Crackling sound from the lungs in Sclero Forums (MAIN) Posted April 30, 2015 · Report reply Hello Ronald, Over the years the main cause of death in scleroderma has changed, it was from kidney involvement and is now, I believe, lung involvement. However, this is not because lung involvement has become worse or ever was worse but because survival of a renal crisis has improved. Doctors are now aware of renal crises in scleroderma, I have a friend who had a renal crisis some 20+ years ago and nearly died before they tied it to the scleroderma, today they would be more on the ball and as we know, prompt diagnosis also enables prompt treatment. Now lung involvement, including pulmonary fibrosis and pulmonary hypertension, is the most critical symptom. Well that's what it says "on paper" but of course the most critical symptom is the one that affects the individual the worst. I have a friend with complete gastric failure, the doctors pronounced her terminal but she's still alive and has even gained weight albeit through TPN (total parenteral nutrition). She was advised that gastrointestinal involvement was the biggest killer and I have also read that somewhere myself. I myself have myocardial fibrosis and had a 2 year life expectancy back in 2010 but thanks to a biventricular ICD I'm still here! I am sure that statistically something or another is more likely to kill in systemic scleroderma but then how accurate are the stats and what about individual circumstances? Firstly stats, there must be as many undiagnosed and misdiagnosed people with scleroderma as there are those diagnosed with it and I wonder what they die of and whether that would shift the focus from lung back to kidney or to the heart or remain on the lung? Of course personal circumstances are also a big player, someone with systemic scleroderma living in the US who has say moderate to severe pulmonary hypertension may well live longer than someone living in India with say complete gastric failure. Someone with pulmonary fibrosis living in the UK, diagnosed and treated immediately may well live longer that someone with kidney involvement, misdiagnosed and then given high dose steroids. It's swings and roundabouts for sure! For you the priorities are diagnosis of the systemic involvement, which you now have, then treatment which you will soon have hopefully and support which will be ongoing if you stick around...I means as in if you choose to keep posting! I am still amazed at how people buck the trend and continue to live for years when they should in fact have kicked the bucket or passed over or whatever euphemism is preferred. Remember that you have lived for nearly 30 years with this disease so you have had a good long run and it's perfectly possible that you will continue to do so! Clearly you're doing many things right! Take care.