M34.9 - Systemic sclerosis, unspecified

Approximate Synonyms

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

• Autoimmune cholangitis
• Autoimmune hepatitis
• Biliary cirrhosis
• Cardiomyopathy due to connective tissue disease
• Cutaneous complication of systemic sclerosis
• Cutaneous complication of systemic sclerosis
• Cutaneous complication of systemic sclerosis
• Dilated cardiomyopathy due to systemic sclerosis
• Disorder of connective tissue co-occurrent and due to systemic disease
• Fibrosis of bile duct
• Glomerulonephritis co-occurrent and due to scleroderma
• Hypermelanosis due to connective tissue disorder
• Hypothyroidism due to systemic sclerosis
• Limited systemic sclerosis
• Limited systemic sclerosis
• Necrotizing vasculitis secondary to connective tissue disease
• Occupational scleroderma
• Pediatric onset systemic sclerosis
• Pericarditis secondary to scleroderma
• Primary biliary cholangitis
• Progressive systemic sclerosis
• Renal involvement in scleroderma
• Reynolds syndrome
• Scleredema
• Sclerema
• Scleroderma-associated calcinosis
• Scleroderma-associated hypermelanosis
• Scleroderma-associated nail dystrophy
• Scleroderma-associated nailfold telangiectasia
• Scleroderma-associated necrotizing vasculitis
• Scleroderma-associated poikiloderma
• Scleroderma-associated telangiectasia
• Systemic sclerosis
• Systemic sclerosis with limited cutaneous involvement
• Systemic sclerosis, diffuse
• Telangiectasia of nailfolds
• Thickening of skin

Clinical Information

• Autoimmune Hepatitis-. hepatitis caused by autoantibodies. drugs, infections, and toxins may trigger the production of the autoantibodies against the liver parenchyma.
• Type 1 Autoimmune Hepatitis|Autoimmune Hepatitis, Type 1|Type 1 AIH-. the most common subtype of autoimmune hepatitis (aih). it is characterized by the presence of antinuclear antibody (ana) and anti-smooth-muscle antibody (asma), a prevalence of female patients, and is highly corticosteroid-responsive. it may be associated with other concomitant autoimmune disorders including autoimmune thyroiditis, graves disease, and ulcerative colitis.
• Type 2 Autoimmune Hepatitis|Autoimmune Hepatitis, Type 2|Type 2 AIH-. a subtype of autoimmune hepatitis (aih). in contrast to type 1 aih, patients tend to be younger, have more severe disease, a poorer response to corticosteroid therapy, and more frequent relapses. it is characterized by the presence of anti-liver kidney microsomal antibody type 1 (anti-lkm1) and/or anti-liver cytosol type 1 (anti-lc1) autoantibodies. it may be associated with other concomitant autoimmune disorders including diabetes mellitus type 1, vitiligo, and autoimmune thyroiditis.
• Variant Autoimmune Hepatitis|Autoimmune Hepatitis, Variant|Variant AIH-. a form of autoimmune hepatitis (aih) that has features of overlapping cholestatic autoimmune liver diseases including primary biliary cholangitis (pbc), primary sclerosing cholangitis (psc), or autoimmune cholangitis (aic).

Diagnostic Related Groups - MS-DRG Mapping

The is diagnosis code is grouped in the following groups for version MS-DRG V40.0 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 10/01/2022 through 09/30/2023.

Convert to ICD-9 Code

Patient Education

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

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.

Scleroderma

Code History

• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)