ICD-10-CM Code M34.1

CR(E)ST syndrome

Version 2020 Billable Code

Valid for Submission

M34.1 is a billable code used to specify a medical diagnosis of cr(e)st syndrome. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code M34.1 might also be used to specify conditions or terms like crest syndrome, crst syndrome, limited systemic sclerosis, limited systemic sclerosis, raynaud's phenomenon, raynaud's phenomenon, etc

ICD-10:M34.1
Short Description:CR(E)ST syndrome
Long Description:CR(E)ST syndrome

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code M34.1:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Combination of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code M34.1 are found in the index:


Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • CREST syndrome
  • CRST syndrome
  • Limited systemic sclerosis
  • Limited systemic sclerosis
  • Raynaud's phenomenon
  • Raynaud's phenomenon
  • Secondary Raynaud's phenomenon
  • Secondary Raynaud's phenomenon
  • Systemic sclerosis with limited cutaneous involvement
  • Systemic sclerosis with limited cutaneous involvement

Clinical Information

  • CREST SYNDROME-. a mild form of limited scleroderma a multi system disorder. its features include symptoms of calcinosis; raynaud disease; esophageal motility disorders; sclerodactyly and telangiectasis. when the defect in esophageal function is not prominent it is known as crst syndrome.

Diagnostic Related Groups

The ICD-10 code M34.1 is grouped in the following groups for version MS-DRG V37.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2019 through 09/30/2020.

  • 545 - CONNECTIVE TISSUE DISORDERS WITH MCC
  • 546 - CONNECTIVE TISSUE DISORDERS WITH CC
  • 547 - CONNECTIVE TISSUE DISORDERS WITHOUT CC/MCC

Convert M34.1 to ICD-9

  • 710.1 - Systemic sclerosis (Approximate Flag)

Code Classification

  • Diseases of the musculoskeletal system and connective tissue (M00–M99)
    • Systemic connective tissue disorders (M30-M36)
      • Systemic sclerosis [scleroderma] (M34)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients


Scleroderma

Scleroderma means hard skin. It is a group of diseases that cause abnormal growth of connective tissue. Connective tissue is the material inside your body that gives your tissues their shape and helps keep them strong. In scleroderma, the tissue gets hard or thick. It can cause swelling or pain in your muscles and joints.

Symptoms of scleroderma include

  • Calcium deposits in connective tissues
  • Raynaud's phenomenon, a narrowing of blood vessels in the hands or feet
  • Swelling of the esophagus, the tube between your throat and stomach
  • Thick, tight skin on your fingers
  • Red spots on your hands and face

No one knows what causes scleroderma. It is more common in women. It can be mild or severe. Doctors diagnose scleroderma using your medical history, a physical exam, lab tests, and a skin biopsy. There is no cure, but various treatments can control symptoms and complications.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases


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Systemic scleroderma Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. The word "scleroderma" means hard skin in Greek, and the condition is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The condition is also called systemic sclerosis because the fibrosis can affect organs other than the skin. Fibrosis is due to the excess production of a tough protein called collagen, which normally strengthens and supports connective tissues throughout the body.The signs and symptoms of systemic scleroderma usually begin with episodes of Raynaud phenomenon, which can occur weeks to years before fibrosis. In Raynaud phenomenon, the fingers and toes of affected individuals turn white or blue in response to cold temperature or other stresses. This effect occurs because of problems with the small vessels that carry blood to the extremities. Another early sign of systemic scleroderma is puffy or swollen hands before thickening and hardening of the skin due to fibrosis. Skin thickening usually occurs first in the fingers (called sclerodactyly) and may also involve the hands and face. In addition, people with systemic scleroderma often have open sores (ulcers) on their fingers, painful bumps under the skin (calcinosis), or small clusters of enlarged blood vessels just under the skin (telangiectasia).Fibrosis can also affect internal organs and can lead to impairment or failure of the affected organs. The most commonly affected organs are the esophagus, heart, lungs, and kidneys. Internal organ involvement may be signaled by heartburn, difficulty swallowing (dysphagia), high blood pressure (hypertension), kidney problems, shortness of breath, diarrhea, or impairment of the muscle contractions that move food through the digestive tract (intestinal pseudo-obstruction).There are three types of systemic scleroderma, defined by the tissues affected in the disorder. In one type of systemic scleroderma, known as limited cutaneous systemic scleroderma, fibrosis usually affects only the hands, arms, and face. Limited cutaneous systemic scleroderma used to be known as CREST syndrome, which is named for the common features of the condition: calcinosis, Raynaud phenomenon, esophageal motility dysfunction, sclerodactyly, and telangiectasia. In another type of systemic scleroderma, known as diffuse cutaneous systemic scleroderma, the fibrosis affects large areas of skin, including the torso and the upper arms and legs, and often involves internal organs. In diffuse cutaneous systemic scleroderma, the condition worsens quickly and organ damage occurs earlier than in other types of the condition. In the third type of systemic scleroderma, called systemic sclerosis sine scleroderma ("sine" means without in Latin), fibrosis affects one or more internal organs but not the skin.Approximately 15 percent to 25 percent of people with features of systemic scleroderma also have signs and symptoms of another condition that affects connective tissue, such as polymyositis, dermatomyositis, rheumatoid arthritis, Sjögren syndrome, or systemic lupus erythematosus. The combination of systemic scleroderma with other connective tissue abnormalities is known as scleroderma overlap syndrome.
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