ALTERNATE NAMES

Diffuse Alveolar Septal Amyloidosis; Pulmonary Primary Amyloidosis; Systemic AL Amyloidosis with Pulmonary Involvement

DESCRIPTION

Amyloidosis is a group of disorders in which an abnormal protein called amyloid builds up in tissues and organs causing damage to organs and tissues. AL amyloidosis is the most common form. Pulmonary amyloidosis - AL type occurs when plasma cells in the bone marrow produce abnormal immunoglobulin light chains that form these amyloid deposits accumulate in the lungs. When amyloid deposits accumulate in the lungs, this can lead to lung dysfunction and various respiratory symptoms depending on the severity and location of the deposits.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: The diagnosis of pulmonary amyloidosis - AL type is made by:

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  Computed tomography (CT) scan of the chest;

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  Radiological findings;

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  Lung biopsy (confirms the diagnosis);

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  Immunohistochemical staining; and

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  Pulmonary function tests.

Physical findings: The signs and symptoms of pulmonary amyloidosis – AL type include:

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  Hoarseness;

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  Stridor (abnormal, high pitched breathing sound);

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  Exertional dyspnea (shortness of breath or difficulty breathing during exercise or physical exertion);

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  Hemoptysis (blood or bloody mucus from lungs and throat);

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  Numbness, tingling, or pain in the hands or feet;

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  Swelling of the ankles and legs;

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  Diarrhea;

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  Constipation;

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  An enlarged tongue, which sometimes looks rippled around its edge; and

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  Skin changes, such as thickening or easy bruising, and purplish patches around the eyes.

ICD-9: 277.39

ICD-10: E85.81

PROGRESSION

Symptoms of this disease may start to appear as an adult. The presence of AL amyloid deposits in the lungs often indicates a wider systemic involvement with a poor overall outlook. Early diagnosis and treatment are crucial for better management and potential survival rates.

TREATMENT

Pulmonary amyloidosis – AL type is difficult to treat, and treatment is generally only marginally effective. Treatment may consist of chemotherapy and/or bone marrow or stem cell transplantation. Treatment generally focuses on the management of the underlying systemic amyloidosis.

Suggested MER for Evaluation:

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  Clinical examination that describes the diagnostic features of lung involvement;

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  CT scan of the chest; and

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  Lung tissue biopsy.

Suggested Listings for Evaluation:

Meets

3.02

3.14

Equals