I recently got together with a wonderful bunch of fellow sclerodermians, we all had scleroderma but not one of us was the same. As expected scleroderma was the hot topic, in particular types of scleroderma, as in what are they and who has what. Good question as I have grown up in the weird ‘n’ wacky world of scleroderma thinking there are two main types of systemic scleroderma, diffuse and limited, with the CREST acronym no longer in use. To my surprise, my dear friend said that a rheumatologist told her she had both limited and diffuse, I queried this having never heard of it before and not being able to fathom out how you could have both limited, slow in onset, slow in progression and more favourable in outcome and diffuse, rapid in onset, rapid in progression, poor outcome. Was localised included in this blurring of types?
Well having thought about it, now it makes perfect sense! My dear friend could have both and as it happens so could I! I was diagnosed August 2007 with diffuse and my symptoms had started in February 2007, within 6 months I went from working full time to being so debilitated that I struggled to walk. I had skin involvement above the elbows and knees and on my back and abdomen, all fitting the diffuse criteria for sure.
However years prior I was diagnosed with IBS and at one point was thought to have an ulcer, remember that gastrointestinal involvement is a hallmark of limited scleroderma. Having been an avid gym attendee I found exercising a struggle being more fatigued than usual, eventually giving up exercise altogether. A patch of tight, waxy skin appeared on my right shin and grew larger, then appeared on the left shin, being misdiagnosed as necrobiosis lipoidica, it was in fact tight skin from scleroderma.
In 2004 I was diagnosed with interstitial cystitis, my first autoimmune disease and one associated with scleroderma. When I was younger my hands had always been white and cold and I was also always cold. I slept a lot as well making sure the weekend included a nap or two, without which I found it difficult to cope. Looking back I could have had mild Raynaud’s as early as my twenties, limited scleroderma as early as 2003, albeit atypical presentation, with diffuse coming on in 2007. I also have localised scleroderma, morphea, but I am not sure where that fits in.
My dear friend also said there may not be limited or diffuse but just scleroderma and when you see how different we all are, it's like we each have our own disease, this makes sense. It certainly makes it easier to fit in those of us with atypical presentations who do not start with Raynaud’s and tight skin on the fingers or hands.
I guess the issue with the lack of distinction is the difference between treatment of limited and diffuse. Diffuse usually demands close monitoring and aggressive treatment whereas the approach to limited can be more relaxed. If you don’t know what type of scleroderma you are dealing with how can you treat it? If treatment is symptom lead surely that ticks the boxes, except what about preventing the symptoms in the first place? Isn’t that where immunosuppressants come in, dampen down the immune system and hopefully slow down disease progression?
Having had scleroderma now for 6 years I find this all fascinating, the very types of scleroderma could be up for discussion! What diagnosis, I wonder will I be left with?
This is such a complex disease! Think about it, everyone of us here has scleroderma but we all have a different experience of it and unlike other diseases there’s no real disease path or predicted outcome, we’re flying by the seat of our pants in the weird ‘n’ wacky world of scleroderma.