Vasculitis can mimic common ailments

TRISHA (16), a Class X student, was preparing for her exams when she came down with fever and sore throat in January this year. Her parents and the treating doctor assumed it to be flu. She was prescribed antibiotics. But within two days, she developed rashes on her legs and within a week, she was experiencing extreme joint pain. Hospitalised at that point, her medical investigations were on and she was continually given antibiotics. However, her condition only worsened. She was eventually referred to the PGIMER, Chandigarh, where she was diagnosed with vasculitis, an autoimmune disease that causes inflammation in blood vessels.

Blood is supplied all across the body through vessels. If any of the vessels gets inflamed (swollen), it can be fatal, as this inflammation affects the wall of the blood vessels and can cause narrowing or complete blocking of these vessels. This results in decreasing or stopping of the blood supply to the organ or tissue supplied by that vessel. Lack of blood flow leads to function impairment or infarction (death) of that organ. Persistent inflammation can sometimes cause the wall of the vessel to become weak, resulting in ballooning that might rupture, causing haemorrhage.

Vasculitis can affect a person of any age or sex. It can affect almost any organ due to either blockage of the inflamed vessels or rupture of the weakened vessel walls. It has various sub-types affecting different age groups, like giant-cell arteritis that affects the aorta in older patients, whereas Takayasu arteritis affects young adults and Kawasaki disease affects children.

Symptoms: Vasculitis can be a primary disease or can be the secondary symptom to other autoimmune conditions like rheumatoid arthritis, systemic lupus erythematosus, etc. Symptoms of various sub-types depend on the type/size of the vessel involved. These can be small vessel, medium vessel and large vessel vasculitis and can cause sinus/lung/kidney/skin or neurological diseases. Other systems like heart, nervous system, intestines, lungs, and kidneys may also be affected.

Symptoms include fever, weight loss, fatigue, a rapid pulse, and diffused aches and pains that are difficult to pinpoint, joint pains and/or swelling, etc. The severity ranges from mild to life-threatening. Many a time, these manifestations occur simultaneously in various organs, giving the clue to this diagnosis.

Causes: Though largely unknown, as in Trisha’s case, sometimes hepatitis B and C infection can cause some sub-types of vasculitis. At other times, it may develop as a reaction to a drug or cocaine.

Genetic factors: Recently, a genetic variant of medium-vessel vasculitis called DADA2 vasculitis has been identified.

Initially, DADA2 was thought to be a disease of childhood. However, recent research at the PGI, Chandigarh, showed that it occurs well into adulthood. As per this research, this disease has been seen more commonly in the Aggarwal/Jain community, particularly in North India. Awareness about this fact, particularly among health-care professionals in North India, is very important.

This disease presents with recurrent strokes that start at a young age. If this disease is diagnosed in time and appropriate treatment (specific injections) is started immediately, then strokes do not recur, and other symptoms also improve. Sometimes, these patients show symptoms of skin rash, joint pains, abdomen pain and nerve damage. Hence, awareness about this disease, especially among general physicians, can make the diagnosis easier.

Largely a pattern recognition, the final diagnosis may require a biopsy or imaging studies. Commonly done biopsies include skin, temporal artery, kidney, sural nerve, and rarely, open-lung biopsy. Other tests include blood tests (antineutrophil cytoplasmic antibodies or ANCA), imaging techniques (MRI/PET scan for Takayasu’s arteritis) and CT angiography (Polyarteritis nodosa). The choice of investigations will depend upon the sub-type.

Vasculitis is not a largely hereditary disease though the genetic variant DADA2 puts children at a slightly higher risk. However, this risk is not absolute. Vasculitis is also not transmissible from one person to another.

Treatment options: Since it is an auto-immune disease, most patients require immunosuppression medications. Steroids and immunosuppressive drugs are the cornerstone of vasculitis therapy. The type and duration of immunosuppressants depend upon the sub-type and organs affected. Various immunosuppressive drugs used in treatment are Cyclophosphamide/Rituximab/Methotrexate/Mycophenolate and Azathioprine.

Curability chances: Vasculitis may be temporary or can be a lifelong problem. However, most types are treatable if detected early enough and before substantial organ damage has occurred. With current treatment strategies, the outcome for patients is often quite good and patients can lead near-normal life. Trisha was able to appear for her board exams and scored over 90 per cent marks. As patients are on immunosuppressant drugs that weaken their immunity, they have to take certain precautions, like avoiding contact with persons having commonly transmitted infections, generally avoid crowded places, cover face with a mask, and eat home-cooked food.

Recurrence or relapsing: In some sub-types, relapse can happen. If the symptoms come back or new symptoms develop, then the patient must consult the specialist. New symptoms may indicate a flare-up or complications of treatment such as infections. However, those affected should never self-medicate. Also, increasing or decreasing medications without consulting a physician can cause more problems.

Diet: No particular diet affects or raises susceptibility to vasculitis. However, patients must keep a check on calorie intake and avoid a high-calorie diet, particularly those on steroids as they are at risk of weight gain and can develop diabetes. Smoking may also exacerbate the symptoms.

In untreated and undiagnosed cases, the chances of fatality may be very high. However, with regular treatment and monitoring and a healthy lifestyle, vasculitis is very much manageable, if not curable, and the patient can lead a normal life.

— The writer is professor, Department of Internal Medicine, PGIMER, Chandigarh