The Fascinating Story of the Immune System and Rheumatology

On my first encounter with a new patient, I always ask, “What made you come to a rheumatologist's office?” Many of the times I get a confused look on my patient's face. These are some of the common answers I get: “I am not sure”, “my primary care doctor wanted me to see you”, “No one knows what is going on with me”. Some of the well-informed patients say: “I have inflammation in my body”, “ I might have an auto-immune disease”. I do not blame them. Rheumatic diseases are rare and complex conditions to understand. Many doctors are even confused about these rare chronic conditions, which leads to misdiagnosis and delayed diagnosis of these conditions. This has significant long-term complications. 

Rheumatic diseases are rare conditions due to a dysregulated immune system. The primary function of the immune system is to protect us from foreign organisms such as viruses and bacteria. The immune system has multiple arms, which enables them to tackle the different pathogens in different ways. You could think of it as the army protecting the country, such as the navy protecting invaders from the sea, the air force protecting us from the sky, and so on. If you are infected with a virus the T-cells are important in killing the virus. To effectively kill the virus and prevent future attacks, the B cells make antibodies. These two players in the immune system (T cells and B cells) work in a coordinated fashion to keep us safe from infections. The vaccines to prevent future infections activate both B cells and the T cells to produce immunoglobulins, which are like glues that attach the pathogen and call for help from other players in the immune system which effectively kills the pathogen before they could attack our cells. Due to these various arms, there is significant redundancy in the immune system. This makes sure the pathogen is effectively killed without causing major harm. As we will discuss later, this redundancy could also make the immune system-related diseases more complicated.

When there is no attack by a pathogen, the immune system is in a dormant stage, waiting quietly for the pathogen. During an attack by a pathogen, the immune system is activated. Such activation is initiated by the pathogens and there are various signals that the immune system uses to alert the various arms of the immune system. These signals are referred to as cytokines. This is very important to turn on the immune system and later to turn it off when the invaders are cleared. These chemical mediators are also very important when we talk about immune-related diseases and their treatments. Many of the treatment options to treat autoimmune diseases block the function of these cytokines.

After a pathogen is killed by the immune system, the activated immune system is shut off. The debris created during the process of killing is cleared by the immune system (cleans its own mess!). This makes sure there is no scaring. When this process of shutting off the immune system and cleaning process is dysregulated, you are at risk of having an auto-immune disease. In some patients with auto-immune rheumatic diseases, the immune system is also confused between foreign invaders and self, leading to activation of the immune attacks causing inflammation. Such immune attack continues and does not turn off leading to continued inflammation and leading to damage to an organ.

The various diseases of the immune system depend on the organs involved. In patients who have rheumatoid arthritis, inflammation and damage occur in various joints. Lupus is much more complex where the inflammation occurs in multiple organs including kidneys, skin, heart, and lungs. Due to this complex nature, these diseases present with multiple symptoms making the diagnosis challenging. One disease that deserves special attention is ankylosing spondylitis. This is a chronic condition where there is inflammation in the low back joint called the sacroiliac joint. This causes chronic low back pain in young patients typically less than 45 years of age. The available scientific data suggests that it takes 5 to 10 years from the onset of symptoms to the diagnosis of this condition. It is a missed opportunity. Many patients who have such delayed diagnoses suffer from complications related to this condition. This leads to limited joint mobility and disability. 

Most rheumatic diseases are chronic conditions that require long-term care. It requires coordinated care by multiple doctors. You need to be on multiple medications. The medications used to treat rheumatic diseases could lead to side effects, which require regular visits to a rheumatologist and blood tests. With such complexity of care, many of you with the rheumatic disease might be overwhelmed. If you feel so, you are not alone. Be open with your doctors including your primary care physician and your rheumatologist. They are your best resource to help you understand these chronic conditions. They could help you understand the benefits and risks related to the treatment and find the right treatment option for you.

When you google about your condition, you might be overloaded with information, which might be more confusing to you. It's always best to go to reliable sources to learn about your disease condition. Verify such online information with your doctors. This helps reduce misinformation and understand whether that information applies to you or not. As we discussed earlier, the immune system and diseases are very complex. You have not been through medical school and further training to understand all of these. You need help from medical expertise to help understand these diseases. The more you understand about the diseases and the available treatment options the better care you get. Hence, be your own health advocate and get the best care you deserve.