About WASOG

The WASOG (World Association for Sarcoidosis and Other Granulomatous Disorders), was founded in 1987 in Milan, Italy, during the World Congress for Sarcoidosis. The topics WASOG is focussing on are interstitial lung diseases (ild). Many ild (e.g. sarcoidosis) are part of a larger systemic problem, where the lung may be a minor target; others (e.g. idiopathic pulmonary fibrosis) are nearly exclusively a lung problem from the beginning. Thus, the future lies as a branch midway between pneumology and internal medicine, but it is clear that currently the WASOG has its own experts, journal, society and patients.
 

From the president: Current concepts of the management of sarcoidosis

Sarcoidosis is a systemic disorder that preferentially affects the lungs. As a multi-organ disorder, patients may present initially to various organ specialists, but a pulmonary specialist should finally take over the management of the patient which often requires a multi-disciplinary approach. The diagnosis of sarcoidosis is based on a compatible clinical and/or radiological picture, histological evidence of non-caseating granulomas and the exclusion of other diseases capable of producing a similar histological or clinical picture. The corner stone of disease management involves careful baseline assessment of disease distribution and severity by organ, with emphasis on vital target organs. Because the clinical course can be unpredictable, regular monitoring for signs of disease progression is necessary, using least invasive and most sensitive tools available. The indication to treat a patient depends on many factors, the most important being whether or not the patient is symptomatic. Except for life- and site-threatening organ involvement, it should be carefully considered whether the patient might benefit from treatment. For asymptomatic pulmonary patients, a watch and wait is appropriate; treatment should mainly be considered if symptoms develop or lung function deteriorates. Initial systemic therapy is still based on corticosteroids. However, most patients with chronic disease require months to years of therapy. Alternatives to corticosteroids include methotrexate, azathioprine, and hydrochloroquine, all given usually in combination with low dose corticosteroids. For refractory sarcoidosis patients, new therapeutic approaches have begun to emerge through the use of immuno-modulatory agents. Based on current understanding of pathogenic mechanisms, these are TNF-alpha-blocking drugs, such as infliximab, thalidomide, and pentoxifyllin.

U. Costabel (CV), MD, PhD. Department Pneumology/Allergy, Ruhrlandklinik, Essen, Germany
 

History of the WASOG and current activities

Prof. Gianfranco Rizzato (Milan), former general secretary of the WASOG (World Association for Sarcoidosis and Other Granulomatous Disorders), describes the history of WASOG in a chapter of the Monograph of the European Respiratory Society (Rizzato G. Eur Respir Mon 2005; 32: 235-236). WASOG was founded on the September 7, 1987 in Milan, Italy, during the World Congress for Sarcoidosis. Activities of the WASOG, the publication of the journal Sarcoidosis, Vasculitis and Diffuse Lung Diseases and current activities are summarized.

 

Interstitial lung disease and sarcoidosis: a historical note

The first description of an interstitial lung disease appears to have been by Bernardino Ramazzini da Capri (1633-1714). The author, in the chapter on the Diseases of Sifters, Measurers, and Handlers of Grain, of his famous book De morbis artificium diatriba, described the occurrence of dry cough, weight loss, breathing difficulty, and dropsy in these workers. 1868, Austin Flint described a nondescript lung disease that was characterized by florid inflammatory exudation, without pus, causing solidification and fibrosis of the lungs. Flint alluded that Rokitansky had observed a similar condition in which exudation had occurred into the interlobular tissue. A few years earlier, Dominic Corrigan, an Irish cardiologist, had called the similar entity cirrhosis of the lung as it was analogous, not identical, to cirrhosis of the liver. Corrigan also described the occurrence of traction bronchiectasis and Flint observed that the association of clubbing and interstitial pneumonitis/fibrosis. Wilson Fox, professor of pathological anatomy at the University College, London, recorded the microscopic changes of capillary edema; accumulation of pigmented epithelium in the alveoli; and thickening of the walls of the alveoli, and veins in lungs with interstitial pneumonitis.

In 1944, Louis Hamman and Arnold Rich, both at the Johns Hopkins University School of Medicine, described four young patients who died of progressive dyspnea within 6 months of onset. The condition was similar to that described earlier by Flint, Corrigan, Fox, Charcot, and Osler. The term Hamman-Rich syndrome, however, became a synonym for an interstitial pneumonia of unknown cause followed by fulminating pulmonary fibrosis. It was soon apparent that the course of this new disease was not always acute, progressive, or fatal. Averil Liebow, on the basis of his extensive clinical and pathologic sciences, classified interstitial pneumonitis into five different histologic types: usual interstitial pneumonitis (UIP), desquamative interstitial pneumonia (DIP), bronchiolitis obliterans interstitial pneumonia (BIP), lymphoid interstitial pneumonia (LIP), and giant cell interstitial pneumonia (GIP). The new classification of idiopathic interstitial pneumonia includes UIP and DIP from Liebow’s original classification and two new entities, acute interstitial pneumonia (AIP, or Hamman Rich syndrome) and NSIP. Bronchiolitis obliterans organizing pneumonia (BOOP) is not included because it is primarily an intra-luminal disease.

Jonathan Hutchinson, a surgeon-dermatologist, identified the first case of sarcoidosis at the King’s College London. In the decades before and following the turn of the nineteenth century, several publications independently drew attention to the multisystem nature of the disease (Sharma OP. Eur Respir Mon 2005; 32: 1-12). This trend continued to the rest of the twentieth century and has persisted into the present century. Although the clinical, radiological, physiological, biochemical, and immunological aspects of the granuloma formation are well investigated, etiology of sarcoidosis needs to be uncovered by developments in the field of genomics and proteomics.

Om P. Sharma MD (CV), FRCP. Keck School of Medicine, Los Angeles, California USA.