| Objective:Clinical evaluation and analysis of patients with systemic scleroderma, leading to more knowledge to the disease and inspiration of the diagnosis and treatment.Methods:71 cases of systemic scleroderma from in-patient department of dermatology of Huashan Hospital during March 2002 and December 2009 were collected. Medical history, clinical manifestation, laboratory and auxiliary examination were analyzed and discussed.Results:Among the 71 cases, ratio of male to female was 1:2.55, and ratio of acroscleroderma and diffuse scleroderma was 1:0.16. The initial onset age ranged from 9 to 78 years old and the duration of disease ranged from 0.06 to 21.83 years. The percentage of diffuse scleroderma in male patients (30.0%) is higher than female (7.8%) with statistical difference (P<0.05). The mean duration of male patients [(0.77±0.59) years] was shorter than female ones [(3.21±4.48) years] with statistical difference (P<0.05). The mean initial onset age of acroscleroderma [(39.31±13.63) years] is less than diffuse scleroderma [(54.80±20.50) years], while the mean duration [(2.76±4.22) years] of acroscleroderma was longer than diffuse scleroderma [(1.08±0.68) years], both showing statistical difference (P<0.05). Sclerotic lesion existed the most generally in all patients (85.9%), while numerous lesion types could be coexistent. Raynaud phenomenon was found in 50 cases, all belonging to acroscleroderma cases. Among all the acroscleroderma cases, the mean duration of disease in patients with Raynaud phenomenon [(3.15±4.56) years] was longer than the ones without Raynaud phenomenon [(0.97±0.69) years], showing statistical difference (P<0.05). The percentage of Raynaud phenomenon in female patients (83.0%) was higher than male ones (78.6%). The percentage of ANA positive and anti-Scl-70 antibody positive (92.0%,44.0%) of patients with Raynaud phenomenon were both higher than the ones without Raynaud phenomenon (72.7%,36.4%). Digital ulcers was found in 8 cases, who were all acroscleroderma ones with Raynaud phenomenon. Among all the acroscleroderma patients with Raynaud phenomenon, males showed a higher percentage of digital ulcers (27.3%) than females (12.8%). ANA positive were found in all patients with digital ulcers, while the percentage of anti-Scl-70 antibody positive in patients with digital ulcers (37.5%) was less than the ones without digital ulcers (45.2%). Puffy fingers were found in 15 cases. The percentage of puffy fingers in female patients (23.5%) was higher than male ones (15.0%), and the percentage in acroscleroderma cases (23.0%) was higher than diffuse scleroderma ones (10.0%). ANA positive were found in all patients with puffy fingers, while the percentage of anti-Scl-70 antibody positive in patients with puffy fingers (27.8%) was less than the ones without puffy fingers (39.6%). Hyperpigmentation was shown in 46 cases (64.8%) and hypopigmentation in 29 cases (40.8%). Poikiloderma-like changes existed in 4 cases (64.8%). Telangiectasis existed in 9 cases (12.7%), which were all acroscleroderma cases. Subjective symptoms were found in 20 cases (28.2%), such as pruritus, numbness, pain and burning sensation. Arthralgia was found in 20 cases (28.2%), activity limitation of articulation in 9 cases (12.7%), muscular soreness in 11 cases (15.5%), muscle weakness in 2 cases (2.8%), dysphagia or heartburn in 9 cases (12.7%), gastric fullness in 1 case (1.4%), dyspnea in 4 cases (5.6%), tachypnea postexercise in 2 cases (2.8%), dry cough in 1 case (1.4%), fever in 2 cases (2.8%), fatigue in 3 cases (4.2%), anorexia in 5 cases (7.0%) and apparent weight loss in 9 cases (12.7%). None of the symptoms above showed statistical differences between male and female cases, or between acroscleroderma and diffuse scleroderma ones (P>0.05). The incidence rate of articular symptoms was higher in male patients (45.0%) than female ones (33.3%), and higher in diffuse scleroderma (40.0%) cases than acroscleroderma ones (36.1%).The percentage of anti-Scl-70 antibody positive was lower in patients with articular symptoms (50.5%) than the ones without the symptoms (28.9%). The incidence rate of muscular symptoms was higher in male patients (20.0%) than female ones (15.7%), and higher in diffuse scleroderma (20.0%) cases than acroscleroderma ones (16.4%). The percentage of anti-Scl-70 antibody positive was lower in patients with muscular symptoms (12.7%) than the ones without the symptoms (40.7%). Abnormalities could be found in laboratory examinations such as blood and urine routine, hepatic and renal function, myocardial enzymes. ANA positive was found in 59 cases (83.1%). The percentage of ANA positive was higher in acroscleroderma patients (88.5%) than diffuse scleroderma ones (50.0%), and also higher in female patients (92.2%) than male ones (60.0%), both showing statistical difference (P<0.05). Anti-Scl-70 antibody was found positive in 26 cases (36.7%). Decrease of complement level, increase of RF and blood sedimentation can also be found. Interstitial change of the lung was found in 12 cases (70.6%) among the 17 patient who took CT examination.50 patients took pulmonary function test, among which restrictive ventilatory disorder was found in 27 cases (54.0%), obstructive ventilatory disorder in 28 cases (56.0%), collapse of small airway in 30 cases (60.0%) and diffusion function disorder in 22 cases (44.0%).70 patients took electrocardiogram test and 37 cases showed abnormalities (52.9%).8 patients took echocardiogram test and hydropericardium was found in 2 cases (25.0%).50 patients took barium examination and 8 cases showed esophageal change (16.0%).16 patients took electromyogram examination and 6 cases showed myogenic lesion (37.5%) and 1 case showed neurogenic lesion (6.3%). None of the abnormal results of the auxiliary examination mentioned above showed statistical differences between male and female cases, or between acroscleroderma and diffuse scleroderma ones (P>0.05). Among the 12 cases in which interstitial change of the lung had been found, the percentage of female (83.3%) was more than the percentage of female in all the 71 cases in this study (71.8%).5 cases'duration of disease were less than 1 year (41.7%), and only 4 cases showed apparent clinical symptoms (25.0%). The initial onset age of 7 cases were not less than 50 years (58.3%). ANA positive was found in all the 12 cases, and the titer ranged from 1:100 to 1:10000. The numbers of speckled pattern, nuclear pattern and homogeneous pattern were more than expected value, while the number of centromert pattern was less than expected. The numbers of digital ulcers, puffy fingers, digital malformations, hyperpigmentation, hypopigmentation, telangiectasis, activity limitation of articulation, apparent weight loss, abnormalities of blood routine, increase of ALT and AST, decrease of albumin and prealbumin, decrease of Scr, ANA positive and anti-Scl-70 antibody positive were more than expected. Among the 8 cases in which esophageal change had been found, the percentage of male (37.5%) in the 12 cases was more than the percentage of male in all the 71 cases in this study (28.2%).5 cases'duration of disease were less than 1 year (62.5%), and 4 cases showed none clinical symptoms (50.0%). ANA positive was found in 6 cases, and the titer ranged from 1:1000 to 1:32000. The numbers of speckled pattern, nuclear pattern and homogeneous pattern were less than expected value, while the number of centromert pattern was more than expected. The numbers of Raynaud phenomenon, digital ulcers, hyperpigmentation, hypopigmentation, telangiectasis, poikiloderma-like changes, muscular soreness, apparent weight loss, increase of ALT, decrease of prealbumin, increase of BUN and decrease of Scr were more than expected, while the number of anti-Scl-70 antibody positive was less than expected.5 cases with renal impairment were all acroscleroderma ones with Raynaud phenomenon, including 3 male and 2 female patients. Nephrotic syndrome and acute renal failure happened in 1 male patient, with increase of BUN. The percentage of renal impairment in male (15.0%) was higher than female (3.9%).3 cases'duration of disease were less than 1 year (60.0%). ANA positive existed in all the 5 cases, and the titer ranged from 1:1000 to 1:32000. The numbers of speckled pattern, nuclear pattern and centromert pattern were more than expected value, while no homogeneous pattern was found. The numbers of Raynaud phenomenon, digital ulcers, puffy fingers, hyperpigmentation, telangiectasis, activity limitation of articulation, muscular soreness, muscle weakness, apparent weight loss, abnormalities of blood routine, decrease of albumin, increase of globulin, increase of BUN and ANA positive were more than expected, while the number of anti-Scl-70 antibody positive was less than expected. Hospital days of all the 71 cases ranged from 3 to 22. The majority showed improvement in the hardness and temperature of cutaneous lesions, Raynaud phenomenon, articular symptoms and muscular symptoms. Edematous lesions showed better improvement, while sclerotic and atrophic lesions'improvement was insignificant or none.Conclusion:1. Systemic scleroderma is observed predominantly in women, young or middle age. Acroscleroderma is the most frequent subset, with an earlier initial onset age. Diffuse scleroderma is seen more frequently in men, with a faster progression of disease. Men show a faster progression of disease.2. Raynaud phenomenon presents only in acroscleroderma, more frequently in women. Patients with Raynaud phenomenon show longer duration and severer state of disease. Patients with ANA and anti-Scl-70 antibody positive are more likely to develop Raynaud phenomenon.3. Digital ulcers presents only in acroscleroderma cases with Raynaud phenomenon, more frequently in men. Puffy fingers are observed more frequently in women and acroscleroderma cases. Patients with ANA positive are more likely to develop digital ulcers and puffy fingers, while patients with anti-Scl-70 antibody positive are less likely to develop digital ulcers and puffy fingers.4. Articular and muscular symptoms are observed predominantly in men and diffuse scleroderma. Patients with anti-Scl-70 antibody positive are more likely to develop articular symptoms, but less likely to develop muscular symptoms.5. The rate of ANA positive is higher in acroscleroderma than diffuse scleroderma, and higher in women than men.6. Lung is the most frequent internal organ involved, and then esophagus, kidney and heart in turn. Internal organ involvement in acroscleroderma cases is not rare. Internal organ involvement often occurs in early stage, always with severer state. Internal organ involvement has nothing to do with extent or stage of cutaneous lesions, or titer of ANA.7. Interstitial change of the lung is seen more frequently in women, middle age or senior. Patients with ANA and anti-Scl-70 antibody positive are more probable to develop the change, while patients with centromert pattern are less probable to develop it.8. Esophageal change is seen more frequently in men. Patients with centromert pattern of ANA are more probable to develop the change, while patients with anti-Scl-70 antibody positive are less probable to develop it.9. Renal impairment is seen more frequently in men with severer state of disease. Patients with ANA positive are more probable to develop the impairment, while patients with anti-Scl-70 antibody positive are less probable to develop it.10. In clinical works, laboratory and auxiliary examination related should be all taken if permission, in order to detect the abnormalities in early stage and give treatment in time, avoiding progressing and aggravation.
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