Lupus
Long-term management of this autoimmune condition, with treatment based on the organs affected
What is lupus?
Lupus is an autoimmune disease in which the immune system makes antibodies against the body's own cells. Those antibodies and the immune complexes they form settle in tissues and cause inflammation, and because they can settle almost anywhere, lupus can involve the skin, the joints, the kidneys, the blood cells, the lining of the heart and lungs and the nervous system.
About nine in ten of the people affected are women, most commonly between the ages of fifteen and forty-five. The susceptibility is partly genetic, and the disease usually needs a trigger on top of it. Sunlight is the best established one, along with infections, hormonal changes including pregnancy, and a small number of medicines.
The course runs in flares and quiet periods instead of a steady decline, and long remissions are common with treatment. Which organs are involved is the thing that decides everything: which medicines you need, how closely you are monitored and what the outlook is. Two people with the same diagnosis can have entirely different treatment for that reason. Involvement of the kidneys is the most important to find early, because it causes no symptoms at all until it is advanced and is detected only by testing.
What you might notice
- A rash across the cheeks and nose
- Several joints swollen and painful at once
- Frothy urine
How it is diagnosed
No single test diagnoses lupus, and expect the diagnosis to be built over more than one visit. Your rheumatologist examines the skin on the face, in the sun-exposed areas and on the scalp, looks inside the mouth and nose for ulcers, checks the joints for swelling and tenderness, listens for fluid around the heart and lungs, and measures your blood pressure at every visit.
Blood and urine are as important as the examination. A full blood count shows whether the immune process is destroying red cells, white cells or platelets. Kidney function is measured, and a urine sample is checked for protein and blood, which is how kidney involvement is found while it is still silent and treatable.
The antibody tests are interpreted with care. A positive antinuclear antibody is present in almost everyone with lupus and also in many healthy people, so on its own it proves nothing. More specific antibodies and complement levels carry more weight and are used to track activity over time. Where the urine is abnormal, a kidney biopsy is usually needed, since what is happening inside the kidney determines the treatment.
Recovery
Think of lupus as a condition that is brought into remission and then kept there. A flare usually settles over two to eight weeks on treatment, and the medicines used to settle it are then reduced slowly over months. Coming off steroids too quickly is a common way to provoke the next flare, so follow the taper exactly as it is written.
Most people stay on a background medicine long term even when they feel entirely well, because stopping it raises the flare rate and the risk of organ damage. Eye checks are scheduled periodically with that medicine.
Monitoring continues for life, typically every three months when you are stable and more often after a change in treatment, with blood and urine tested each time. Sun protection is daily and year-round. Vaccination is planned with your doctor, since live vaccines are avoided on immune-suppressing treatment. If you are planning a pregnancy, raise it early: pregnancy is much safer when the disease has been quiet for about six months and the medication has been reviewed beforehand, and it needs joint care from a rheumatologist and an obstetrician.
If it is left untreated
The damage lupus does accumulates permanently even though the disease itself comes and goes. Each untreated flare leaves a little more scarring behind in whichever organ it involved, and the total damage over the years is what determines how well someone does.
The kidneys are the clearest example. Inflammation there produces no pain and no symptoms until function is well down, and untreated it scars the filtering units irreversibly and can lead to kidney failure requiring dialysis. It is found by a urine test costing very little, which is the reason you are asked for one at every visit even when you feel perfectly well.
Uncontrolled disease also raises the risk of blood clots in the legs and lungs and of pregnancy loss, particularly where a specific group of antibodies is present, and long-standing inflammation accelerates heart disease markedly in a group of women who are otherwise young. Infection is the other major risk, both from the disease and from the treatment, so a fever on immune-suppressing medication is assessed the same day. New chest pain, breathlessness, seizure, confusion, sudden swelling of the legs or face, or a sharp drop in urine output all need urgent assessment.
Questions about lupus
Can I have children?
Most women with lupus can have a successful pregnancy, and the planning is what makes the difference. Pregnancy is considerably safer when the disease has been quiet for about six months beforehand, and particularly when the kidneys are not actively involved, so raise it with your rheumatologist well before you start trying. Some medicines used in lupus are unsafe in pregnancy and need switching to alternatives months in advance, and others are continued deliberately throughout because stopping them raises the risk of a flare. Certain antibodies raise the risk of miscarriage, clots and problems for the baby, and they are tested for so that treatment and monitoring can be arranged. Expect shared care with an obstetrician.
I feel completely well. Why do I need blood and urine tests every three months?
Because the most important thing being looked for causes no symptoms. Inflammation in the kidneys produces no pain and nothing you would notice until a large amount of function has already been lost, and once the filtering units have scarred, that is permanent. A urine test that costs very little detects protein and blood while the problem is still fully treatable. The blood count picks up the immune system destroying red cells or platelets silently, and specific antibody and complement levels often shift before a flare becomes apparent. Feeling well is genuinely good news and it is not evidence about the kidneys, so keep the appointments during the quiet periods.
Does sunlight really matter that much?
Yes. Ultraviolet light damages skin cells in a way that exposes material the immune system reacts against, and in lupus that can set off a rash and a whole-body flare involving the joints, the blood counts and occasionally the kidneys. The effect is not limited to the skin that was exposed and it can be delayed by days, so people frequently fail to connect the two. Use a broad-spectrum sunscreen of SPF 50 daily on the face, neck and arms, reapply it every few hours outdoors, wear long sleeves and a wide hat, and avoid the middle of the day. Fluorescent and some LED lighting emits a small amount as well.
Other rheumatology treatments
Long-term treatment to control autoimmune inflammation affecting the joints and other organs
Day CareManagement of joint pain and stiffness caused by cartilage wear and degenerative joint changes
Day CareTalk to a care coordinator about lupus
Leave a number and a care coordinator calls you back with what the treatment involves and which hospital it would be at. They book a consult only if you want one