1. Discuss HCQ (hydroxychloroquine) use with your rheumatologist.
Rheumatologists vary when it comes to using HCQ for Sjogren’s. It can be helpful to review this blog post and the previous post, HCQ II - Why treat Sjogren’s? to prepare for your discussions with your clinician.
Key points to keep in mind:
Clinical Practice Guidelines from multiple international rheumatology groups (56, 57, 131, 132) recommend HCQ use for many Sjogren’s patients.
Few patients have absolute contraindications to HCQ use. It is one of the safest rheumatologic drugs available.
2. Consider HCQ risks while keeping in mind the benefits of HCQ.
A lot is known about HCQ risks and side effects from studies in other rheumatologic diseases. This information is helpful for Sjogren’s patients.
Retinal damage is the most serious HCQ risk. Damage rarely occurs during the first 5 years of use. The risk increases with the total accumulated dose, with 20% of patients showing retinal damage after 20 years of use. However, “Even after 20 years, a patient without toxicity only has a 4 % chance of converting in the subsequent year.” (140)
This can be reassuring to patients who continue to use HCQ long term, assuming they follow recommended screening guidelines (Item 3). When damage is caught early, HCQ use can be discontinued before serious visual impairment occurs.
The risk of retinal damage is increased with Tamoxifen use, a hormone blocker used to treat breast cancer.
Lower blood glucose. For many, this is a benefit. If you are on diabetes medication, discuss monitoring with your clinician.
Lower blood lipids. This may lower the risk of cardiovascular disease, an important contributor to early mortality in Sjogren's.
Skin rashes and GI problems such as nausea and diarrhea are the most common complaints, impacting a significant minority of patients. While usually mild and temporary, they can be a problem. The skin rash usually does not indicate actual allergy, but this should be reviewed with your clinician. Itching, hives or tingling of the lips and face may indicate an actual allergy.
Headaches, lightheadedness, irritability, and sleep disturbance are occasionally reported.
(126)
Cosmetic side effects include hyperpigmented areas (dark spots) usually on the face, as well as hair loss. HCQ may also improve Sjogren’s-induced hair loss and skin rashes (144).
Sun sensitivity- patients should take precautions to prevent sunburn and skin damage.
pustular skin lesions
severe hypoglycemia in patients on hypoglycemic drugs
bone marrow suppression
hemolysis with severe G6PD deficiency
Liver damage/ liver enzyme elevation
Addendum 12-2-2021: HCQ does not appear to increase the risk of cardiac rhythm problems (arrhythmias) in rheumatology patients, but more studies are needed, especially regarding long QT syndrome. Please consult with your doctor about your individual situation.
HCQ is associated with an overall reduction in cardiovascular disease risk, a significant comorbidity in Sjogren’s. (135, 126, 144)
3. Follow the recommended retinal screening guidelines for HCQ from the American Academy of Ophthalmology (AAO). See the AAO guidelines for complete details.
A baseline retinal screening exam should occur either before starting HCQ or within the first year of use.
Starting at 5 years, if there are no special risk factors, screen on an annual basis.
Screen more frequently and monitor blood levels of HCQ in patients with kidney disease, tamoxifen use, and possibly liver disease.
Miscellaneous considerations:
Patients of Asian descent require additional screening to look for a more peripheral pattern of damage that can occur in this group.
Low HCQ blood levels have been seen in patients with gastric bypass surgery. These patients may require higher doses and should have their blood levels monitored (143).
Higher HCQ blood levels may be seen in patients with kidney failure, and may require close monitoring of blood levels.
4. Practical Tips for using HCQ
The recommended HCQ dose is based on body weight, with the current maximum recommended dose of 5 mg/kg day. Keeping at or below the recommended dose reduces the risk of retinal damage. You may use this online dosage calculator to check your dose. The maximum dose is usually 400 mg/day for larger people.
You don’t have to cut pills in half if your dose is 300 mg/day. You can alternate days, 400 mg then 200 mg. HCQ is extremely long acting so you don’t need to take the same dose each day. Some brands now carry 100 mg and 300 mg pills.
If you have trouble with side effects early on (or simply want to be cautious), ask about starting at a lower dose and slowly increasing to the recommended dose. See the link at the bottom of the page for detailed strategies for easing your way into taking HCQ. If your side effects, especially GI or mood changes, are severe and persistent, consider switching brands. Many patients report doing well on one brand but having persistent side effects on another. This may be due to reactions from inert ingredients. You may need to do a bit of trial and error if your problematic (but not dangerous) symptoms are not starting to ease up after 2-4 weeks. Rashes are common, but even these can often be managed by desensitization as described by Dr. Donald Thomas (see the link below).
You may find it helpful to take HCQ with food to decrease GI irritation.
Be patient! Remember that HCQ is very long-acting. It usually does not usually help dryness, but patients often feel lower levels of musculoskeletal pain, stiffness, and fatigue. Most people notice no change in symptoms for at least 3 months. Some patients report it taking 9 months to a year before noticing an improvement. Others discontinue HCQ because it does not seem to be helping, only to find themselves feeling much worse a few months after stopping.
Each patient responds to HCQ differently. For some, the symptom improvement with HCQ is remarkable. For others, it is more subtle. Some report no symptom relief, but may benefit by reducing progression.
It is good to keep in mind that HCQ may have other long term benefits, not just improved pain and fatigue.
