Lupus shows up in many different ways. Symptoms can affect the skin, joints, or various organs (kidneys, lungs, heart, eyes, etc.). Systemic lupus is identified by the combination of several clinical and laboratory abnormalities (presence of specific antibodies).
Types of lupus
Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) or systemic acute erythematosus (LEAD) is lupus that affects any organ. It is the most common form of lupus. It usually manifests as inflammation of the joints or tendons and the most characteristic symptom is a “butterfly wing” rash on the cheeks and bridge of the nose.
Discoid lupus erythematosus
Discoid lupus erythematosus is most often confined to the skin and does not affect the internal organs of the body. It is manifested by very limited, often multiple and symmetrical plaques which predominate in the face, sometimes also taking a butterfly wing arrangement or circular shapes.
It can also affect the ears, eyebrows, and scalp. The lesions of discoid lupus have a different course from those of systemic lupus, and leave very unsightly permanent scars, hence the urgency of treatment. There are forms of disseminated discoid lupus with lesions that may appear on the trunk and limbs; lesions then predominate on the elbows and extremities.
Isolated skin lupus
Unlike systemic lupus, isolated cutaneous lupus exclusively affects the skin on the face. Certain treatments, such as the use of UV protection, antimalarial therapy, corticosteroids, and/or calcineurin inhibitors, can improve the appearance of the skin, limiting scarring and preventing new skin lesions.
Drug Induced lupus
Induced lupus occurs following the prolonged use of certain drugs such as doxycycline (antibiotics prescribed against acne), Bactrim® (another antibiotic), certain anti-tuberculosis drugs, beta-blockers (antihypertensive drugs), certain antiepileptics (carbamazepine), and anti-epileptics -TNF. Stopping these medications usually causes symptoms to go away rather quickly.
Neonatal lupus is linked to the presence in the mother of anti-SSA (also called anti-Ro) or SSB (or anti-La) antibodies which will affect the fetus or newborn.
Lupus can start with one or more of the following signs appearing at a push and is often associated with moderate but sustained fever, tiredness, or lack of appetite.
The initial symptoms are most often joint pain and skin damage. But in the course of the disease, other organs can be affected.
Skin and scalp damage
Skin and scalp problems occur in about 80% of people with lupus. They are frequently triggered or worsened by exposure to the sun.
The most suggestive symptom of systemic lupus erythematosus is a rash on the face, appearing during a flare-up. These red patches predominate on areas of skin exposed to the sun (photosensitivity). They take on the appearance of a mask in the shape of butterfly wings, around the eyes, on the nose, and on the cheekbones.
They can be accompanied by edema (swelling of the skin) more or less marked, especially on the eyelids, hindering the opening of the eyes.
These lesions can also be visible on other areas of the body exposed to the sun: the neckline, the hands.
This type of rash goes away at the end of the flare-up.
Slowly evolving skin damage
Slow or chronic skin damage over the whole body: ring-shaped rashes, purplish patches, nodules, plaques with desquamation (resembling psoriasis plaques) may also appear.
Damage to the mucous membranes and scalp
Canker sores in the mouth, even in the nose and the back of the nose (pharynx) can appear.
At the time of or within three months of a lupus outbreak, diffuse hair loss can also be responsible for a thinning scalp. This diffuse alopecia gradually disappears after starting treatment.
Joint pain is very common and often present at the onset of systemic lupus erythematosus.
Joint pain affects different joints, especially the small joints of the fingers and wrists, but also the knees, feet, and ankles.
The affected joints are inflammatory: they become hot and swollen, but they are not deformed.
The pain predominates at night and generally affects the joints symmetrically. The back is usually spared from pain. Muscle pain is sometimes associated.
Raynaud’s phenomenon is a color change affecting the tip of the fingers or toes, sometimes accompanied by pain. Under the effect of cold or stress, the affected extremities become pale then bluish, and sometimes red. This phenomenon is often present in people with lupus. However, it is not specific for lupus. Indeed, it is very often found in the general population, without necessarily being associated with a disease.
Phlebitis (formation of a blood clot in a vein) or thrombosis of an artery: cerebrovascular accident (AVC), myocardial infarction, pulmonary embolism … can inaugurate the disease.
These thromboses are related to the presence of an antiphospholipid antibody syndrome often associated with lupus.
Kidney damage during lupus
Kidney involvement is less common. It sometimes occurs at the onset of systemic lupus or appears gradually. This is a very variable severity of the renal glomeruli which filter urine (glomerulonephritis). It sometimes progresses to chronic kidney disease.
Heart and respiratory problems
A cough, chest pain, and shortness of breath that occurs during lupus may be:
- pericarditis (inflammatory disease of the pericardium or envelope of the heart),
- pleurisy (inflammation of the pleura formed of two membranes surrounding the lungs, with or without the production of a fluid).
Rare neurological or psychological disorders
Headaches, epilepsy attacks, hallucinations, confusion, mood disorders, disorders of depression, difficulty concentrating, memory problems, sleepiness… occur in about 10 % of cases of lupus.
Lupus cannot be cured, but it can be controlled. And if the disease is stabilized and taken care of early, it is possible to have almost a normal life with lupus, in particular thanks to treatments that are certainly heavy, but effective. These treatments make it possible to reduce inflammation and symptoms associated with lupus.
Treatment with corticosteroids (cortisone) in particular prednisone and methylprednisone is generally the most effective in treating systemic lupus. These drugs are administered in very high doses. However, today, doctors try as much as possible to gradually reduce the doses of cortisone until they eventually stop it, especially if the disease is under control.
Indeed, prolonged use of cortisone increases the risk of osteoporosis and diabetes. And above all, patients on cortisone are much more vulnerable to viral and microbial attacks.
Anti-inflammatory drugs and immunosuppressants
Doctors may also prescribe nonsteroidal anti-inflammatory drugs (NSAIDs) to relieve symptoms of mild lupus. Other drugs such as immunosuppressants can be used to treat severe and intractable lupus. Regular biological monitoring is often carried out throughout the treatment.
Finding the right treatment and the right dosage takes a lot of time because it’s all a question of dose. Doctors will then base themselves on the patient’s feelings, their tolerance to the treatment, and whether or not they have side effects.
The monitoring of lupus is, as with all rare diseases, fundamental: thus, the patient must feel confident with their doctor and feel listened to.
Hydroxychloroquine is a drug that can be given to patients with discoid lupus erythematosus and lupus erythematosus. Its effectiveness relates to the symptoms associated with this disease on the skin and joints. Treatment sometimes lasts several months.
Hydroxychloroquine can also be prescribed to prevent relapses. Among its contraindications: if the patient is hypersensitive to the active substances of the drug, in case of retinopathies, breastfeeding, in children under 6 years old, and in case of combination with citalopram, escitalopram, hydroxyzine, domperidone and piperaquine (increased risk of heart rhythm disturbances).
What is the life expectancy of someone with lupus?
The prognosis for survival in people with lupus has improved dramatically in recent decades. Life expectancy exceeds 95% at 10 years with appropriate care.
Jenny holds a Master’s degree in psychiatry from the University of Illinois and Bachelors’s degree from the University of Texas in nutritional sciences. She works as a dietician for Austin Oaks Hospital in Austin, Texas. Jenney writes content on nutrition and mental health for the Scientific Origin.