1. Raynaud's
1.1. Drug-induced
1.1.1. Beta blockers, bleomycin/cisplatin, amphetamines, pseudoephedrine, & illicit drugs
1.2. Prevention & tx:
1.2.1. Vasodilation to improve blood flow
1.2.1.1. **Nifedipine**
1.2.1.2. Topicla nitro
1.2.1.3. PDE-5 inhibitors
2. Celiac
2.1. Primary tx is to avoid gluten entirely
2.2. Some drugs contain gluten excipients
2.2.1. Can be labeled "gluten-free" if gluten < 20ppm
3. Myasthenia gravis
3.1. Autoimmune that attacks connections btwn nerves & muscles, resulting in weakness in skeletal muscles
3.1.1. Usually targets Ach receptor
3.2. Sx: vision changes; drooping eyelid (ptosis)
3.3. Drugs which can worsen
3.3.1. Aminoglycosides, quinolones
3.3.2. Magnesium salts
3.3.3. Select antiarrhythmics
3.3.4. BB & CCB
3.3.5. Select antipsychs
3.3.6. Muscle relaxants
3.3.7. Local anesthetics
3.4. Pharm tx
3.4.1. **Pyridostigmine (MEstinon)**
3.4.1.1. Cholinesterase inhibitor: blocks breakdown of Ach
3.4.1.2. **Cholinergic effects:** SLUDD
4. Sjogrens Syndrome
4.1. Severe dry eyes & dry mouth
4.1.1. Can be secondary to RA or SLE
4.2. Primary tx: artificial tears
4.2.1. **Restasis or Xiidra**
5. Psoriasis
5.1. Non-pharm
5.1.1. Brief daily exposure to sunlight
5.1.2. UVB phototherapy for mild-mod disease
5.2. Pharm
5.2.1. Topical steroids, tazarotene, coal tar products
5.2.2. If above fail, topical calcineurin inhibitors **(Prototopic, Elidel)**
5.2.2.1. Preferred for face
5.2.3. Retinoid
5.2.3.1. **Acetretin (soriatane)**
5.2.3.1.1. Only in severe cases
5.2.3.1.2. BBW: hepatotoxicity & pregnancy
5.2.4. PDE-4 inhibitor
5.2.4.1. **Apremilast (Otezla)**
5.2.4.1.1. Warnings: weight loss, depression, suicidal ideation
6. Systemic steroids
6.1. Used for inflammatory conditions, immunosuppression, & adrenal insufficiency
6.1.1. Cortisol: replaceable by any steroid
6.1.1.1. Systemic steroids can cause adrenal gland to stop producing cortisol due to feedback inhibition
6.1.1.1.1. "Suppression of the HPA axis"
6.1.1.1.2. When long-term steroids d/c, need to be **tapered**
6.1.2. Aldosterone: replaced by fludrocortisone (mimics aldosterone)
6.1.2.1. Used to maintain balance of water & electrolytes which keeps BP stable
6.1.2.2. Fludrocortisone FDA approved for **Addison's disease** but also used off-label for **orthostatic hypotension**
6.2. Cushing's syndrome
6.2.1. When adrenal gland makes too much cortisol or if steroids taken in doses higher than normal amount of cortisol
6.2.1.1. Long-term effects
6.2.1.1.1. Glaucoma
6.2.1.1.2. Fat deposits in face, abdomen, & upper back
6.2.1.1.3. Psych changes: anxiety, depression, delirium, psychoses; HA, intracranial HTN, hypOthyroidism
6.2.1.1.4. Acne
6.2.1.1.5. GI bleeding, esophagitis, ulcers
6.2.1.1.6. Hirsutism, irregular menstrual
6.2.1.1.7. Infection, impaird wound healing, poor bone health
6.3. Addison's
6.3.1. Not making enough cortisol or if steroids stopped suddenly
6.3.1.1. Volume depletion
6.3.1.2. Hypotension
7. Steroid potency
7.1. Cortisone < hydrocortisone < prednisone < prednisolone < methylprednisolone < triamcinolone < dexamethasone < betamethasone
7.2. Pt is immunosuppressed when using 2+mg/kg/day or 20+mg/day of prednisone or prednisone equivalent for > 2 weeks
8. Rheumatoid arthritis
8.1. Sx: joint swelling, pain, stiffness, bone deformity, weakness, edema, redness
8.1.1. Stiffness/pain usually worse after rest
8.1.2. Usually bilateral/symmetrical (OA usually unilateral)
8.2. Dx: anti-citrullinated peptide antibody (ACPA) & rheumatoid factor (RF)
8.3. Pharm tx: If symptomatic, start a DMARD, regardless of severity
8.3.1. DMARDs slow disease process & prevent further damage
8.3.1.1. **Methotrexate (trexall)**
8.3.1.1.1. Irreversibly binds & inhibits dihydrofolate reductase
8.3.1.1.2. 7.5-20mg once weekly; never daily for RA
8.3.1.1.3. BBW: hepatotoxicity, myelosuppression, mucositis/stomatitis, pregnancy
8.3.1.1.4. Monitoring: CBC, LFTs, chest xray, hep B & C serology
8.3.1.1.5. Folate can be given to :arrow_down: hematological, GI, & hepatic side effects
8.3.1.2. **Hydroxychloroquine (plaquenil)**
8.3.1.2.1. Warnings: irreversible retinopathy, myopathy, neuropathy; QT, hypOglycemia, psych events
8.3.1.2.2. Immune modulator
8.3.1.2.3. Monitoring: eye exam
8.3.1.2.4. Lower risk of liver toxicity than MTX
8.3.1.2.5. Dosed daily; **take w/ food or milk**
8.3.1.3. **Sulfasalazine (azulfidine)**
8.3.1.3.1. C/I in sulfa & **salicylate** allergy
8.3.1.3.2. Immune modulator
8.3.1.3.3. Warnings: blood dyscrasias, SJS/TEN, hepatic failure, pulmonary fibrosis
8.3.1.3.4. Can cause yellow/orange skin
8.3.1.4. **Leflunomide (arava)**
8.3.1.4.1. inhibits pyrimidine synthesis (MTX is purine); prodrug of teriflunomide
8.3.1.4.2. BBW: do not use in pregnancy; hepatotoxicity
8.3.1.4.3. Warnings: SJS/TEN, peripheral neuropathy, HTN
8.3.1.4.4. Accelerated drug elimination options (for d/c): cholestyramine or activated charcoal suspension
8.3.2. Tx goal is remission of disease
8.3.3. JAK inhibitors
8.3.3.1. **Tofacitinib (Xeljanz)**
8.3.3.2. **Baricitinib (olumiant)**
8.3.3.2.1. BBW: serious infections including TB; malignancy; thrombosis; risk of CV events
8.3.3.3. **UPadacitinib (rinvoq)**
8.3.4. Anti-TNF **biologic** DMARDs
8.3.4.1. **Etanercept (enbrel)**
8.3.4.1.1. SQ weekly
8.3.4.2. **Adalimumab (humira)**
8.3.4.2.1. SQ every other week
8.3.4.3. **Infliximab (remicade)**
8.3.4.3.1. IV at weeks 0, 2, & 6, then every 8 weeks (NS only
8.3.4.3.2. Give w/ MTX
8.3.4.4. **Certolizumab pegol (cimzia)**
8.3.4.4.1. SQ every other week
8.3.4.5. **Golimumab (Simponi)**
8.3.4.5.1. SQ monthly
8.3.5. Other **biologic** DMARDs
8.3.5.1. **Rituximab (rituxan)**
8.3.5.1.1. depletes CD20 B cells
8.3.5.1.2. + MTX
8.3.5.1.3. BBW: infusion-related rxns, PML, SJS/TEN; HBV/HBC reactivation
9. Systemic lupus erythematosus
9.1. DILE drugs
9.1.1. Methimazole, PTU, methyldopa, minocycline, procainamide, hydralazine, Anti-TNF agents, terbinafine, isoniazid, quinidine
9.2. Presentation:
9.2.1. Fatigue, depression, weight loss, muscle pain, malar rash, photosensitivity, joint pain/stiffness
9.3. Complications: lupus nephritis, renal, hematologic, & neurologic
9.3.1. Positive ANA findings
9.4. Pharm tx: try to minimize chronic steroid exposure
9.4.1. Mild disease may do well on NSAID; use with PPI to reduce GI risk
9.4.2. Usually will require 1+ immunosuppressant/cytotoxic agent
9.4.2.1. Hydroxychloroquine, cyclophosphamide, azathioprine, mycophenolate, & cyclosporine are all options
9.4.2.1.1. May take up to 6 months to see max benefit
9.4.2.2. Anifrolumab (Saphnelo) recent FDA-approved tx for lupus that inhibits type-1 interferons
9.4.2.3. **Belimumab (Benlysta)**
9.4.2.3.1. IgG1-lambda MAb
9.4.2.3.2. Warnings: infections, do not give w/ other biologic DMARDs or live vaccines
9.4.2.4. **Voclosporin (Lupkynis)**
9.4.2.4.1. Calcineurin inhibitor
9.4.2.4.2. BBW: infections; malignancies
9.4.2.4.3. Warnings: nephrotoxic, HTN, do not give w/ live vaccines
10. Multiple sclerosis
10.1. Presentation: fatigue, numbness, blurred vision, deterioration of cognitive fx, muscle spasms, pain, incontinence, gait instability
10.2. Primary goal of tx: prevention of disease progression (what is lost cannot be regained)
10.2.1. Steroids used to tx relapses: usually methylpred IV for 3-7 days; w/ or w/o PO steroid taper after
10.3. Disease-modifying tx
10.3.1. **Glatiramer acetate (Copaxone)**
10.3.1.1. Immune modulator thought to induce & activate T-lymph suppressor cells in relapsing forms of MS
10.3.1.1.1. **preferred agent in pregnancy if needed**
10.3.1.2. SC daily or higher dose SC 3x weekly
10.3.1.3. Warnings: chest pain
10.3.1.3.1. AE: injection site rxns, flushing, diaphoresis, dyspnea
10.3.2. Interferon beta products
10.3.2.1. **beta-1a (Avonex)**
10.3.2.1.1. IM weekly or **Rebif** is SC 3x/week
10.3.2.2. **beta-1b (Betaseron)**
10.3.2.2.1. SC every other day
10.3.2.3. **peginterferon beta-1a (Plegridy**
10.3.2.3.1. SC every 14 days
10.3.3. Pyrimidine synthesis inhibitor
10.3.3.1. **Teriflunomide (Aubagio)**
10.3.3.1.1. C/I in pregnancy & severe hepatic impairment
10.3.4. Sphingosine 1-phosphate receptor modulators
10.3.4.1. **Fingolimod (Gilenya)**
10.3.4.1.1. Monitor HR (brady risk)
10.3.4.2. **Ozanimod (Zeposia)**
10.3.4.3. **Siponimod (Mayzent)**
10.3.5. MAbs
10.3.5.1. **Natalizumab (Tysabri)**
10.3.5.1.1. See IBD chapter
10.3.5.1.2. BBW: PML; only available through REMS TOUCH program
10.4. Sx control
10.4.1. Anticholinergics for incontinence, laxatives for constipation (loperamide for diarrhea), skeletal muscle relaxants, analgesics
10.4.1.1. Drugs used for sx control can worsen other sx
10.4.2. Propranolol for tremor