April 23, 2024

Pharming reveals ICD-10-CM code for APDS

Anurag Relan, Chief Medical Officer of Pharming

Pharming Group N.V. (“Pharming” or “the Company”) announces that a new diagnosis code for reporting cases of activated phosphoinositide 3-kinase delta syndrome (APDS), a rare primary immunodeficiency, will be added to the International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) by the US Centers for Disease Control and Prevention (CDC). The diagnosis code, D81.82 ‒ Activated Phosphoinositide 3-kinase Delta Syndrome (APDS), will be effective starting October , 2022.

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Anurag Relan, Chief Medical Officer of Pharming, commented “By assigning this ICD-10-CM code, the CDC is formally recognizing APDS as a discrete immunological disease, and that will make a life-altering difference for people affected by the condition. By using the unique diagnostic code to identify both established and new patients with APDS, physicians will increase care options for affected individuals while helping to boost the world’s understanding of the prevalence, mechanisms, and outcomes of this progressively debilitating disease. For healthcare practitioners, this milestone marks an opportunity to make a big difference by taking a simple action.”

The assignment of the ICD-10-CM code will, for the first time, enable physicians and payors in the US to add a diagnosis of APDS to patients’ health records, which will help connect these individuals with researchers studying the prevalence and course of the disease. In addition, by allocating a specific diagnosis, the new ICD-10-CM code may help confirm medical necessity in individual patients, thus improving their access to relevant care options through US health insurance plans.

Pharming Group Lab Image:source

Caused by genetic variants affecting approximately one to two people per million, APDS causes significant lymphoproliferation and immune dysfunction, as well as an increased risk of lymphoma. There is no approved therapy for the disease and treatment is generally limited to supportive care, such as antibiotics and immunoglobulin replacement therapy. Physician and patient advocacy groups specializing in immunodeficiency disorders, along with Pharming, expect the decision to raise awareness about this rare disease.

Vicki and Fred Modell, co-founders of the Jeffrey Modell Foundation, commented “We are excited that US regulatory authorities have assigned APDS an ICD-10-CM code. As a foundation dedicated to early diagnosis, meaningful treatments, and cures for primary immunodeficiency, we are aware of the physical and emotional challenges people with APDS face due to misdiagnosis of their disease. By increasing recognition of the condition, we expect the new diagnostic code to help ensure that every patient is included when it comes to the delivery of appropriate and meaningful treatments for APDS.”


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