CLASSIFICATION OF PULMONARY SARCOIDOSIS

According to the accepted classification, five stages are distinguished:

Stage 0 (the mildest stage): no pathological changes are visible on the chest image.

Stage 1: the patient’s lymph nodes are enlarged, but the lung tissue is healthy.

Stage 2: additional lymph nodes are inflamed and granulomas are present in the lung tissue.

Stage 3: pathological changes are present in the tissues without further enlargement of the lymph nodes.

Stage 4: pulmonary fibrosis with irreversible impairment of respiratory function.

Treatment

No specific treatment has been shown to be effective. Supportive treatment consists of oxygen for hypoxaemia and antibiotics for pneumonia.

In end-stage disease, lung transplantation should be considered in selected cases. Corticosteroids and cytotoxic medicines (cyclophosphamide and azathioprine) are given empirically to patients with IPF, with the aim of stopping progression of inflammation. However, little evidence confirms their effectiveness. Prednisone is usually tried first (0.5 to 1.0 mg/kg orally once daily for 3 months, followed by a gradual reduction to 0.25 mg/kg once daily for the next 3 to 6 months) in combination with cyclophosphamide or azathioprine (1 to 2 mg/kg/day orally).

For one year, a clinical examination and chest X-ray should be performed every three months, together with an assessment of the physiological response to treatment. Medicine doses can be increased or reduced according to the findings. Treatment should be stopped if there is no objective response.

Pirfenidone, an antifibrotic medicine, may stabilise lung function and reduce the number of exacerbations. Antifibrotics that inhibit collagen synthesis (relaxin), profibrotic growth factors (suramin) and endothelin-1 (an angiotensin-receptor blocker) have shown effectiveness only in vitro.