Systemic Sclerosis N064

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-9-CM Codes: 710.1
• ICD-10-AM Codes: M34

Brief description

Systemic sclerosis (AKA scleroderma) is a condition involving thickening and hardening of body tissues.  This autoimmune condition can affect every organ in the body, with a widespread obliterative vasculopathy of small arteries and arterioles leading to fibrosis.  Involvement may be limited to the skin and the disease is then called localised sclerosis.  Localised sclerosis is covered by a separate SOP. 

Confirming the diagnosis

The diagnosis can be made on clinical grounds.  Serological markers such as anti-topoisomerase-I (anti-Scll-70) antibodies and anti-centromere antibodies are useful for diagnosis.  Histopathological confirmation is sometimes required.

The relevant medical specialist is a dermatologist or physician.

Additional diagnoses covered by SOP

• CREST syndrome  -  This is an acronym for Calcinosis, Raynaud’s phenomenon, Oesophageal dysfunction, Sclerodactyly and Telangiectasia
• Diffuse cutaneous systemic sclerosis
• Limited cutaneous systemic sclerosis
• Progressive systemic sclerosis
• Systemic sclerosis sine scleroderma

Related conditions that may be covered by SOP (further information required)

• Scleroderma – It depends on whether this is systemic scleroderma or localised scleroderma.

Conditions excluded from SOP

• Circumscribed scleroderma* - localised sclerosis SOP
• eosinophilic fasciitis^#
• Linear scleroderma* - localised sclerosis SOP
• Localised scleroderma* - localised sclerosis SOP
• Localised sclerosis*
• Mixed connective tissue disorder^#
• Morphea^#
• Multifocal fibrosclerosis^#
• Multiple sclerosis*
• Scleromyxoedema^#

* another SOP applies

^# non-SOP condition

Comments

There may be circumstances where the veteran was correctly diagnosed with localised sclerosis and then, with the efflux of time and the development of further systemic manifestation, also correctly diagnosed as systemic sclerosis.  In this situation, the original diagnosis is no longer valid, having been replaced by the systemic sclerosis diagnosis.  As such the new condition will need to be re-determined using the SOP for systemic sclerosis.

Clinical onset

The condition generally presents with skin changes on the hands and face, which can include itchiness, swelling and pigment changes along with thickening and hardening.  Generalised symptoms can include fatigue, joint stiffness, loss of strength and joint and muscle pain.

Clinical worsening

Clinical worsening may be in the form of scleroderma renal crisis, for which there is a specific SOP factor.