1st Symptom at Onset of SSc Should Be Considered a Prognostic Factor, Study Shows

1st Symptom at Onset of SSc Should Be Considered a Prognostic Factor, Study Shows

The first clinical symptom at onset, also called the mode of onset, should be considered a prognostic factor in patients with systemic sclerosis (SSc), new research shows.

The study. “First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study,” was published in the journal Clinical Rheumatology.

SSc is an autoimmune disorder that can involve a form of Raynaud’s phenomenon, which refers to the discoloration of fingers or toes after exposure to changes in temperature or emotional events. The condition usually affects smaller arteries that supply blood to skin and distal parts of the body (such as fingers and toes).

Raynaud’s phenomenon is prevalent in almost all SSc patients, affecting 95% of patients that fit the 2013 ACR/EULAR criteria.

The onset of SSc is most frequently in the form of Raynaud’s phenomenon. However, some patients have other modes of onset including visceral involvement, musculoskeletal involvement or skin thickening without Raynaud’s phenomenon.

Several risk factors are related to poor SSc prognosis, including increased age at onset, male gender, diffuse subtype, and visceral involvement. However, no study until now has taken into account the mode of onset and how it can relate to a poor prognosis.

Therefore, researchers described the clinical spectrum of a large group of patients with SSc from the Spanish Scleroderma Registry (RESCLE) to determine the relationship between mode of onset and prognostic outcome.

Among the cohort of 1,625 patients, 1,342 patients (83%) presented Raynaud’s phenomenon as the first symptom. The remaining 283 patients (17 percent) did not have Raynaud’s phenomenon, but did have puffy hands, arthralgia/arthritis, skin sclerosis, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), or esophageal hypomotility.

When patients had with more than one symptom, it was up to the discretion of the physician to choose the first symptom.

Survival was determined at different intervals. At each time point, patients with Raynaud’s phenomenon as the mode of onset had higher survival than patients with non-Raynaud’s phenomenon onset. The percentage of survival for patients with Raynaud’s onset vs. non-Raynaud’s onset at five years was 97% vs. 90%, at 10 years 93% vs. 82%, at 20 years was 83% vs. 62%, and at 30 years was 71% vs. 50%, respectively.

The mode of onset of puffy hands or pulmonary involvement were particularly associated with higher mortality.

When looking at other factors, mortality was also associated with older age at onset, male gender, diffuse cutaneous systemic sclerosis (dcSSc), ILD, PAH, scleroderma renal crisis (SRC), and heart involvement.

“The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud’s phenomenon may be considered of poor prognosis,” the researchers concluded.


  1. Joe says:

    I thought it was the length of time you had Raynaud’s before other symptoms that was prognostic…, a short time means more like to be diffuse,while many with limited have had Raynaud’s for years brefore any other symptoms?

  2. Marilyn Gigliuto says:

    Interesting,my Scleraderma did start the acid reflux and reynauds at basically the same time,although it meant nothing to me,I was extremely healthy at the time,went to a great(lucky for me)Rheumatology for right hand finger tips questioning if it was my arthritis,he ordered Hand x rays,He then asked questions,I told him the past started that winter hands getting numb like,so I’d run them under hot water,then rub them awhile,then they’d be fine,I had lived in Florida the past 20 years,just recently moved back to Connecticut,So I thought the cold climate change affected my hands really thought nothing of it.This was now July.When discussing things with Dr.Wong,he ordered Blood tests.Called me a week later,for me to comeback to discuss tests.Well,long story shorter,said it showed Scleraderma and lupus!My husband and I had never heard of scleroderma,I had discoid lupus,which went into remission when I was 40,si he explained it,but I was I thought healthy.Now 2 or 3 was later my arms had tremendous pains running up and down,like my veins or arteries were feeding,very painful,he ordered plaquenil and prednisone,I was leaving the next day for a two week stay in Florida,walking normal,just couldn’t have arms touching armrest,sensitivity,pains still running up and down,landed ,deplaned,got my luggage,my sister met me,left airport,made a stop to have dinner,still fine,leaving restaurant walking back to car,turned to enter back seat,got dizzy for a minute,got into car,my feet started,had to take off my shoes,the carpet felt like needles in my feet,that was the start of it,well,3 weeks later it was full on,start now finally getting appt.with a rheumatologist,more blood test to determine,yes Scleraderma and neuropathy.So much more time changed and pain,never going away,legs now stiff,hard,like steel vise inside now up to top of knees,wrist to elbows,hands now totally numb,but folding well,still walking.So hoping for magic,Lyrica does nothing.

  3. Sherryl says:

    Do you think the cold weather triggered it? I moved from souther Ca to the Pacific Northwest although beautiful the cold winters triggered my CREST. I hadn’t seen a dr for Raynauds for 35 yrs.

    • Delia says:

      I sometimes think the cold weather could trigger it. I am from Spain, and since I am living in Ireland I started to have Raynaud phenomenon. I sometimes think to go to Canary Island to see if this would disappear or at least would not have symptoms.

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