Organ affected: Retroperitoneal

Patient stories: Living with retroperitoneal fibrosis

Retroperitoneal fibrosis (RPF) often begins with symptoms that do not point immediately to a rare immune-mediated disease. Some people experience sudden severe pain. Others have few symptoms until imaging reveals a problem with the kidneys or ureters or a mass in some other part of the retroperitoneum. Many are first evaluated for more common conditions such as kidney stones, cancer, gallbladder disease, or back injuries.

The stories in this lesson show that there is no single path to diagnosis. Although each person's journey is unique, common experiences are shared among many with retroperitoneal fibrosis: pain, uncertainty, delayed diagnosis, multiple tests and visits to different specialists, concern about cancer, worries about kidney damage, invasive procedures, and relief when a diagnosis finally provides answers.

Patient story: Donna Shipp

A surprise finding becomes a long diagnostic journey

Donna Shipp, a nurse from Massachusetts, first noticed pain after eating and assumed it might be a gallbladder problem. An ultrasound unexpectedly revealed severe hydronephrosis, a buildup of urine caused by blockage of the ureter. Further imaging showed a ureteral obstruction that radiologists described as "highly suspicious for malignancy."

Over the following months, Donna underwent multiple scans, biopsies, and consultations. Despite repeated testing, doctors could not determine whether the obstruction represented cancer or another condition. The function of the affected kidney continued to decline, and eventually surgeons removed the kidney and part of the ureter to obtain a definitive diagnosis.

The pathology examination of the kidney ultimately showed no cancer. Combined with elevated inflammatory markers, elevated IgG4 levels, the details of the pathology findings, and extensive retroperitoneal fibrosis found during surgery, physicians concluded that IgG4-related disease was the cause.

What we learn

Donna's experience highlights how difficult RPF can be to diagnose. Imaging suggested cancer, biopsies were inconclusive, and her blood tests provided clues but not definitive answers. In her case, the diagnosis emerged only after surgery revealed extensive fibrosis throughout the retroperitoneum. Her story also demonstrates how kidney damage can occur even when symptoms are relatively limited.

Patient story: John Durcan

A sudden pain during a walk

John Durcan was taking his usual walk around Jamaica Pond in Boston when he suddenly developed severe stabbing pain in his left flank.

The pain felt exactly like what he imagined a kidney stone would feel like. A month later, routine blood work showed worsening kidney function. Imaging revealed an abdominal mass and hydronephrosis caused by ureteral obstruction.

Doctors placed a ureteral stent to relieve pressure on the kidney and performed a needle biopsy. Although the biopsy suggested the mass was not cancer, it did not provide a definitive diagnosis. Eventually he was diagnosed with retroperitoneal fibrosis associated with IgG4-related disease.

John initially received prednisone but saw little improvement. Later, treatment with B-cell depletion therapy led to major improvement and allowed removal of a long-term ureteral stent.

What we learn

John's story illustrates a classic presentation of RPF. Fibrosis around the aorta trapped the ureter, leading to hydronephrosis and sudden flank pain. His experience also shows how a biopsy may help rule out cancer while still leaving uncertainty about the exact diagnosis.

Patient story: Serkis Kaya

Years of pain before answers

Serkis Kaya's symptoms began with a burning sensation in his lower back that quickly progressed to severe stabbing pain. Initially, doctors thought the problem might be a herniated disk or something of that nature. Although he eventually underwent surgery for an inguinal (groin) hernia, his pain continued to worsen.

After months of escalating symptoms and repeated emergency room visits, a CT scan revealed a retroperitoneal mass. A biopsy ruled out cancer and led to a diagnosis of retroperitoneal fibrosis.

His kidney function declined, requiring the placement of a ureteral stent to free the urine flowing from his right kidney. Over the next decade, Mr. Kaya underwent repeated stent exchanges and long-term prednisone treatment. Although these treatments helped control symptoms, they also caused significant side effects, including steroid-induced diabetes. The pain from his stent was treated with daily narcotics.

Finally, years later, reevaluation of the kidney biopsy that had been taken 12 years earlier demonstrated that the correct diagnosis was IgG4-related retroperitoneal fibrosis. To his amazement and that of his daughter Lisa, treatment with B-cell depletion therapy improved his condition quickly, allowing for the removal of the stent that had affected his quality of life for years, the tapering of his prednisone, and the discontinuation of his chronic narcotics.

What we learn

Mr. Kaya's story demonstrates the primitive treatment of RPF that was typically employed before IgG4-related disease became a known entity and was as widely recognized as it is today. His experience highlights how advances in understanding IgG4-RD have helped physicians better identify the cause of some cases of retroperitoneal fibrosis and offer more targeted treatments.

Common themes from IgG4-RD patients

Although Donna, John, and Serkis had different experiences, several themes appear repeatedly.

• Symptoms can vary widely. Pain after eating, sudden pain in one of the flanks, a chronic back pain – and even no clear symptoms at all – are all common.

• Cancer is often considered first. Imaging frequently reveals a mass or abnormal tissue that can resemble malignancy.

• Kidney and ureter problems are common. Hydronephrosis and ureteral obstruction were major features in all three stories.

• Diagnosis may take time. Multiple scans, specialist visits, and procedures (particularly biopsies) are often needed.

• Biopsy can be challenging. Obtaining enough tissue from retroperitoneal lesions is not always easy.

• Treatment often requires multiple approaches. Stents, steroids, nephrostomy tubes, surgery, and immune-targeted therapies may all play a role.

• Support matters. Meeting others with the disease helped patients feel less isolated and brought about greater understanding of their condition.

Lesson summary

These stories show that retroperitoneal fibrosis is often discovered only after a long and complicated medical journey. Patients may be told they could have cancer, kidney stones, or another condition before the true diagnosis becomes clear. Yet these stories also demonstrate an encouraging reality: once RPF is recognized and treated appropriately, many patients experience significant improvement, stabilization of kidney function, relief of symptoms, and a clearer path forward.