Which diseases are best managed in a multidisciplinary setting? The short answer is: any disease that cannot be optimally treated by one discipline alone. 

The diseases that do not require multidisciplinary involvement are those that are either self-resolving or can be treated using a single therapeutic. Examples such as the common cold and certain bacterial infections come to mind. 

However, a number of diseases cannot be adequately treated by a general practitioner or a consultant specializing in only one field. For example, patients with systemic mastocytosis can experience a number of symptoms affecting various organs, depending on levels of mast cell activation and organ filtration. 

Read more about systemic mastocytosis etiology 

Systemic mastocytosis is a challenging disease to manage from start to finish; it is difficult to diagnose, difficult to treat, and difficult to evaluate as it progresses. 

Diagnostic Challenges

Let’s take a look at the diagnosis of systemic mastocytosis. The difficulty in reaching this diagnosis lies in the vague presentation of the disease. Among its symptoms are vomiting, abdominal pain, malabsorption, skin changes, urticaria, pruritus, and migraine. These symptoms may lead physicians to suspect a disease that originates from the skin or the gastrointestinal system.

However, should systemic mastocytosis be suspected, there are clear criteria for diagnosing the disease, including both major and minor criteria. If these criteria are met, the diagnosis can be made with confidence. The difficulty, of course, is suspecting this diagnosis in the first place. 

In a review published in the British Journal of Haematology, Scherber and Borate wrote, “Careful diagnosis, judicious symptom management and close surveillance of those who may have yet undiagnosed disease is paramount in providing optimal management.” 

Managing Complications 

Let us now review a few complications of systemic mastocytosis and how they are best managed. First, the gastrointestinal effects—such as abdominal pain, diarrhea, and gastritis—can be mild or severe. These symptoms can also be brought about by various types of stimuli. 

The list of differential diagnoses for this group of symptoms is extensive; to make matters even more difficult, the endoscopic findings tend to be nonspecific. Hence, a good medical history is essential in order to identify potential triggers for gastrointestinal symptoms. As for treatment options, proton pump inhibitors and H2 blockers are usually used.

Another common manifestation of systemic mastocytosis is bone disease, particularly osteoporosis. Zanotti and colleagues wrote in the Mediterranean Journal of Hematology and Infectious Diseases, “[Systemic mastocytosis (SM)]-related fragility involves most prominently the spine, provoking frequently vertebral fragility fractures. Given this, SM diagnosis should be considered when approaching a patient with unexplained fragility fractures, osteoporosis, or inappropriate low bone mineral density (BMD), as a possible underlying etiology.” 

Once again, these symptoms are often treated as standalone features, especially in elderly patients. “Osteoporosis” is often regarded as a self-sufficient diagnosis, with certain criteria that need to be met. Hence, it is entirely possible for a patient to see a doctor for bone fragility and be treated for that issue alone. 

Bone disease associated with systemic mastocytosis is often treated with calcium and Vitamin D. Severe cases of osteoporosis are sometimes treated with bisphosphonates and interferon. 

Read more about systemic mastocytosis therapies

Another aspect of systemic mastocytosis that highlights the need for a multidisciplinary approach is pregnancy in patients. Existing studies do not seem to suggest this disease affects fertility. The challenge is to ensure no drugs prescribed are harmful to the fetus.

“The management of symptoms of women with SM during pregnancy and early postpartum should combine drugs with no or very limited teratogenic potential to relieve symptoms of MC activation, avoid known triggers, and eventually use prophylactic anti-mediator drugs (steroids, antihistamines), as needed,” Zanotti et al wrote. 

A Complex Disease

In general, patients with systemic mastocytosis should always be mindful of avoiding potential triggers. Reported triggers include sudden changes in temperature, vigorous skin rubbing, and extensive tissue trauma. Because these triggers can cause the acute worsening of a patient’s condition, it is important for physicians to be mindful of them to avoid unnecessary clinical deterioration. 

This is why the prescription of anesthetic drugs remains controversial. There is still no consensus in the medical literature regarding the safety of anesthetic drugs in patients with systemic mastocytosis. Hence, “it is impossible to provide general recommendations on the safety of drugs, or drug families, used in the perioperative period,” Zanotti and colleagues wrote. 

This is a problem, of course, in patients who need anesthesia for surgery. This is where the expertise of an anesthesiologist should come in. Generally, if the patient has had previous surgery with anesthesia, the patient’s previous perioperative notes should be consulted. Another approach is to use drugs that are largely considered to be safe for general anesthesia, such as propofol, fentanyl, and sevoflurane. 

The main reason for the need for a multidisciplinary team to manage systemic mastocytosis is that various systems are typically involved in this disease. Ideally, a patient with systemic mastocytosis should be seen by various specialties for pathology that involves the specific specialty. Compounding the problem is the fact that many patients with systemic mastocytosis also struggle with mental health issues, such as depression and anxiety, which require treatment by a qualified psychiatrist. 

Zanotti et al concluded, “Given the complexity of this rare and often misdiagnosed disease, it is of great importance to address the patient to well experienced and specialized center, possibly performing a multidisciplinary diagnostic evaluation.” 

References

Scherber RM, Borate U. How we diagnose and treat systemic mastocytosis in adultsBr J Haematol. 2018;180(1):11-23. doi:10.1111/bjh.14967

Zanotti R, Tanasi I, Crosera L, et al. Systemic mastocytosis: multidisciplinary approachMediterr J Hematol Infect Dis. 2021;13(1):e2021068. doi:10.4084/MJHID.2021.068