Article about a Still’s patient

Hello all,

There isn’t much published about Still’s disease so when a support group member posted this article last week I thought it might be nice to share with you.

If anyone reads or listens to press about Still’s disease and would like to share with us, please do so by using our contact form here at this site. We, here at the foundation,  believe that by sharing information research and benefits will increase for those that live with Still’s disease in their lives.

We thank you.

 

Article by:
Robert Holtzman,
For The Inquirer, Philadelphia, PA

Posted: Sunday, August 11, 2013, 8:52 AM

One in an occasional series on attempts to solve a medical mystery.

In early May, a young woman began feeling fatigued and developed a mild cough and sore throat. She attributed it to a mild cold and her busier work schedule and pushed through her symptoms. When she started vomiting and having fevers a week later, she went to her doctor’s office and, subsequently, the emergency room. She was told she had a stomach bug and a urine infection and was given an antibiotic. The drug did not help, and a week later, she was still having high fevers every afternoon, with sweats that soaked her clothes and simultaneous chills that rattled her teeth as she huddled in bed. When she noticed a rash on her arms and legs, she became even more concerned and went back to her primary doctor. They felt the antibiotic was the cause of the rash, but it continued after she stopped taking the drug. She noted that her rash seemed to appear and disappear at various times of the day and that it was most noticeable whenever she had a fever. Three weeks into her illness, and frustrated and exhausted by her symptoms, she returned to her primary doctor yet again. No answers could be gleaned from all the blood work and X-rays done previously. Her doctor decided to admit her to the hospital so she could get additional testing done more quickly, along with the input of several specialists. An extensive battery of tests revealed mild hepatitis (inflammation of the liver), possible pneumonia, and signs of significant generalized inflammation. But despite all these tests, no underlying reason for her illness could be found. More antibiotics were given to treat common and uncommon causes of pneumonia, but her fevers and rash persisted. The infectious-disease doctors asked question after question about where she had traveled and what hobbies and interests she pursued, trying to find a clue that might tip them off. The rheumatologists and dermatologists meticulously examined her joints and skin, looking for a pattern to tie all her symptoms together. CAT scans were done to look for a tumor or infection hidden deep within her body, which are common causes of Fevers of Unknown Origin. (FUOs) Then, a week into her hospitalization, blood tests returned suggesting she had been recently infected with two bacteria, Rickettsia ricketsii and Anaplasma phagocytophilum. Ticks transmit both bacteria, but different tick species transmit them. The woman had been in a wooded area only once in recent months – a brief hike in Fairmount Park along the Wissahickon Creek – several days after her initial symptoms began. Although the syndromes caused by both bacteria resembled this woman’s illness in a number of ways (R. Ricketsii causes a condition called Rocky Mountain spotted fever and A. Phagocytophilum is responsible for human granulocytic anaplasmosis), certain key parts did not fit, including the fact that she had not improved despite receiving for several days the antibiotic that treats both bacteria. Solution: Left with a mass of negative test results and still without any sound explanation for the woman’s symptoms, her doctors turned to the diagnoses of exclusion” on their long list of possible diagnoses. These conditions lack a specific diagnostic test that can confirm or refute their presence. Instead, all other possible diagnoses need to be excluded, and the constellation of symptoms is found to be consistent enough with the diagnosis of exclusion” to warrant treatment for it. For this woman, the diagnosis of exclusion that stuck out to her doctors was adult-onset Still’s disease. This is a disorder of inflammation characterized by daily fevers, arthritis, and a fleeting rash. It is the adult version of a condition called juvenile idiopathic arthritis, which occurs in teenagers and young adults. Both conditions are rare, with an estimated 500 cases combined every year in the United States. Her symptoms fit a number of the criteria used to help evaluate for Still’s disease, although she did not have any joint problems, and a biopsy of her skin rash was more suggestive of an infection like Rocky Mountain spotted fever than Still’s disease. Left with no better explanation, her team of doctors agreed she should start on steroids, the primary treatment for Still’s disease. Within two days, her fevers remitted and her rash disappeared. Her hepatitis also resolved quickly, and the markers of inflammation improved, although they remain abnormal even weeks into care. Blood tests done several weeks later suggested she had not been truly infected with the bacteria that cause Rocky Mountain spotted fever or anaplasmosis. She is now slowly recuperating from her month long (ordeal), which left her quite weakened, though she has been able to return to work. She continues to take steroids; the dose is slowly being reduced with the hope that this was an isolated flare-up of her Still’s disease, though about two-thirds of people with the condition have at least one such reoccurrence.
She is grateful for the care of her medical team, though her case highlights that, despite the innumerable advances in medicine, there are still many medical mysteries to be solved, especially involving Fevers of Unknown Origin. Dr. David Holtzman is an infectious-disease fellow at the Hospital of the University of Pennsylvania.

Robert Holtzman

I would like to end this post with the comments that not all patients get all symptoms and not everyone has the same intensity of disease or reaction to treatments within any given time frame. Though the disease has many common features it can vary from person to person as to it’s severity and response to medications.

Please visit the other areas of our site for additional information about  Still’s and other conditions.

Patricia L. Boerner,

President, ISDF, Inc.