Lymphangioleiomyomatosis
ICD-10 J84.81 is a billable code used to indicate a diagnosis of lymphangioleiomyomatosis.
Lymphangioleiomyomatosis (LAM) is a rare lung disease characterized by the abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs, leading to the obstruction of lymphatic vessels and the formation of cystic lung lesions. Clinically, patients often present with progressive dyspnea, cough, and recurrent pneumothorax. The disease primarily affects women of childbearing age, and its etiology is linked to mutations in the TSC2 gene, which is associated with tuberous sclerosis complex. As LAM progresses, it can lead to significant respiratory impairment and reduced quality of life. Diagnostic considerations include high-resolution computed tomography (HRCT) scans, which reveal characteristic cystic changes in the lungs, and lung function tests to assess the degree of airflow obstruction. A multidisciplinary approach is often required for management, including pulmonology, genetics, and sometimes oncology, due to the potential for associated renal tumors. Early diagnosis and intervention are crucial to managing symptoms and improving patient outcomes.
Standard ICD-10-CM documentation requirements apply
Various clinical presentations within this specialty area
Follow specialty-specific billing guidelines
Standard ICD-10-CM documentation requirements apply
Various clinical presentations within this specialty area
Follow specialty-specific billing guidelines
J84.81 specifically covers lymphangioleiomyomatosis, a rare lung disease characterized by cystic lung lesions and lymphatic obstruction due to the proliferation of LAM cells. It is primarily associated with women and can be linked to tuberous sclerosis.
J84.81 should be used when a patient is diagnosed with lymphangioleiomyomatosis, particularly when imaging studies show characteristic cystic changes and when other lung diseases have been ruled out.
Documentation for J84.81 should include clinical notes detailing symptoms, imaging results showing cystic lung changes, and any genetic testing results confirming TSC2 mutations.
