Organ affected: lungs

Living with lung IgG4-RD: a patient’s story

Sometimes IgG4-related disease (IgG4-RD) is found because a person feels sick for months and keeps looking for answers. Other times, it is found by accident.

Andy Provencher’s lung IgG4-RD story began the second way. He experienced a serious bicycle crash in which he broke bones and punctured a lung. During a CT scan at the hospital, doctors noticed shadows and areas that looked like masses in his lungs.

Suddenly, what had started as a bike accident became something much scarier. Andy was told the scan looked like an aggressive cancer. In the middle of the COVID pandemic, with hospital rules keeping loved ones away from many appointments, he had to move through tests, scans, biopsies, and see many specialists while carrying the fear that he might have lung cancer.

This lesson uses Andy’s story to help you understand one of the hardest things about lung IgG4-RD: it can look like cancer, lymphoma, sarcoidosis, vasculitis, infection, or other diseases before the right diagnosis is made.

From bike accident to cancer scare to IgG4-RD diagnosis

Andy Provencher’s story: IgG4-RD affecting the lung

Andy’s story

Andy was active and loved cycling. Before his accident, he did not think he had major symptoms. Looking back, he remembered feeling a little short of breath at times, but he thought it might be age, fitness, or needing to get back into shape.

In June 2020, Andy crashed while mountain biking. He broke his collarbone and several ribs, and he punctured and collapsed a lung. In the emergency room, a CT scan done for the collapsed lung showed shadows and mass-like areas. The first concern raised to him was cancer.

More testing followed. A PET scan, an imaging test that can show areas of high activity in the body, showed bright areas in his lungs and lymph nodes. Andy saw multiple specialists and heard several possible diagnoses, including lung cancer and lymphoma—and diseases with long names he’d never heard of: sarcoidosis, Castleman’s disease, vasculitis, and granulomatosis with polyangiitis.

Biopsies of a lymph node and lung tissue did not show cancer, but didn’t yield a clear answer, either. Andy describes this period as a long diagnostic ride, with one doctor after another and one possible diagnosis after another. He was close to starting chemotherapy for a diagnosis of cancer when a closer review of the lung pathology, an alert physician assistant, and evaluation by an expert turned the story in a different direction.

Finding an answer

A local rheumatology physician assistant raised the possibility of IgG4-related disease, or IgG4-RD, a condition in which the immune system causes inflammation, swelling, and sometimes scar-like tissue in organs.

Andy was referred to Mass General Hospital in Boston, where further review led to a clear diagnosis and treatment plan. Andy began treatment with prednisone, a steroid medicine that calms inflammation, and later received B-cell depletion therapy, which lowers certain immune cells involved in IgG4-RD activity.

He improved with treatment, but recovery was gradual. His lung nodules did not grow much, yet they also did not quickly disappear, and he continued to live with fatigue, stiffness, breathing limits, and the emotional weight of the experience.

What Andy’s story teaches us

Lung IgG4-RD may be found unexpectedly

A lung nodule is a small spot seen on imaging. A mass is a larger area of abnormal tissue. These words can be concerning because doctors often use them when evaluating the possibility of cancer.

As is true for so many organs that can be affected by IgG4-RD, IgG4-RD affecting the lung can look like cancer. Andy’s lung findings were discovered by accident, after imaging for a collapsed lung. This underscores the point that IgG4-RD affecting the lung can be quiet. Some people have cough, shortness of breath, chest discomfort, fatigue, or abnormal breathing tests. Others have few symptoms or none at all, and the disease is found when imaging is done for another reason.

Not every lung spot is IgG4-RD. Many conditions can cause lung nodules or enlarged lymph nodes. But IgG4-RD belongs on the list of possibilities when biopsies do not show cancer and the overall pattern begins to fit an immune-mediated disease.

IgG4-RD can mimic other conditions

In the lungs, IgG4-RD may appear as shadows, nodules, masses, or enlarged lymph nodes. These findings can make doctors worry about lung cancer or lymphoma.

Andy heard many possible diagnoses. Each one brought new fear and more testing. His story shows why a biopsy—a small tissue sample of the affected organ that can be studied under a microscope—is often so important. In his case, biopsies helped rule out cancer and eventually supported the right diagnosis.

For patients and families, this waiting period can feel like an eternity of being stuck between two worlds: still undiagnosed but filled with worry. That fear can be overwhelming.

Diagnosis may require persistence and the right team

Andy jokes about seeing many “-ologists,” but his point is serious. IgG4-RD often needs a team because it can involve different organs and resemble multiple other diseases.

A lung specialist, chest surgeon, blood specialist, radiologist, pathologist, and rheumatologist may all become part of the search for answers. In Andy’s case, his local clinician knew only enough about IgG4-RD to suspect it and make a referral to an expert center. That open-minded step changed his path.

Treatment can help, but healing may be slow

Andy’s treatment included prednisone, a steroid medicine that calms inflammation, and B-cell depletion therapy, which lowers certain immune cells involved in IgG4-RD.

He felt more like himself after treatment, but improvement was not instant. The imaging changes did not disappear right away. Some of the symptoms he had that were NOT related directly to the lung—fatigue, pain in his extremities, and the emotional struggle of wondering whether he was really finally on the right path—remained difficult. Inflammation may calm before scans look better. Fibrosis, or scar-like tissue, can be slower to improve and may not fully resolve—ever.

Progress is not measured only by a perfect scan. It may also mean the disease is no longer advancing, symptoms are better, organ function is protected, and daily life is becoming more possible.

Fatigue is more than being tired

Andy describes fatigue as something that can hit like a switch. One moment he may be part of family life; the next, his energy is gone.

Fatigue shows up as deep physical or mental exhaustion that rest may not fully fix. In IgG4-RD, it may relate to inflammation, sleep, medicines, pain, stress, mood, or the burden of living with a rare disease.

For caregivers, it helps to know this is not laziness. A person may want to participate, but the body may not cooperate that day.

Mental health is part of medical care

Andy speaks openly about mental health and the fear of being “locked” inside uncertainty. Diagnostic delays, cancer fears, repeated tests, physical symptoms, and the feeling that few people understand can all affect emotional well-being.

Treating IgG4-RD should include the whole person. Medicines may calm inflammation, but patients may also need support for anxiety, depression, trauma, sleep, family stress, and grief over the life they expected. Naming that need is not weakness. It is part of healing.

In this video, Andy Provencher and Dr. John Stone discuss the mental health and the importance of treating the entire person with IgG4-related disease.

Community can turn isolation into hope

Andy points to community as one of his most important supports. Meeting others with IgG4-RD can remind patients, “This is real. You are not imagining it. You are not alone.”

For someone watching Andy’s clip, the goal is not to compare every detail. The goal is to recognize shared feelings: fear, confusion, relief, frustration, hope, and the power of finding people who understand.