EARLY DIAGNOSIS of the potentially fatal disease scleroderma is essential if patients are given a chance to survive, a conference was told at the weekend.
Scleroderma is an incurable and potentially fatal disease of the immune systems, blood vessels and connective tissue, which can affect all major organs of the body. If caught in time, the disease’s progress can be arrested.
The 20th annual conference of the Irish Raynaud’s Scleroderma Society (IRSS) was told it takes almost 10 years to diagnose scleroderma in Ireland.
A survey of scleroderma sufferers found it took them 3.1 years from the outset of symptoms of Raynaud’s, which usually involve painful circulation problems, to be diagnosed with that disease and a further 6.6 years to the diagnosis of scleroderma. About 2 per cent of Raynaud’s patients develop scleroderma, by far the most common way in which the disease is contracted.
Italian professor Marco Matucci-Cerinic, a world authority on scleroderma, said that although the disease was difficult to diagnose, there were telltale signs signalling onset of scleroderma which could lead to early diagnosis.
He told the conference that patients who presented themselves with severe Raynaud’s and with puffy fingers would go on to have scleroderma and they should be monitored on a regular basis to ensure early intervention.
About 150 specialists and health professionals working in the field attended the conference which was addressed by the Minister for Health and Children, Mary Harney, at the Stillorgan Park Hotel, in Dublin.
Ms Harney praised the recent establishment of the first National Scleroderma Service which opened in St Vincent’s Hospital, Dublin, last month.
The service will consist of a scleroderma specialist nurse who will be able to refer patients to other specialists such as rheumatologists and cardiologists.
Consultant rheumatologist Prof Douglas Veale, who is overseeing the service in St Vincent’s, said he was sure that it would be of benefit for patients as it was supported by consultants in different units.
He said that scleroderma was a relatively rare disease and early assessment and therapy now appeared to be both “feasible and beneficial”.
Prof Sean Gaine, a respiratory consultant at the Mater Hospital, said most patients who developed scleroderma were dying now from lung diseases, most notably pulmonary-hypertension and fibrosis.
“The good news is that early diagnosis and early institutional treatment is very effective in reducing symptoms and delaying the progression of the disease,” he said.
Ann Tyrrell Kennedy, the chief executive of the Irish Raynaud’s and Scleroderma Society, said scleroderma patients suffered because the disease was difficult to diagnose and GPs were not trained to notice the symptoms.
