Systemic Sclerosis (CREST Syndrome): Complications and Management

00:00 So let's talk generally about systemic sclerosis.

00:03 First off, it's a chronic, progressive, multi-system disease and it's characterized by the pathologic fibrosis of a variety of organ systems due to an immune mediated replacement of normal tissue architecture and blood vessels with scar tissue.

00:18 That being said, it's -- at the present time, still largely idiopathic.

00:22 As of -- alluded to thus far, there are two different sub types.

00:26 There's limited cutaneous, lc, systemic sclerosis which most commonly presents as the CREST syndrome which is a more favorable prognosis. That being said, it still has a five-year survival of 90% and it is characterized by potentially disfiguring morbidity.

00:42 Next stop is diffused cutaneous systemic sclerosis.

00:46 For which you're gonna see more extensive cutaneous involvement often times proximal to the elbows and the survival for this is significantly less with about 70% five-year survival.

00:57 Lastly, diffused cutaneous systemic sclerosis is more commonly associated with aggressive interstitial lung disease and pulmonary artery hypertension.

01:05 So, let's talk a little bit more about those specific complications.

01:09 First off, we just alluded to the pulmonary complications and since pulmonary artery hypertension can be largely a somatic early disease, it's really important that patients are getting screened with an echocardiogram and ultimately, PFTs annually. Next stop is the skin manifestations.

01:26 We've already talked about sclerodactyly, you can also have microstomia which is well where the lips get increasingly contracted and it's harder and harder to open the mouth.

01:34 Calcinosis cutis and digital necrosis can also occur as well.

01:39 Importantly, those with more diffused skin involvement are more likely to have visceral organ involvement as well.

01:46 GI symptoms, esophageal dysmotility, gastroparesis, scleroderma, renal crisis.

01:53 This is a potentially devastating diagnosis as well.

01:55 Historically, it was almost universally fatal, associated with malignant hypertension, proteinuria and AKI.

02:02 Nowadays however, it's actually fairly responsive to ACE inhibitors so it's not quite the death sentence that it used to be.

02:08 Next stop is carpal tunnel syndrome, arthralgias, joint stiffness.

02:13 These are just other sorts of symptoms that go along for the ride and lastly, cardiac symptoms can be present as well with some evidence of myofibrosis.

02:22 In terms of the management, it's generally anticipatory rather than reactive.

02:26 You want this patient to be seen regularly by a number of different specialists and certainly somebody who's well-trained in managing patients with systemic sclerosis.

02:34 You don't wanna be catching up with diseases as they manifest.

02:37 You wanna look for them before they do. In terms of how to manage the disease, you maybe using methotrexate, hydroxychloroquine, or low-dose steroids which can be effective to help with a number of the manifestations that are present.

02:48 ACE inhibitors as I mentioned are best used to manage renal crisis.

02:52 If you're dealing with lung fibrosis, you can use cyclophosphamide or mycophenolate mofetil.

02:58 There's a lot of evidence that stem cell transplantation can really help with all the different manifestations of this disease and lastly, for folks who have intractable Raynaud's disease or progressive pulmonary hypertension, phosphodiesterase inhibitors can be helpful.

03:12 As you can sort of tell from our list here, the treatment here is largely supportive.

03:17 There's no cure for systemic sclerosis.

03:20 With that, let's highlight some key points in systemic sclerosis.

03:24 First, it's a chronic, progressive multi-system, inflammatory fibrotic disease of unknown etiology.

03:32 There's multiple phenotypes; namely, the limited cutaneous and diffused cutaneous types with varying severity and disease progression. Raynaud's is a classic presentation.

03:42 Skin thickening called sclerodactyly and the esophageal symptoms are things to look for.

03:47 In addition, the morbidity and mortality from systemic sclerosis is most often due to pulmonary artery hypertension and potentially renal crisis.

03:55 And lastly, this is a treatment limited disease. There are no cures.