Diagnosing LGMD

ICD-10 codes for LGMD

New ICD-10 codes for limb-girdle muscular dystrophy (LGMD) became available for use in October 2022.

The ability to accurately record a patient’s LGMD subtype through specific ICD-10 codes, when possible, is an important advancement for several reasons. Adoption of the new ICD-10 codes will support clinical and research communities in ongoing efforts to:   

  • Understand LGMD epidemiology
  • Assess natural history / disease progression of the condition
  • Understand economic burden of LGMD   
  • Help manage care of patients   
  • Facilitate reimbursement and patient access when targeted therapies become available in the future


What are ICD-10 codes?    

ICD-10 refers to the 10th edition of the International Statistical Classification of Diseases and Related Health Problems (ICD), a medical classification list endorsed by the World Health Organization (WHO). The ICD provides a standardized method for classifying and coding health conditions and diseases, with codes representing diagnoses, symptoms, and procedures.

ICD-10 codes are used:  

  • By hospitals to bill insurance companies for healthcare provided to patients  
  • To gather data on illnesses and track health statistics  
  • To inform research, such as understanding the epidemiology of a disease


What can healthcare professionals do?    

Up until now, LGMD patients were likely coded under G71.0 “Muscular Dystrophy”:  

  • G71.00 – “Muscular dystrophy, unspecified”  
  • G71.09 – “Other specified muscular dystrophies”  

If you have LGMD patients captured under G71.00 or G71.09, please refer to the updated ICD-10 codes to accurately capture their diagnosis at their next appointment.


What can individuals living with LGMD do?    

At your next visit, speak with your physician about LGMD subtype-specific ICD-10 codes to help ensure that your diagnosis is coded properly.


ICD-10 Code Table

Diagnosis code Description Subtype, if applicable
G71.031 Autosomal dominant LGMD LGMD1/D
G71.032 Autosomal recessive LGMD due to calpain-3 dysfunction (calpainopathy) LGMD2A/R1
G71.033 LGMD due to dysferlin dysfunction (dysferlinopathy) LGMD2B/R2
G71.0340 LGMD due to sarcoglycan dysfunction, unspecified (sarcoglycanopathy)  
G71.0341 LGMD due to alpha sarcoglycan dysfunction (alpha-sarcoglycanopathy) LGMD2D/R3
G71.0342 LGMD due to beta sarcoglycan dysfunction (beta-sarcoglycanopathy) LGMD2E/R4
G71.0349 LGMD due to other sarcoglycan dysfunction LGMD2C/R5*  
G71.035 LGMD due to anoctamin-5 dysfunction (anoctaminopathy) LGMD2L/R12
G71.038 Other LGMD** Other autosomal recessive forms of LGMD, such as LGMD2I/R9 (caused by mutations in fukutin-related protein gene, FKRP)
G71.039 LGMD, unspecified**  

*LGMD2C/R5 is caused by mutations in the SGCG gene, which encodes gamma-sarcoglycan. LGMD2F/R6 is caused by mutations in the SGCD gene, which encodes delta-sarcoglycan. 
**G71.038 is intended for all other forms of autosomal recessive LGMD. G71.039 is intended for patients that do not have a genetically confirmed LGMD diagnosis. If a patient has not yet received genetic testing to confirm their LGMD diagnosis, please visit limbgirdle.com/genetic-testing for more information. 



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What Is LGMD?

A group of genetic diseases that cause progressive muscle weakness…

Disease Management

Current approaches focus on symptom management for individual patient needs…