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D G James

Publications and source records attributed to D G James.

At least 37 records · Page 2Linked to original sources

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Granulomatous Disease, Chronic↗

The sarcoidosis movement and its personalities.

Sarcoidosis has been recognized for 125 years, but in every generation it makes a chameleon-like change, so updating is needed every so often. In Jonathan Hutchinson's day it was a dermatological curiosity, which gradually evolved into a multisystem disorder associated with bone cysts, and, with the introduction of X-rays, respiratory abnormalities. During the 1960s, epidemiology showed that this disorder, involving all systems of the body, was widely disseminated throughout the world. Each fresh approach, by investigators in different disciplines, has provided a new dimension, particularly in biochemistry and immunology. Each advance in knowledge brings us nearer the enigma which continues to elude us, namely the aetiology of sarcoidosis (Tables 1 and 2). This paper draws attention to the pioneers and personalities of the movement, and records the succession of congresses which have helped to define it worldwide.

History, 19th Century↗

A comparison of sarcoidosis and Behçet's disease.

There are several diseases which present with both ocular and thoracic disease (Table I). Behçet's disease (BD) may be confused with sarcoidosis because they are both multisystem inflammatory disorders presenting with uveitis, polyarthritis, meningitis, cardiovascular disease, erythema nodosum and other cutaneous lesions, and abnormal chest radiographs. The aetiology of both disorders continues to elude us. Hopefully, a careful study of the one may shed light and knowledge on the other.

Adult↗

A comparison of Blau's syndrome and sarcoidosis.

Blau's syndrome is a familial multisystem granulomatous inflammation which may be confused with childhood sarcoidosis because it presents with iridocyclitis, posterior uveitis, granulomatous skin disease, arthritis and elevated serum angiotensin-converting enzyme. They are distinguished by the absence of pulmonary involvement and a negative Kveim-Siltzbach skin test.

Adult↗

Sarcoidosis of the liver.

There are many causes of granulomas of the liver including infections, immunological aberrations, enzyme defects, drugs and other chemicals, and neoplasia (Table I). The commonest causes are tuberculosis, sarcoidosis, schistosomiasis and intrinsic liver disease. About 10 per cent of granulomas remain unidentified. In sarcoidosis, liver granulomas are usually inconspicuous and featureless. They are not associated with symptoms or abnormal physical signs. Sarcoidosis of the liver has three well-recognised clinical syndromes-chronic intrahepatic cholestasis, portal hypertension and the Budd-Chiari Syndrome.

Adult↗

Complications of sarcoidosis. Chronic fatigue syndrome.

Well-recognised complications are pulmonary fibrosis, cor pulmonale, glaucoma, cataract and nephrocalcinosis causing failure of lungs, heart, vision and kidneys. Less well-recognised is the post-sarcoidosis chronic fatigue syndrome. The afflicted join sarcoidosis patients' associations because of their profound symptoms of myalgia, fatigue, sleep reversal and low-spiritedness. The symptoms are out of proportion to the lack of physical signs and the absence of objective evidence of sarcoidosis. Management includes unremitting sympathy and replenishment of essential neurochemicals.

Fatigue Syndrome, Chronic↗

Pulmonary Langerhans' cell granulomatosis (LCG).

Pulmonary Langerhans' granulomatosis (LCG) is characterised by infiltration of peripheral airways by dendritic cell precursors and Langerhans' cells (LC) together with eosinophils and mixed chronic inflammatory cells. Consequent airways destruction commonly leads to cystic changes and frequent pneumothorax. LC's are best identified by the electron microscope finding of Birbeck granules. Immunocytochemical markers are also of value. They are frequent in the early stages though absent in end stage disease. LCG is distinguished from sarcoidosis by the absence of epithelioid cell granulomas, association with heavy cigarette smoking in young males and frequency of pneumothorax. Both diseases show lymphocytosis in bronchoalveolar lavage but differ in that LCG shows a predominance of T-suppressor cells and sarcoidosis an excess of T-helper cells.

Diagnosis, Differential↗

Lupus pernio.

Thirty-five patients with lupus pernio have been observed in a series of 818 patients with clinical and histological evidence of sarcoidosis. This analysis provides the natural history of lupus pernio and its associated clinical and radiological features. It predominates in women, and particularly in West Indians. The chronic persistent violaceous skin lesions have a predilection for the nose, cheeks, lips, eyelids, ears and fingers, ranging from a few nodules under the tip of the nose to exuberant plaques spreading across the nose and both cheeks. There was intrathoracic involvement in 74% of patients, upper respiratory tract disease in 54%, reticulo-endothelial involvement in 54%, bone cysts in 43% and ocular lesions in 37%. It is distinguished from lupus vulgaris and lupus erythematosus by clinical features, histology and radiology.

Adult↗

Bronchopulmonary lavage (BAL). A window of the lungs.

Bronchopulmonary lavage (BAL) has provided a fresh dimension for the investigation of pulmonary and multisystem disorders. BAL fluid may be analysed for cells and chemical mediators in the diagnosis and also serially for the management of several granulomatous disorders including sarcoidosis, extrinsic allergic alveolitis, chronic beryllium disease, talc granulomatosis, tuberculosis, Langerhans' histiocytosis-x and Crohn's disease. It may also provide information in fibrosing alveolitis, collagen vascular disease, occupational and drug-induced lung disease, acquired immune deficiency syndrome, bronchial asthma, neoplasia, transplantation, pulmonary alveolar proteinosis and eosinophilic lung disease. This survey analyses the value of BAL and how it has provided a new window for the chest physician.

Bronchoalveolar Lavage Fluid↗