Mixed Connective Tissue Disease Flares: Symptoms and Treatment

Mixed connective tissue disease is a rare inflammatory autoimmune disorder that overlaps with several other rheumatic conditions—lupus, scleroderma, polymyositis, and rheumatoid arthritis. If you’ve been diagnosed with mixed connective tissue disease, you may wonder how to manage disease flares or MCTD exacerbations.

An MCTD flare is marked by an increase in symptoms that can vary in severity. These symptoms can include pain, fever, skin rash, and extreme fatigue, depending on which overlap autoimmune conditions your MCTD more closely resembles.

Common symptoms for all MCTD patients are pain or inflammation in joints, Raynaud’s phenomenon, skin rashes in sun-exposed areas, hair loss, hand swelling, fatigue, and weakness of the proximal muscles of the neck, upper arms, and upper legs.

While you may experience some degree of MCTD symptoms daily, a flare is an exacerbation of your symptoms that is worse than what you experience in your day-to-day life or a new symptom.

Living with MCTD can be unpredictable. You may experience periods of time when your symptoms are well controlled and your disease is quiet, perhaps even barely noticeable. You may also experience periods of time where your symptoms become worse, or you experience new symptoms. When this exacerbation period occurs, you are in a flare.

Flares can be mild and short, lasting just a few days. Or they can be more severe, involving major organs and lasting for longer periods of time. Treatment of your flare depends highly on the symptoms that are present.

One-third of patients with MCTD will experience a mild disease course and go into remission. It has been reported that up to 40% of patients may enter sustained remission of symptoms with proper treatment. However, other patients may experience flares that come and go.

Being in a flare can affect your daily activities. If you are experiencing arthralgia, joint pain, and inflammation, you may have difficulty exercising or moving around. Other symptoms of a flare could include rashes that are a bit more manageable, and though less likely, a more severe flare could involve the heart, lungs, and kidneys.

To live well with MCTD, it’s important to understand the symptoms and possible triggers of flares so that you can properly manage your health.

Frequent Symptoms

The most common and frequent symptoms of MCTD that can occur during a flare are dependent upon which overlap syndrome your disease course more closely follows. Almost all patients with MCTD will experience joint pain.

You may also have inflamed muscles that cause weakness and soreness around the shoulders and hips. Having swollen hands and fingers is common, as is a butterfly-shaped rash on the cheeks and bridge of the nose. 

Similar symptoms or flares are seen in undifferentiated connective tissue disease, an autoimmune disorder with similar overlap syndromes but doesn’t fulfill enough criteria to reach a specific diagnosis of lupus, MCTD, or any of the other connective tissue disease.

Classic Symptoms of MCTD

Though MCTD is often difficult to diagnose because of its overlap with other syndromes, four classic symptoms are characteristic of the disease:

• Raynaud’s phenomenon: Often the first sign of the disease, this constriction of the blood vessels in the fingers and sometimes toes causes loss of feeling and color changes.
• Swollen fingers are sometimes temporary but can progress into a state of hardened skin with limited movement.
• Inflamed joints and muscles may be present.
• Pulmonary hypertension may be noted, which is high blood pressure in the blood vessels of the lungs.

Nearly half of all MCTD patients may experience lung involvement at some point, leading to difficulty breathing. This difficulty in breathing can be caused by pulmonary hypertension or interstitial lung disease. 

Rare Symptoms

Though MCTD usually has a mild course, the disease can affect major organs. It is important to continue to be cared for by a doctor who can monitor your health and watch for any of these symptoms:

• Esophageal: With MCTD, you may experience esophageal trouble that manifests as heartburn or difficulty swallowing solid foods.
• Neurological: Ten percent of MCTD patients may experience neurologic abnormalities such as vascular headaches, a mild form of meningitis, or sensory disturbance in the limbs (pins and needles sensations). 
• Cardiac: Sometimes, the heart can be weakened, leading to heart failure. Symptoms of failure include shortness of breath, fatigue, fluid retention. You can also develop pericarditis, an inflammation of the sac around the heart.
• Renal: In 25% of patients with MCTD, the kidneys are affected. However, the damage is usually mild compared to the damage caused by lupus.

Causes of a Flare-Up

MCTD may be marked by periods of no symptoms followed by exacerbations. Not much is known about triggers, but they may include pregnancy, stress, emotional distress, cold exposure, switching or stopping medications.

Pregnancy can cause stress on the body and trigger a flare, either during pregnancy or after the baby’s birth. One study found that 26.7% of patients who had stable MCTD at the time of conception went on to relapse during the pregnancy.

If you are pregnant or plan on becoming pregnant, it is best to talk to your doctor to ensure that you are receiving the proper treatment to keep you and your baby healthy and safe.

Stress can be a major trigger of a flare. Most patients report that stress is one of the most significant factors in their illness. Stress can be caused by work, financial worries, traumatic life events, and a general sense of trying to do too much in too little time.

Cold exposure can cause an exacerbation of current or past symptoms, especially those of Raynaud’s phenomenon.

Following your doctor’s treatment plan for MCTD is key to reducing the frequency and severity of flares, even if you feel good or reach a state of remission.

In one study, lupus patients who did not comply with their treatment plans were at higher risk for flares. Patients prescribed hydroxychloroquine who reached an inactive state of the disease but continued their medication experienced a 74% lower risk of developing severe flares than those who went off the drug.

Treatment

Symptom management depends highly on which symptoms are present. Treatment for MCTD flares may also be based on the management of other autoimmune disorders.

If arthritis signs are present, disease-modifying antirheumatic drugs (DMARDs), may be recommended. If there are lupus symptoms, antimalarials may be used. There’s no one-size-fits-all answer for MCTD.  

Medication

While there is no uniform treatment for MCTD, some common drugs are prescribed for various symptoms. The aim of prescription medicines for MCTD is to control the disease from preventing further damage and ease symptoms.

Most people can be well treated with systemic steroids, disease-modifying antirheumatics like hydroxychloroquine or methotrexate, or immunosuppressive drugs like cyclosporine. You may also be advised to take NSAIDs for pain and inflammation. Whatever state you are in, your doctor will work with you to find the best treatment plan.

Lifestyle Changes

Living well with MCTD requires you to treat yourself well. Following your doctor’s treatment plan is key, and so is following good practices to keep your body healthy and strong.

Learning to rest when you need it is good for preventing over-fatigue. Resting when necessary does not mean you’re lazy. It means you understand your body’s limits.

It is also important to eat a healthy diet. Anti-inflammatory diets have been shown to help with symptoms. Avoid smoking and practice stress-reducing activities such as exercise, yoga, meditation, even reading a book.

If you are currently in a flare, you may need to reduce the amount of exercise or take it slow. You do not want to cause more stress on your body.

Support Groups

Living with an unpredictable disease like MCTD can be worrying. Talking to others experiencing similar symptoms can help you feel less alone and help relieve some of the stress.

Places like the Lupus Foundation of America, the Scleroderma Foundation, the Myositis Association, the Arthritis Foundation all have resources to help you find support groups. These groups may be in person or online. Your local hospital may also have support groups as well. Check with them to find out.

When to See a Doctor

If you are in a flare that is not calmed by self-care measures like rest, anti-inflammatory medicines, or avoiding known triggers like stress or cold, you should call your doctor. You may need an adjustment to your medication. If you are experiencing recurring or frequent flares, you may also require further treatment.

You should also see your doctor if you experience symptoms that are worse than before or if your symptoms become debilitating. It is also important to keep all doctor’s appointments.

Continue to see your doctor regularly regardless of how you feel. It may be tempting to skip an appointment if you feel well, but maintaining regular care is important for good outcomes with MCTD.

A Word From Verywell

Living with an unpredictable disease like MCTD can feel overwhelming at times. When you feel overwhelmed, talk to someone—a friend, a loved one, your doctor, or a therapist. You do not have to face the disease alone. In fact, that may cause more stress.

Adjusting to the unpredictability of the ups and downs of MCTD can be difficult, but the more you understand what triggers your symptoms and flares, the more you can feel confident that you can lead a good, healthy life.

Taking charge of your health by maintaining your treatment plan and avoiding triggers known to cause flares can help you feel more in control of your life.