Histiocytosis X Medical Assignment Help

CLINICAL FEATURES

There are three variants of this disease, all characterized by the presence of granulomas consisting predominantly of characteristic histiocytes intermingled with eosinophilic and neutrophilic granulocytes, giant cells and lymphocytes. Electron microscopic studies have shown that the histiocytes contain granules characteristic of Langerhan cells. Fibrosis may occur early, with the development of multiple small cysts producing a honeycomb appearance.

Eosinophilic granuloma

This is the commonest variant in adults. It presents with increasing effort dyspnoea and cough. The chest X-ray shows diffuse bilateral mottling with translucencies with thick walls, and gas transfer is decreased. Recurrent pneumothorax occurs. The granulomas can also be found in bones.

Letterer-Siwe disease

This is usually a fatal disease of infancy that occurs under the age of 3 years. It is a widespread disease with skin lesions, lymphadenopathy, hepatosplenomegaly and bone lesions.

Hand-Schiiller-Christian disease

This usually begins under the age of 5 years. It is characterized by defects in bone, exophthalmos and diabetes insipidus. Pulmonary lesions show diffuse nodular shadows with hilar lymphadenopathy simulating sarcoidosis.

PROGNOSIS

Both Hand-Schuller-Christian disease and eosinophilic granuloma may recover spontaneously; survival for 20 years has been reported for both conditions.

TREATMENT

Treatment is with corticosteroids and cyclophosphamide, though there is no evidence as yet that this treatment substantially alters the outcome.

Wegener’s granulomatosis

This disease. of unknown aetiology is characterized by lesions involving the upper respiratory tract, the lungs and the kidneys. Often the disease starts with severe rhinorrhoea with subsequent nasal mucosal ulceration followed by cough, haemoptysis and pleuritic pain. Occasionally there may be involvement of the skin and nervous system. A chest X-ray usually shows single or multiple nodular masses or pneumonic infiltrates with cavitation. The most remarkable radiographic feature is the migratory pattern, with large lesions clearing in one area and new lesions appearing in another.
The typical histological changes are usually best seen in the kidneys, where there is a necrotizing glomerulonephritis. This disease responds well to treatment with cyclophosphamide 150-200 mg daily. A variant of Wegener’s granulomatosis called ‘mid-line granuloma’ particularly affects the nose and paranasal sinuses and is particularly mutilating; it has a poor prognosis. Anti-neutrophil cytoplasmic antibodies (ANCA) ANCA is found in the acute phase of vasculitides associated with neutrophil infiltration of the vessel wall and may be found in a wide range of diseases. cANCA is more specific for Wegener’s granulomatosis (greater than 90%) but only in the active stage. cANCA positivity is found in only one-third of inactive or treated cases. pANCA is closely associated with a number of vasculitic syndromes, Churg-Strauss syndrome and microscopic polyarteritis; 10-15% of cases of progressive glomerulonephritis with anti-glomerular basement membrane (GBM) antibodies are pANCA positive and these are the most likely to suffer pulmonary haemorrhage.

RESPIRATORY MANIFESTATIONS OF SYSTEMIC CONNECTIVE TISSUE DISEASE

Rheumatoid disease

The features of respiratory involvement in rheumatoid disease are illustrated.

Pleural adhesions, thickening and effusion are the commonest lesions. The effusion is often unilateral and tends to be chronic. It has a low glucose content but this can occur in any chronic pleural effusion. Rheumatoid diffuse fibrosing alveolitis can be considered as a variant of the cryptogenic form of the disease (see p. 694). The clinical features and gross appearance are the same but the disease is often more chronic. Rheumatoid nodules showing the typical histological appearances are rare in the lung. On the chest X-ray these appear as single or multiple nodules ranging in size from a few millimetres to a few centimetres. The nodules frequently cavitate. They usually produce no symptoms but can give rise to a pneumothorax or pleural effusion. Obliterative disease of the small bronchioles is rare. It is characterized by progressive breathlessness and irreversible airflow limitation. Corticosteroids may prevent progression.
Involvement of the cricoarytenoid joints gives rise to dyspnoea, stridor, hoarseness and occasionally severe obstruction necessitating tracheostomy. Caplan’s syndrome is due to a combination of dust inhalation and the disturbed immunity of rheumatoid arthritis. It occurs particularly in coal-worker’s pneumoconiosis but it can occur in individuals exposed to other dusts, such as silica and asbestos. Typically the lesions appear as rounded nodules 0.5-5 cm in diameter, though sometimes they become incorporated into large areas of fibrosis that are indistinguishable radiologically from progressive massive fibrsis. There may be little evidence of simple pneumoconiosis prior to the development of the nodule.
These lesions may precede the development of the arthritis. Rheumatoid factor is always present in the serum.

The respiratory manifestations of rheumatoid disease

The respiratory manifestations of rheumatoid
disease

Systemic lupus erythematosus

The commonest respiratory manifestation of this disease, occurring in up to two-thirds of cases, is the development of pleurisy, with or without an effusion. Effusions are usually small and bilateral. Basal pneumonitis is often present, perhaps as a result of poor movement of the diaphragm, or restriction of chest movements due to pleural pain. Pneumonia also occurs, either due to infection or to the disease process itself. Unlike rheumatoid arthritis, diffuse pulmonary fibrosis is rare.

Systemic sclerosis

Autopsy studies have indicated that there is almost always some evidence of diffuse fibrosis of alveolar walls and obliteration of capillaries and the alveolar space. Severe changes result in nodular then streaky shadowing on the chest X-ray, followed by cystic changes, ending up with a honeycomb lung. Function tests indicate a restrictive lesion and poor gas transfer.
Pneumonia may occur due to inhalation from the dilated oesophagus. Breathlessness may be worsened by restriction of chest wall movement due to thickening and contraction of the skin and trunk.

PULMONARY INFILTRATION WITH EOSINOPHILIA {PIE}

The common types and characteristics of these diseases are shown in Table 12.9. They range from very mild, simple, pulmonary eosinophilias to the often fatal polyarteritis nodosa.

Common types and characteristics

Common types and characteristics

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