When 21-year-old Manisha (name changed) first walked into a doctor’s office at the age of 15 with complaints of chronic fever, fatigue, and weakness, doctors suspected autoimmune haemolytic anaemia, a blood disorder in which the immune system attacks the body’s red blood cells. Manisha was immediately put on steroids and seemed to improve.
Yet, every time doctors tried to taper her off from the medication, the symptoms returned with more effect. It took four years and multiple consultations before Manisha finally received a proper diagnosis of her condition — systemic lupus erythematosus (SLE) often referred to simply as ‘lupus’.
Sadly, says Dr S Sham, Consultant Rheumatologist, Kauvery Hospital, Chennai, Manisha is not alone in facing this long road of misdiagnosis and lengthy use of steroids, risking such complications as osteoporosis, eye conditions such as glaucoma or cataracts, high blood sugar, and so on. “The presentation of lupus is so varied, it can start as anaemia, rashes, joint pains, fever, and so on. This means that many patients could get stuck with various misdiagnoses for years,” observes the doctor.
Not only is the condition difficult to diagnose, there is also a widespread lack of awareness among the public, and even in the medical fraternity, says Dr Sham. This is problematic because delayed diagnosis can mean significant and sometimes irreversible damage to various organs in the body.
Lupus is an autoimmune disorder, meaning that it involves the body’s own immune system attacking tissues and organs. Lupus can affect many different systems in the body, causing aches and pains, creating skin problems and affecting organs such as kidneys, heart, and lungs. While anyone of any age can have lupus, it is much more common among women than men, and most often diagnosed in the age group of 15-45 years.
It is currently not known what causes lupus, though a range of genetic and environmental factors are suspected to be involved in triggering the disease. Because lupus is seen to affect many more women than men, it is also suspected that certain hormones play a role, though this has not been proven.
While not a very common condition, estimates suggest that four lakh people are diagnosed globally with lupus every year. Studies also suggest that the number of cases in low and middle income countries are on the rise. In India, it is estimated that there are a few lakh people living with lupus.
Dr Sham points out that there is quite a large variation among patients in the ways lupus presents. However, certain common symptoms do arise in many cases.
“One common symptom of lupus is a continuous fever lasting many days. Also, many lupus patients may have joint pains, but without the swelling that accompanies conditions like rheumatoid arthritis,” says the doctor.
He adds that the skin is often affected by lupus, with patients reporting rashes as well as frequent mouth ulcers, particularly on the palate or roof of the mouth. The condition also commonly involves excessive hair loss or alopecia.
Other symptoms include fatigue, chest pain, difficulty breathing, dizziness and confusion, anaemia, blood clots, and issues with the kidney, heart and lungs, among others.
“But it’s important that doctors look for a combination of these symptoms. Just having hair loss does not mean lupus,” cautions the doctor. Besides these symptoms, doctors also conduct a series of clinical tests including blood and urine tests, kidney and liver tests, and tests for particular antibodies associated with lupus.
Early and proper detection of lupus can sometimes mean the difference between life and death, says the doctor. “We had one patient who initially came in with joint pain and whose investigation for SLE was positive. But there were not enough clinical symptoms,” he says. However, when she returned some days later with a high fever, they were quickly able to diagnose a life-threatening complication because of the previous investigations. He says, “Because of her high fever, the diagnosis could have easily gone down the route of infections like dengue or malaria, which would have wasted vital time needed for her proper treatment.”
Try to read up on lupus online and it can seem a terrifying ordeal. “Many of our patients initially think we’ve handed them a death sentence,” says Dr Sham. However, while there is no cure, the long-term management of lupus has come a long way, he adds.
In the pre-2000s, steroids were seen as the only long-term treatment option for lupus, though their long-term use came with various side effects. However, more recently, a range of effective and safe immunosuppressants have been discovered as useful for managing lupus. Originally developed for patients receiving organ transplants, these have now been found to be more sustainable substitutes for steroids, and are hence called steroid-sparing medications.
In the last few years, further advancements have led to the development of intravenous medications called biologics, which help to target certain parts of the immune system instead of generally dampening immune function. “While one of these called Rituximab is available in India, two others – Belimumab and Anifrolumab – are expected to be available in India in the future,” explains Dr Sham. Looking forward, there is a lot of research going into targeting the immune system at the molecular level in order to finetune lupus treatment, he adds.
Thanks to these advances, the doctor says, a long, healthy, and functional life can now be assured for many lupus patients. “Earlier, many in the medical fraternity believed that women with lupus should not get pregnant, because it was an unnecessary risk. These days, many of the young women who come to us are able to lead very functional lives and even get pregnant if they want to. Regular follow-ups and strict adherence to treatment schedules is necessary. If managed properly, lupus need not get in the way of a long and healthy life,” says Dr Sham.
This article was published in association with Kauvery Hospital.