinal damage)

Monitoring: eye exam at baseline and every three months, CBC, LFTs

Notes: pregnancy category C may take 4 to 6 weeks to respond,

9

Sulfasalazine (Azulfidine)-DMARDS

Contraindications: sulfa or salicylate allergy, G.I. or GU obstruction

Side effects: headache, anorexia, dyspepsia, G.I. upset, oligospermia, rash, folate deficiency, arthralgias, Crystalluria

Notes: Can cause yellow – orange coloration of skin or urine; impairs folate absorption may give 1 mg per day folate supplement

10

Leflunomide (Arava)-DMARD

Blackbox warning: women of childbearing age should be excluded until pregnancy ruled out; hepatotoxicity

Pregnancy category X

11

Tofactinib (Xeljanz)- DMARD

Blackbox warning: increased risk for serious infections, risk for developing active tuberculosis, increased risk for lipomas or other malignancies, invasive fungal, viral, bacterial or opportunistic infections

Warning not recommended in severe hepatic impairment

Side effects: bone marrow suppression, infections, diarrhea, headache, increased blood

Note: pregnancy category C, can be used monotherapy only with non-Biologics, do not use with biologic DMARDs or potent immunosuppressants must dispense Medguide

12

Methotrexate counseling

1. Only use this medication weekly
2. If you're pregnant or have a chance of becoming pregnant, you should not use this medicine as it is a pregnancy category X also do not use if you are breast-feeding
3. Your doctor will need to perform frequent liver function test
4. Consult your physician before taking NSAIDs or other anti-inflammatories with methotrexate
5. Do not drink alcohol with this medicine as alcohol can cause damage to the liver
6. Consult your doctor if you notice black tarry stools, red swollen or blistered skin, persistent diarrhea, shortness of breath, signs of infection, pain, sore throat, stomach pain, yellowing of the skin or eyes

13

Anti-TNF BIOLOGICS

examples: entanercept (enbrel), adalimumab (humira), infliximab (remicade), golimumab (simponi)

Blackbox warning: serious infections discontinue treatment patient develops a severe infection; lymphomas and other malignancies; reactive or latent TB or new infection; perform test for latent TB prior to starting

Contraindications: active systemic infection

Warnings: can calls demyelinating disease, hepatitis B reactivation, heart failure, hepatotoxicity, lupus Like syndrome, severe infections and should not be used with other TNF inhibitors or immunosuppressive Biologics or live vaccines

Monitoring: TB test, signs and symptoms of infection, CBC, LFTs, HBV, heart failure, malignancy

Note: do not shake. Requires refrigeration. Allow to reach room temperature before injecting (approximately 15 to 30 minutes), methotrexate is typically first line but these agents will be used in severe disease. Do not use more than one biologic concurrently. Do not use why vaccines if using this drug

Dispense med guide as all TNF inhibitors. Blackbox warning for risk of serious infection

14

Infliximab or Remicade

Contraindications: does greater than 5 mg per kilogram in moderate to severe heart failure

All others are SC but this is an IV infusion and may have infusion reactions such as: hypertension, fever, chills, puritis and may benefit with pretreatment of acetaminophen, antihistamine, steroid

Also note that a delayed hypersensitivity reaction can occur at 3 to 10 days after administration and appears as fever, rash, myalgia, headache and sore throat

15

Non-TNF biologics

Rituximab: given with methotrexate in RA and patient should be pre-treated with a steroid. Carries a black box warning for severe and fatal infusion related reactions, PML due to JC virus, tumor lysis syndrome, severe and fatal rxn (SJS, TEN). All patient should be screened for latent TB prior to initiation

Anakinra (kineret): can cause serious infection, and you should screen for latent TB prior to initiation, do not give with other Biologics or live vaccines

Abatacept (orcenia): same considerations as others. This should be used with caution in COPD PTS as could exacerbation

Tocilizumab (actemra): same as others. Warnings: screen for TB, inc risk of infection, do not give with other biologics, INC LFTS ( do not give if AST OR ALT > 1.5 times ULN)

16

Systemic Lupus Erythematosus (SLE)

10 to 1 ratio of female to male; predominately age 15-45 and African American or Asian descent

Hallmark: development of auto-antibodies against multiple systems

Presentation: fatigue, fever, anorexia, weight loss, muscle aches, arthritis, rash (butterfly), photosensitivity, joint pain, and stiffness, Renal, hematologic and neurologic manifestations contribute largely to morbidity and mortality

Lupus nephritis develops in over 50% of patients

17

Drug-induced lupus

Procainamide, hydralazine, isoniazid, quinidine, methyldopa, propylthiouracil, methimazole, terbinafine, anti-TNF agents

18

Non-pharm

Rest and proper exercise to manage fatigue, also smoking cessation and use of sunscreen/avoid sunlight due to photosensitivity

19

Drug therapy:

Immunosuppressants, cytotoxic agents, and/or anti-inflammatory agents

Use combination therapy to minimize chronic exposure to corticosteroids

Goal of therapy: suppress the immune system to avoid disease flares and keep the patient in remission

20

Antimalarial agents-impair complement dependent antigen-antibody reaction

Drugs: hydroxychloroquine, Chloroquine

Hydroxychloroquine is safer but takes six months to see maximum effect

Used for mild disease

21

Corticosteroids: prednisone or methylprednisolone

Methylprednisolone IV if life-threatening disease

Used acutely for control of flares at higher doses taper to lower doses for chronic suppressive therapy

22

Cytotoxic agents used in severe disease

Drugs: cyclophosphamide, azathioprine (azasan), mycophenolate mofetil (cellcept), cyclosporine (neoral, sandimmune)

Cyclophosphamide: side effects include bone marrow suppression, infections, hemorrhagic cystitis (give mesna therapy and keep patient well hydrated to prevent), malignancy, sterility, teratogenesis (Notes: pregnancy category, and can be used IV or PO; used for flares as induction therapy; very toxic for chronic therapy

Azathioprine: blackbox warning includes chronic immunosuppression which can lead to increased risk of neoplasia, metalogic toxicity such as leukopenia thrombocytopenia and mutagenic potential; side effects include G.I. upset, rash, increased LFTs, bone marrow suppression

Mycophenolate mofetil: blackbox warning includes increased risk of infection, increased risk of development of lymphoma and skin malignancy, increased risk of congenital malformation and spontaneous abortions when used during pregnancy. Side effects include diarrhea, G.I. upset, vomiting, hypo tension, edema, tachycardia, please come hyperglossal, hyper and hypokalemia, hypomagnesemia, hypocalcemia, hypercholesterolemia, Tremor, acne, infection. This drug lowers efficacy of birth control pills

Cyclosporine: blackbox warning Reno impairment, increase risk of lymphoma, skin cancer, infection, make cause hypertension, note that modified neoral has increased bioavailability compared to Sandimmune cannot be used interchangeably. Side effects include hypertension, nephropathy, increased triglycerides, nausea

23

Multiple sclerosis

Patient's immune system attacks the myelin peptide antigens, destroying myelin sheaths that surround the axons of the brain and spinal cord

Early symptoms: weakness, tingling, numbness and blurred vision

Later symptoms: deterioration of cognitive function, fatigue, muscle spasms, pain, incontinence, depression, heat sensitivity, sexual dysfunction, difficulty walking instability from a weakness and visual disturbances

Occurs in men and women but it's more common in women and the typical onset is ages between 20 and 40 years old

Primary goal of therapy is to prevent disease progression; what is lost in neuronal function cannot be regained

24

Pharmacologic treatment of MS

Mitoxantrone (chemo agent), steroids are used for exacerbations, disease modifying drugs (interferon beta, glatiramer acetate (copaxone) are mainstay of therapy) (first oral agents fingolimod (gilenya) and teriflunomide (aubagio))

25

Glatiramer (copaxone)

SC daily injection

Side effects: injection site reactions, infection, pain, Flushing, chest pain, weakness, anxiety, rash, nausea

Notes: pregnancy category B, can we get that room temperature for the one month or the refrigerator, and Inject at the same time each day into the fatty skin (SC)

26

Interferon beta

Avonex: IM once weekly injection
Rebif: SC three times a week
Betaseron, Extavia: SC injection every other day

Warning: depression/suicide, injection site necrosis, bone marrow suppression, increased LFTs, thyroid dysfunction, infections, anaphylaxis, worsening cardiovascular disease

Side effects: flu like reaction that can be pretreated with acetaminophen or NSAIDs

If refrigerated let stand at room temperature prior to injection but do not expel small air bubble in prefilled syringe because dose will be reduced

27

Fingolimod (Gilenya)

Daily capsule

Contraindications: recent MRI, unstable angina, stroke, TIA, heart failure requiring hospitalization, NYHA class three or four heart failure, history of second or third degree heart block or six sinus syndrome, QTC interval greater than 500 ms, use of antiarrhythmics

WARNING: DECREASE IN HEART RATE, DECREASE IN PULMONARY FUNCTION TEST, INCREASE IN LFTS

monitoring: CBC, ECG pre-and post dose

28

Interferon counseling

1. This drug may lower your ability to fight infections so monitor for signs of infection such as fever, chills, sore throat, cough, sputum or change in the color of sputum, sweating or pain
2. May cause liver damage therefore monitor for signs of liver damage such as tiredness, skin or eyes that look yellow, poor appetite or vomiting, pain on the right side of your stomach
3. Be mindful of allergic reactions following injection
4. Most of these drugs are not recommended in pregnancy (pregnancy category C) particularly Teriflunomide (pregnancy category X) therefore talk to your doctor before becoming pregnant

29

Celiac disease

Immune response to eating gluten, a protein found in wheat, barley and rye

Primary and effective treatment is to avoid gluten

Common symptoms: diarrhea, abdominal pain, bloating and weight loss, patients may also have vitamin deficiencies due to abnormal absorption, dermatitis herpetiformis (extremely itchy, blustery skin rash with chronic eruptions)

Serologic assays antibodies tests are used for diagnosis

Higher risk in Caucasians and twice as likely to occur in females than males

Notes Drugs themselves are gluten-free but the excipients often are not, look in the package insert to find out if the package insert says starch only, the manufacturer must be called

30

Sjorgren's Syndrome

Severe dry eyes and mouth

And be associated with other autoimmune diseases such as RA or SLE

31

Treatment of dry eyes

Artificial teardrops is the primary treatment: Systane, refresh, Clear Eyes and Liquifilm

Use an appointment if this primarily occurs during sleep

Cyclosporine eyedrops or Restasis can be used in patients who do not get satisfactory relief from other measures

cyclosporine (Restasis): one drop b.i.d., not administer while wearing contact lenses

32

Treatment of dry mouth

Not pharmacologic: sugar-free chewing gum, lozenges, daily rinses with antimicrobial mouthwash, salivary substitute (plax, oralube, salivart)

pharmacologic: OTC does not work
1. Pilocarpine: four times daily. Contraindications include uncontrolled asthma, narrow angle glaucoma, severe hepatic impairment
2. Cevimeline (evoxac): three times daily and contraindications include uncontrolled asthma, narrow angle glaucoma, acute iritis