Retrospective studies in scleroderma: Effect of potassium para-aminobenzoate on survival

Abstract

Demographic and survival data are presented for 390 patients with scleroderma. For the entire group an estimated 81.4% survived 5 years from diagnosis and 69.4% survived 10 years. Life-table analyses revealed that adequate treatment with potassium para-aminobenzoate (Potaba KPAB) was associated with improved survival (p less than 0.01); 88.5% 5 year survival rate and 76.6% 10 year survival rate for adequately treated patients. Five and ten year survival rates for patients never treated with KPAB were 69.8 and 56.6%, respectively. Similar findings were obtained by comparing observed to expected mortality for these patients; again, KPAB therapy showed prolongation of survival. The Cox proportional hazards model was also applied to this retrospective study adjusting for baseline clinical involvement, demographics and KPAB treatment. There were some interesting results including a high significance for skin involvement per se as a prognostic indicator: the greater the extent of skin involvement the poorer prognosis. Time from first diagnosis to first University Hospital visit or admission when included as a covariate did not influence survival.

Survival and causes of death in 366 Hungarian patients with systemic sclerosis

“…Previous univariate survival analysis studies indicated that the presence of diffuse skin involvement,1 – 9 male sex,4 6 8 11 older age at the onset of the SSc,1 – 3 5 – 7 12 – 14 internal organ manifestations including the heart,1 – 7 10 – 13 15 – 17 kidney,2 – 4 6 7 13 – 15 17 18 gastrointestinal tract,2 15 18 lung involvements,1 – 7 10 11 14 15 18 pericarditis,3 4 14 clinical signs of right heart failure,7 11 pigmentation disturbances,2 nailfold capillary loss seen on capillaroscopy,14 joint contractures,10 and decreased oral aperture1 were associated with poor survival. Anti-topoisomerase antibody,1 5 7 anaemia,1 – 3 13 14 18 increased erythrocyte sedimentation rate (ESR),1 – 3 5 10 13 increased C-reactive protein (CRP) level,4 abnormal urine sediment,1 16 proteinuria without scleroderma renal crisis1 13 were also associated with a bad prognosis of the disease, while anti-centromore (ACA) positivity6 was a favourable prognostic factor.…”

“…The Cox proportional-hazards model showed that older age,3 6 11 12 14 19 renal involvement,3 6 14 19 severe lung involvement,2 5 13 18 clinical signs of right heart failure,7 11 the presence of diffuse SSc,3 6 and a greater extent of skin involvement2 were unfavourable independent prognostic signs.…”

Hematopoietic stem cell transplantation for systemic sclerosis

“…Several studies have evaluated extent of skin and organ involvement, nail bed capillary involvement, autoantibodies, and signs of inflammation or immune activation for prognostic significance. In univariate analysis, numerous poor prognostic factors have been reported including diffuse skin involvement [3][4][5][6][7][8][9][10][11][12][13], male sex [3,8,10,14], older age at the onset of the SSc [3][4][5][6][7]9,13,[15][16][17], internal organ involvement including the heart [3-9,12, [14][15][16][18][19][20], kidney [3,4,6-8, 13,[16][17][18]20,21], gastrointestinal tract [6, 18,21], lung [3-9, 14,17,18,21,22], presence of pericardial effusion or pericarditis [7,8,17], clinical signs of right heart failure [9,14], antitopoisomerase antibody positivity [4,…”

Survival, Mortality, Causes of Death and Risk Factors of Poor Outcome

“…[43]. In base of this last study based on 17 studies, we could state that mortality is over 2.7-fold compared to the general population ( Table 4 and Figure 2) [5,17,24,26,29,30,31,33,37,40,42,[48][49][50][51][52][53]. Mortality has been decreasing over time and notoriously after 1990, being more reasonable to accept nowadays an SMR over 2.4-fold in a patient diagnosed today ( Figure 2).…”