Lupus, Sjögren's and scleroderma

What are these conditions?

Lupus, Sjögren’s syndrome and scleroderma are a group of autoimmune diseases, also called connective tissue diseases. In all of them the immune system attacks the body’s own healthy tissues, but each has its own pattern. All of them are long-term and have no cure, but treatment can control the symptoms and help prevent damage to organs.

Lupus (systemic lupus erythematosus). NIAMS writes that lupus can affect the skin, joints, heart, lungs, kidneys, blood cells and brain, and it goes through flares and periods of calm. Women are affected about nine times as often as men, and most diagnoses are made between ages 15 and 45. The NHS says it is not contagious.

Sjögren’s syndrome. The glands that make tears and saliva are affected. The NHS writes that the most common symptoms are dry eyes and a dry mouth, and the condition is seen more in women. Most people with it have a normal life expectancy.

Scleroderma. NIAMS describes it as an autoimmune disease in which extra collagen makes the skin, and sometimes other tissues, tight and thick. It has two main forms: the localised type, which stays limited to the skin and the tissue under it, and systemic sclerosis, which can also affect blood vessels and internal organs. The NHS writes that systemic sclerosis affects mostly women and usually starts between ages 30 and 50.

Symptoms

Lupus: joint pain and swelling, severe tiredness that does not improve well with rest, fever, hair loss, mouth ulcers, skin rashes (including a rash across the nose and cheeks that gets worse after sun), colour change in the fingers in the cold (Raynaud’s), headache, and sometimes chest pain when breathing deeply. If the kidneys are affected, signs such as foamy urine and swollen legs appear (NIDDK).

Sjögren’s: dry eyes and mouth, muscle and joint pain, a lot of tiredness, dry, itchy skin, a persistent cough, and sometimes swelling of the glands in the cheeks, neck or jaw (NHS). Severe dry mouth can also lead to tooth decay and gum disease.

Scleroderma: Raynaud’s, which is often the first sign of the limited systemic type, tightness of the skin of the hands and face, sometimes heartburn and difficulty swallowing, and in the diffuse type the heart, lungs or kidneys may be affected, with shortness of breath or high blood pressure (NHS).

Who should see a doctor?

If you have several of these symptoms together and no other cause has been found for them, it is better to see a doctor. The NHS writes that because the symptoms of lupus are similar to those of other conditions, it is not easy to diagnose, and early diagnosis helps with better management. The ACR also counts mouth ulcers, hair loss, suspicious rashes and eye changes among the signs that may call for a referral to a rheumatologist.

How is it assessed?

NIAMS writes that no single test confirms lupus. The doctor puts together your history and family history, an examination and tests.

• ANA: almost everyone with lupus has a positive ANA, but healthy people can also be positive. So a positive result alone is not a diagnosis. When it is positive, more specific tests are requested (such as anti-Smith and anti-dsDNA antibodies and antiphospholipid tests).
• Blood cell count, kidney tests and urine tests: to look at platelets and blood cells and for protein leaking into the urine. In lupus these tests are repeated over time.
• Sometimes a biopsy of the skin or kidney.
• For Sjögren’s, the NHS lists blood and eye tests, a saliva test and sometimes a lip biopsy.
• For scleroderma, regular blood pressure checks and tests to assess the organs are suggested (NHS).

What does treatment look like in general?

According to NIAMS, the aim of treatment is to control symptoms, prevent flares, keep disease activity low and prevent organ damage.

• Lupus: anti-inflammatory medicines, hydroxychloroquine (for tiredness, skin and joints, and NIAMS says it may also keep flares from coming back), steroids at the lowest effective dose, and, in more severe cases, immune-suppressing or biologic medicines. If the kidneys are affected, NIDDK describes treatment with steroids and another immune-suppressing medicine and blood pressure control, and stresses that kidney scarring is irreversible and early diagnosis matters.
• Sjögren’s: drops and gels to keep the eyes and mouth moist, medicines that stimulate tear and saliva production, and sometimes a short course of steroids for severe joint pain (NHS).
• Scleroderma: medicines to improve blood circulation, immune-modulating medicines, treatment of symptoms (heartburn, blood pressure, pain), and physiotherapy to keep the skin and muscles supple (NHS).

Hydroxychloroquine needs regular eye checks. The NHS links long-term use with eye check-ups to detect retinopathy, and the AAO writes that a baseline examination should be done in the first few months, and yearly screening should begin no more than five years later (in people without special risk factors). Your doctor may give you a different schedule.

Do not stop or reduce any medicine, especially steroids, without your doctor’s advice.

What can you do?

• Protect yourself from the sun: a hat, covering clothing and sunscreen. In lupus the NHS names sunscreen with an SPF of at least 50.
• Stop smoking. The NHS says smoking makes lupus worse, and NIAMS recommends quitting to reduce the risk of heart disease.
• Learn the signs of a flare: more tiredness, joint swelling, rashes, fever, abdominal pain and headache (NIAMS).
• In Raynaud’s, keep your hands and feet warm and avoid the cold and sudden changes in temperature (NHS).
• In Sjögren’s, care of the mouth and teeth (brushing with a fluoride toothpaste and regular dental visits) and regular eye checks matter.
• Talk to your doctor before starting new exercise.
• Talk to your doctor before pregnancy. NIAMS says some lupus medicines are not safe in pregnancy, and the full list of medicines should be reviewed beforehand.
• Do not treat diet and supplements as a replacement for treatment, and talk to your doctor before trying them.

When to get help urgently

• Foamy urine, or new swelling of the legs and ankles, or high blood pressure in a person with lupus. NIDDK counts these as possible signs of kidney involvement and says early diagnosis and treatment can protect the kidneys. Tell your doctor the same day.
• Sudden chest pain or shortness of breath. Call Iran’s emergency number (115).
• Fever or a sign of infection while taking an immune-suppressing medicine. Tell your doctor the same day.
• A sore, severe pain, redness or pus on a fingertip. SRUK says a finger ulcer in scleroderma should be checked soon, because infection can make it worse. See a doctor the same day.
• Blurred vision or a red, painful eye: go to an eye doctor or an eye emergency department the same day. Sudden loss of vision: call 115 or go to the emergency department.

See the full list in the guide “When should I call urgently?”