Hydroxychloroquine (HCQ)

  • Class: 4-aminoquinolines
  • Derived from Cinchona tree (all antimalarials)
  • <5mg/kg (real weight)
  • In PCT: 100mg 3x/week

Anti-inflammatory

  • ↓IL1,6 (B cell diff to plasma), TNF, IFNγ), ↑IL10
  • ↓WBC chemotaxis
  • ↓ AA pathway → ↓ prostaglandins, leukotrienes 

Immunosuppressive

  • ↓ Ag presentation (↑lysosomal pH –↓Ag breakdown→ no TLR7, TLR9 activation) → inhibition of cell mediated immunity 

Light filtration

  • Bind melanin → absorbs light→ ↓ UVR mediated injury

Antiproliferative 

  • Intercalates DNA → inhibits synthesis
  • Modest antiviral (HIV)
  • Anti-thrombocyte aggregation  (Antiphospholipid syndrome)
  • ↓ Lipids (HMG-CoA reductase)
  • ↓ Glycemia
  • May ↓ Vitamin D levels
FDA

  • Malaria
  • Rheumatoid arthritis
  • Discoid lupus
  • Systemic lupus erythematosus (arthralgias, myalgias, serositis, B-symptoms, aphthae & skin response)

Off label

  • Photosensitive disorders (PCT, PMLE, solar urticarial, DM)
  • Granulomatous (sarcoidosis, GA)
  • Lymphocytic infiltrates (Lymphocytoma cutis, Jessner, REM syndrome)
  • Panniculitis (EN, lupus)
  • Other (oral LP, chronic ulcerative stomatitis, PF, AD, urticarial vasculitis, localized scleroderma, follicular mucinosis, psoriatic arthritis)
Absolute

  • Hypersensitivity
  • Retinopathy

Relative

  • Pregnancy
  • Lactation
  • Retinal/visual field changes
  • Severe blood dyscrasias
  • Significant Hepatic disorder
  • Significant Neurologic impairment e.g. myasthenia gravis 
  • Psoriasis (may lead to erythrodermic psoriasis)

Most common

  • Gastrointestinal SE
  • Blurry vision (transient & self-limited from muscle)
  • Skin pigmentations (10-30% with long-term use→ blue-black cutaneous discoloration shins, face, hard palate, & nail beds. More pronounced with HCQ)
  • Rashes (10-20%) 

Most important

Ocular toxicity: 

  • Reversible
    1. Corneal deposits 
    2. Premaculopathy (reversible retinopathy)
    3. Loss of accommodation
  • Irreversible
    1. True retinopathy (bull’s eye, central scotoma, changes in visual acuity) 

Cutaneous side effects

  • Minor hypersensitivity reactions: lichenoid> eczematous, & morbilliform eruption 
  • Major hypersensitivity reactions: erythroderma, urticaria, AGEP
  • Induction or worsening of psoriasis/ erythrodermic psoriasis
  • Bleaching of hair root: in 10% (interference with melanosome function)
  • Skin pigmentation (as above) + transverse nail pigmentation 
  • Dose-related yellowish staining with QE (skin, sclera, body fluid)

Reported

  • Leukopenia (reversible)
  • Agranulocytosis (CQ)
  • G6PD hemolysis (rare)
  • Neurologic & cardiac SE
  • Transaminitis 

Intoxication

  • Blurry vision
  • Cardiovascular toxicity
  • Cardiovascular/respiratory arrest
  • Neurologic toxicity
↓ Efficacy

  • Smoking (dose-dependant)
  • Antacids (↓ absorption)

↑ Toxicity

  • HCQ + CQ = retinal toxicity
  • Local anesthetics (benzocaine, prilocaine): ↑Methemoglobenemia
  • Cimetidine (↓ metabolism)

Antimalarial ↑ toxicity of

  • Cyclosporine
  • Digoxin
  • Antiarrhythmics

Baseline

  • Baseline eye exam (within 1st year): slit lamp, Funduscopy, visual fields & acuity
  • CBC, chemistry profile, creatinine, LFT
  • G6PD (with 8- aminoquinolines mainly/ Unnecessary to screen for G6PD due to low risk of hemolysis at doses of antimalarials utilized in dermatology) +/- urine porphyrins

FU

  • CBC, LFT, creatinine qmonthly x3months, then q3-6months
  • Eye exam annually after 5 years of use

Retinopathy risk factors

  • Cumulative dose & daily dose
  • Elderly (> 60 years)
  • Impaired liver &/or renal function 
  • Pre-existing macular/retinal disease
  • Tamoxifen with HCQ
  • HCQ + CQ combined

Notes

  • Half-life 40-50 days (except QE 1-2 weeks)
  • Likes melanin rich tissue (skin & retina) 
  • Response seen within 2-3 months
  • Should be continued in pregnant systemic lupus patients as benefits out-weight risks (↓ risk of clots & neonatal lupus heart block)
  • Skin pigmentation on histopathology → hemosiderin around capillaries & dermal melanin
  • Highest risk for drug eruption (HCQ) is with anti-SAE DM & no risk with anti- MDA-5 DM (1)