Autoimmune Conditions Flashcards
common labs used in diagnosis
C-reactive protein, erythrocytes sedimentation rate, rheumatoid factor, anti nuclear antibodies
Examples
RA, SLE, MS, celiac dx, Sjorgen’s syndrome
Immunocompromised pts are more at risk of:
- TB and HepB: must test and treat prior to starting immune-suppressing agents
- Viruses: if can be prevented by live vaccine; give prior to starting therapy
- Skin cancer and lymphoma are normally suppressed by the immune system
- Infections (monitor CBC and symptoms)
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
RA
Chronic, symmetrical, systemic, and progressive
Symptoms: joint swelling, stiffness, pain, and eventually bone deformity
Diagnosis of RA
Criteria 1-4 must be present for >/= 6 weeks and 4 or more criteria must be present:
- Morning stiffness around joints lasting >1hr
- Soft tissue swelling in 3 or more joints
- Sweeping of hand, foot, or wrist joints
- Symmetric involvement
- Rheumatoid nodules
- Positive serum rheumatoid factor
- Radio graphic erosions or osteopenia (hand or wrist joints)
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)
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
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,
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
Leflunomide (Arava)-DMARD
Blackbox warning: women of childbearing age should be excluded until pregnancy ruled out; hepatotoxicity
Pregnancy category X
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
Methotrexate counseling
- Only use this medication weekly
- 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
- Your doctor will need to perform frequent liver function test
- Consult your physician before taking NSAIDs or other anti-inflammatories with methotrexate
- Do not drink alcohol with this medicine as alcohol can cause damage to the liver
- 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
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
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
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)
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
Drug-induced lupus
Procainamide, hydralazine, isoniazid, quinidine, methyldopa, propylthiouracil, methimazole, terbinafine, anti-TNF agents
Non-pharm
Rest and proper exercise to manage fatigue, also smoking cessation and use of sunscreen/avoid sunlight due to photosensitivity
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
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
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
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
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
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))
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)
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
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
Interferon counseling
- 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
- 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
- Be mindful of allergic reactions following injection
- 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
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
Sjorgren’s Syndrome
Severe dry eyes and mouth
And be associated with other autoimmune diseases such as RA or SLE
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
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
