Doctors in the Syrian capital, Damascus, have reported a very rare medical condition discovered in a Syrian woman in her twenties. The Al-Mujtahed Hospital management in Damascus announced the diagnosis of a rare disease (macrophage activation syndrome), confirming it as the fourth case of its kind worldwide.
Ahmad Abbas, the director of Al-Mujtahed Hospital, stated that a medical team from the rheumatology and arthritis department successfully treated a rare condition in a 26-year-old female patient who was referred to the hospital after being diagnosed with "Still's disease," a rare type of arthritis.
The patient exhibited symptoms of persistent peak fever reaching 40 degrees Celsius for over a week, a non-itchy rash, persistent joint inflammation lasting more than two weeks, and pharyngeal pain, alongside secondary supportive criteria including enlarged lymph nodes, elevated liver enzyme levels, and negative ANA and Rf tests, according to the local news website "Ather Press," which is close to the Syrian regime.
Abbas indicated that the diagnosis and treatment of the case took approximately eight months, emphasizing that early diagnosis contributed to saving the patient's life, as this type of disease can lead to immune deficiency and severe inflammation that can result in death.
He explained that macrophage activation syndrome is a "dangerous systemic complication that threatens life, which can occur due to various reasons including inflammatory diseases and autoimmune disorders, especially in patients with Still's disease, characterized by uncontrolled proliferation and activation of macrophages leading to cytokine storms and multiple organ failure."
Dr. Widad Aaji, a consultant in rheumatology and immunology and head of the musculoskeletal diseases department, clarified that during her evaluation, the patient presented with palpable petechiae on the lower extremities and lower abdomen with bleeding and mucosal peeling, along with persistent fever of 39 degrees and mild hepatomegaly, lymphadenopathy, and laboratory findings confirming the diagnosis of macrophage activation syndrome. Improvement in the patient's condition was noted after a single dose of methotrexate, which showcased signs typical of macrophage activation syndrome.
She reported that the patient was placed on a methotrexate regimen of four tablets per week, and the medical team began managing and closely monitoring her condition, starting with aggressive treatment using steroids until signs of improvement appeared, after which immunotherapy was continued, leading to disease remission for over a year.
Dr. Aaji highlighted that macrophage activation syndrome is a serious systemic complication that can occur due to multiple factors, including inflammatory and autoimmune diseases, particularly in patients with Still's disease, marked by uncontrolled macrophage proliferation and activation resulting in cytokine storms and multiple organ failure.
She added that, in this case, based on the importance of continuous education, the role of a single dose of methotrexate as a potential triggering agent for macrophage activation syndrome was discussed, referencing its involvement in three cases documented in medical literature, making this the fourth case globally.
