Studies of scleroderma at The Alfred Hospital, Melbourne

Abstract

Scleroderma had been virtually unrecognized in this country before this study. Our interest in this condition was raised by the discovery that certain patients being investigated for ischaemic disease of the hand had scleroderma. Although uncommon, it is not excessively rare and we have been able to study an increasingly large number of patients, eventually resulting in 177 patients over a period of 35 years. The clinical features in these patients have been delineated. At first, the patients were subdivided into types: type 1, skin changes obvious only in the hands; type 2, skin changes extending beyond the hands but excluding the trunk; type 3, skin changes diffuse and involving the trunk. All types have similar visceral changes, but these are more severe and there is a worse prognosis in type 3 patients. Types 1 and 2 can conveniently be combined as acrosclerosis. Types 1 and 2 have a similar and good prognosis with survival at 30 years of 40%. Type 3 patients have a much worse prognosis, with no type 3 patients living more than 20 years. All types have a high incidence of autoantibodies, but these are generally not related to the severity of the disease and do not occur in relatives or spouses, this being the evidence of the absence of hereditary and environmental factors in their presence. Although patients may receive much relief from symptomatic measures, no treatment had lessened the skin stiffness and there is no specific treatment for the visceral lesions. The cause of the condition remains unknown.

Scleroderma: it has been a long hard journey

“…Now in his nineties, we are fortunate that Dr Barnett has remained an active researcher; in the current issue of this journal, we read of his 40 years experience with this illness. 3 Perhaps, his most insightful contribution to the scleroderma literature was the realization that scleroderma is a heterogenous disorder and that its prognosis is in part related to the extent of skin involvement. Dr Barnett suggested that three clinical variants could be defined depending on the extent of skin involvement: type 1, skin involvement of hands only; type 2, skin changes extending beyond the hands but excluding the trunk and type 3, diffuse skin changes involving the trunk; with the worst outcome being in patients with type 3 variant.…”

“…One Australian worker who has made a substantial contribution to scleroderma research is Dr Alfred Barnett, formerly of the Alfred Hospital, Melbourne. Now in his nineties, we are fortunate that Dr Barnett has remained an active researcher; in the current issue of this journal, we read of his 40 years experience with this illness 3 . Perhaps, his most insightful contribution to the scleroderma literature was the realization that scleroderma is a heterogenous disorder and that its prognosis is in part related to the extent of skin involvement.…”