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Subject: Department of Veterans' Affairs -

N045

Determination

of

Statement of Principles concerning

RELAPSING POLYCHONDRITIS

ICD CODE: 733.99

Veterans' Entitlements Act 1986

1. This Statement of Principles is determined by the Repatriation Medical Authority under subsection 196B(2) of the Veterans' Entitlements Act 1986 (the Act).

Kind of injury, disease or death

2. (a) This Statement of Principles is about relapsing polychondritis and death from relapsing polychondritis.

( For the purposes of this Statement of Principles, "relapsing polychondritis" means an episodic and often progressive multisystem inflammatory disorder affecting predominantly the cartilage of the ears, nose, and tracheobronchial tree, and which may also affect the internal structures of the eyes and ears, the heart, blood vessels, kidneys, skin and joints, attracting ICD code 733.99.

Basis for determining the factors

3. The Repatriation Medical Authority is of the view that there is sound medical-scientific evidence that indicates that relapsing polychondritis and death from relapsing polychondritis can be related to relevant service rendered by veterans, members of Peacekeeping Forces, or members of the Forces.

Factors that must be related to service

4. Subject to clause 6, the factor set out in the paragraph in clause 5 must be related to any relevant service rendered by the person.

Factors

5. The factor that must as a minimum exist before it can be said that a reasonable hypothesis has been raised connecting relapsing polychondritis or death from relapsing polychondritis with the circumstances of a person's relevant service is:

(a) inability to obtain appropriate clinical management for relapsing polychondritis.

Factors that apply only to material contribution or aggravation

6. Paragraph 5(a) applies only to material contribution to, or aggravation of, relapsing polychondritis where the person's relapsing polychondritis was suffered or contracted before or during (but not arising out of) the person's relevant service; paragraph 8(1)(e), 9(1)(e), 70(5)(d) or 70(5A)(d) of the Act refers.

Other definitions

7. For the purposes of this Statement of Principles:

"ICD code" means a number assigned to a particular kind of injury or disease in the Australian Version of The International Classification of Diseases, 9th revision, Clinical Modification (ICD-9-CM), effective date of 1 July 1996, copyrighted by the National Coding Centre, Faculty of Health Sciences, University of Sydney, NSW, and having ISBN 0 642 24447 2;

"relevant service" means:

(a) operational service; or ( peacekeeping service; or © hazardous service.

N045

Determination

of

Statement of Principles concerning

RELAPSING POLYCHONDRITIS

ICD CODE: 733.99

Veterans' Entitlements Act 1986

1. This Statement of Principles is determined by the Repatriation Medical Authority under subsection 196B(3) of the Veterans' Entitlements Act 1986 (the Act).

Kind of injury, disease or death

2. (a) This Statement of Principles is about relapsing polychondritis and death from relapsing polychondritis.

( For the purposes of this Statement of Principles, "relapsing polychondritis" means an episodic and often progressive multisystem inflammatory disorder affecting predominantly the cartilage of the ears, nose, and tracheobronchial tree, and which may also affect the internal structures of the eyes and ears, the heart, blood vessels, kidneys, skin and joints, attracting ICD code 733.99.

Basis for determining the factors

3. On the sound medical-scientific evidence available, the Repatriation Medical Authority is of the view that it is more probable than not that relapsing polychondritis and death from relapsing polychondritis can be related to relevant service rendered by veterans or members of the Forces.

Factors that must be related to service

4. Subject to clause 6, the factor set out in the paragraph in clause 5 must be related to any relevant service rendered by the person.

Factors

5. The factor that must exist before it can be said that, on the balance of probabilities, relapsing polychondritis or death from relapsing polychondritis is connected with the circumstances of a person's relevant service is:

(a) inability to obtain appropriate clinical management for relapsing polychondritis.

Factors that apply only to material contribution or aggravation

6. Paragraph 5(a) applies only to material contribution to, or aggravation of, relapsing polychondritis where the person's relapsing polychondritis was suffered or contracted before or during (but not arising out of) the person's relevant service; paragraph 8(1)(e), 9(1)(e) or 70(5)(d) of the Act refers.

Other definitions

7. For the purposes of this Statement of Principles:

"ICD code" means a number assigned to a particular kind of injury or disease in the Australian Version of The International Classification of Diseases, 9th revision, Clinical Modification (ICD-9-CM), effective date of 1 July 1996, copyrighted by the National Coding Centre, Faculty of Health Sciences, University of Sydney, NSW, and having ISBN 0 642 24447 2;

"relevant service" means:

(a) eligible war service (other than operational service); or ( defence service (other than hazardous service).

RELAPSING POLYCHONDRITIS

ICD CODE 733.99

Definition "Relapsing polychondritis" means an episodic and often progressive multisystem inflammatory disorder affecting predominantly the cartilage of the ears, nose, and tracheobronchial tree, and which may also affect the internal structures of the eyes and ears, the heart, blood vessels, kidneys, skin and joints, attracting ICD code 733.99 Acceptance standard The diagnosis of relapsing polychondritis can be accepted by the Repatriation Commission when it is confirmed by a general medical practitioner or specialist as the final diagnosis or on advice by a Departmental Medical Officer. If a medical report has been received with an unconfirmed or provisional diagnosis:

check for other evidence on file to see if there is supporting evidence which may possibly allow confirmation of diagnosis - information contained within this protocol should be of assistance, and where you are unable to decide whether the diagnosis can be confirmed refer this evidence to a DMO for advice as to whether confirmation of diagnosis is possible at this stage. Otherwise request advice from a DMO upon appropriate action to obtain a confirmed diagnosis - examination, referral, special investigations etc.

Generally speaking, claims received from overseas should be discussed with a DMO to ensure the adequacy of supportive medical evidence. ICD Coding standard Use ICD Code 733.99 which specifically includes the condition of relapsing polychondritis. General information and aetiology Relapsing polychondritis [also known as chronic atrophic polychondritis] is a degeneration and inflammation involving many cartilages of the body, in particular the cartilage of the nose, ears and tracheobronchial tree, as well as internal structures of the eyes and ears. The cartilage softens and collapses, and may result in gross deformity of ears, nose or spine. It is an episodic and often progressive disorder of unknown aetiology which may affect either sex, and is most common between the ages of 40 and 60 years. Polychondritis may also result in polyarthritis, vasculitis, cardiac abnormalities, skin lesions and glomerulonephritis. About 30 percent of patients have another rheumatological disorder, the most frequent being systemic vasculitis, rheumatoid arthritis, systemic lupus erythematosus or Sjogren's syndrome.

Clinical features associated with relapsing polychondritis

The disease is most frequently first noticed in the ears which, eventually, are affected in 90 percent of patients. Usually both ears are involved and initially there is complaint of sudden onset of pain, tenderness, and swelling of the cartilaginous parts of the ear. The overlying skin has a beefy red appearance and repeated or prolonged attacks result in a flabby or droopy ear. Swelling may close off the eustachian tube or the opening of the external ear and impair hearing. Inflammation of the internal auditory artery or its cochlear branch may produce hearing loss, vertigo, loss of muscular coordination, nausea and vomiting. The cartilage of the nose becomes inflamed during the first or subsequent attacks, with approximately 80 percent of patients being eventually affected. The bridge of the nose becomes red swollen and tender. It may collapse with a resultant saddle-shaped deformity. A running nose and/or epistaxis may develop. Many patients may present with arthritis for some months before other features appear but, eventually, about 50 percent will have arthritis. Attacks of arthritis occur episodically, may involve both large and small joints and last for a few days to a few weeks before resolving spontaneously. Eye manifestations affect greater than 50 percent, and include conjunctivitis, episcleritis, iritis and keratitis. Corneal ulceration and perforation resulting in blindness may occur. Cataracts, proptosis, optic neuritis, extraocular muscle palsies, retinal vasculitis and retinal vein occlusion may also be associated. Laryngotracheal symptoms affect about 70 percent, and include hoarseness, a non-productive cough and throat tenderness. Collapse of the cartilage rings of the larynx and trachea may cause serious airway obstruction and, where the bronchial structures are involved, pneumonia or respiratory insufficiency. Cardiac symptoms may commonly include aortic regurgitation from dilatation of the aortic ring or from destruction of the valve cusps, myocarditis, pericarditis and abnormalities of electrical conduction in the heart muscle. Aneurysms, which may rupture, can develop in any part of the aorta. Manifestations of vasculitis may involve large blood vessels, such as giving rise to thrombosis in intracerebral, mesenteric or peripheral arteries. It may also affect the smaller blood vessels and give rise to cutaneous lesions and peripheral or cranial neuropathy. Skin lesions may affect approximately 25 percent of patients and include such conditions as erythema nodosum, erythema multiforme, angioedema/urticaria, livedo reticularis and panniculitis. The course of the disease is very variable , ranging between short acute episodes lasting from a few days to a few weeks before subsiding spontaneously, to cases where the disease assumes a chronic and drawn-out course. Investigations Biopsy of involved cartilage [usually from the nose or ear]. Treatment Prednisone in gradually tapering-off doses although, occasionally, continuing low-dosage therapy is required to suppress the disease process. Appropriate clinical management Biopsy to establish the diagnosis and immediate institution of medication. Reference has been made to material contained within on's Principles of Internal Medicine (13th edition)

Alphabetical listing of Statements of Principles:

A

B

C

D

E

F

G

H

I

J

K

L

M

N

O

P

Q

R

S

T

U

V

W

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