Scleroderma is an incidious and chronic disease of unknown cause. The clinical picute of scleroderma is characterized by the apperance of circumscribed of diffuse, hard, smooth, ivory colored areas that are immobile upon upon the underlying tissues. It may be classified to as a localized form (morphea) and a systemic scleroderma or progressive systemic sclerosis (PSS) which is characterized by diffuse involvment of the connective tissue of the skin and certain intemal organs. The authors observed 3 cases of PSS and 6 cases of morphea. They have complained of marked sclerosis or thickening of skin or slowly developing dyspneadue to interstitial pulmonary fibrosis which were confirmed by chest X-ray. Recently, penicillamine-D has been advocated as a beneficial agent in scleroderma by Asboe-Hansen. therefore, penicillamine-D was administered to the above patients. Two of PSS were markedly improved with normal skin softness. Two of morphea were also moderately improved. Other patients remained stationary or no effect.
Chronic Disease ; Connective Tissue ; Humans ; Pulmonary Fibrosis ; Scleroderma, Diffuse ; Scleroderma, Localized ; Scleroderma, Systemic ; Sclerosis ; Skin ; Thorax

PURPOSE: The aim of this study was to investigate the anorectal function in patients with progressive systemic sclerosis (PSS), thus to define the clinical role of anorectal manometry in the earlier diagnosis of anorectal involvement of PSS. METHODS: Seventeen consecutive patients (all females) with PSS were evaluated with anorectal manometry by the stationary pullthrough technique using the 8-channel hydraulic capillary infusion system for anorectal function. Functional parameters of the manometry were compared between patients with PSS and 20 normal control subjects, matched for age and sex. RESULTS: The mean resting pressure over the high pressure zone (HPZ) in patients with PSS was significantly lower than that in the control group (70.8 3.4 mmHg vs. 81.5 3.2 mmHg: P=0.046). The HPZ in patients with PSS was also significantly reduced compared with that in the control (1.5 0.1 cm vs. 2.5 0.1 cm: P=0002). The rectoanal inhibitory reflex (RAIR) was detected in only 10 patients (59%) in the PSS group, but was present in all except one (95%) in the control (P=0.022). More interestingly, RAIR in patients with PSS responded at a higher volume of the air insufflated than that in the control (74% vs. 30% at 20 cc, 21% vs. 30% at 30 cc, and 0% vs. 40% at 50 cc, respectively: P=0.031). Other functional parameters, including maximal squeeze pressure, minimal sensory and maximal tolerable volume of the rectum, and rectal compliance were not significantly different between two groups. CONCLUSIONS: Anorectal involvement reflected by the anorectal manometric dysfunction may be rather an earlier event in patients with PSS. An awareness to perform an anorectal manometric study in every case of PSS may be necessary for earlier subclinical detection of anorectal involvement by the disease.
Capillaries ; Compliance ; Diagnosis ; Humans ; Manometry ; Rectum ; Reflex ; Scleroderma, Diffuse*

An open lung biopsy was performed in 12 children with diffuse parenchymal lung disease. A definitive histopathological diagnosis was obtained from all procedures but determined treatment options in only 10 children (83%). Three (25%) children were ventilated for respiratory failure prior to the procedure. Four (44%) of the other 9 children required ventilatory support after the procedure. Three (25%) children developed post-op pneumothorax that resolved fully with chest tube drainage. There were no deaths as a direct result of the procedure. Open lung biopsy is useful in providing a definitive diagnosis in children with diffuse parenchymal lung disease and determining treatment in the majority of cases. The procedure was well-tolerated with minimal complications.
*Biopsy ; Lung/*pathology ; Lung Diseases/*pathology ; Scleroderma, Diffuse

Systemic sclerosis is an intractable clinical subset characterized by diffuse fibrosis, vasculopathy, and immune abnormalities. Diffuse cutaneous systemic sclerosis has a variety of symptoms depending on the internal organs involved, such as the lungs, heart, kidneys, and gastrointestinal system. Gastrointestinal involvement is frequent and one of the major cause of morbidity and mortality. There is no sustained, effective therapy for treating the active gastrointestinal involvement of systemic sclerosis. Although immunoglobulin is commonly used in the treatment of other autoimmune diseases, its effects in the treatment of the gastrointestinal involvement in systemic sclerosis are unclear. Here, we report a patient with a severe cough caused by uncontrolled gastrointestinal involvement in diffuse cutaneous systemic sclerosis treated with immunoglobulin.
Autoimmune Diseases ; Cough ; Fibrosis ; Heart ; Humans ; Immunoglobulins* ; Kidney ; Lung ; Mortality ; Scleroderma, Diffuse ; Scleroderma, Systemic*

Systemic sclerosis is a connective tissue disease characterized by cutaneous and visceral fibrosis, as well as vascular disease involving arterioles, small and medium arteries of the peripheral circulation. Digital ulcers, defined as necrotic lesions that occur either at distal aspects of digits or over bony prominence, occur in up to 50% of patients with limited or diffuse systemic sclerosis. These lesions are exquisitely painful, heal slowly, and lead to substantial functional disability. We describe a 59-year-old woman with systemic sclerosis, suffering from a painful, non-healing digital ulcer despite conventional therapies, who demonstrated dramatic improvement with oral sildenafil treatment.
Arteries ; Arterioles ; Connective Tissue Diseases ; Female ; Fibrosis ; Humans ; Middle Aged ; Scleroderma, Diffuse ; Scleroderma, Systemic* ; Sildenafil Citrate ; Ulcer* ; Vascular Diseases

OBJECTIVE: The aim of present study was to determine the differences in clinical features,laboratory data and autoantibody profile between limited and diffuse subsets of progressive systemic sclerosis in Korea,and to see differences from foreign studies on the same disease. METHODS:Fifty-six patients with systemic sclerosis at the Hospital for Rheumatic Diseases were studied for age at diagnosis,subtype,clinical features, laboratory data,systemic involvement and pattern of autoantibodies by careful review of medical records and interviews. RESULTS:The disease was relatively more prevalent in female groups of third,forth and fifth decade (male:female=1:17.7,mean age:39.5,range:11-68 years).The patients consisted of 29 diffuse and 27 limited subsets. Clinical features were as follows: sclerodactyly (92.9%),Raynaud 's phenomenon (92.9%), dyspnea (71.4%),arthralgia/arthritis (64.3%),dry cough (58.9%),myalgia(51.8%),rale (48.2%),esophageal symptoms (41.1%),digital pitting scar(41.1%),telangiectasia (28.6%).Antinuclear antibody was positive in all 54 cases (100%)which is tested.The respiratory system involvement was statistically higher in diffuse subset than in limited subset (77.4 vs.52.0%,p=0.046). The anti-centromere antibody was detected in 1 case with limited subset and the anti-Scl-70 antibody in 6 cases (11.3%)with diffuse subset. CONCLUSION:Interstitial lung disease was more prevalent in patients of this study than patients of other countries.Investigation of pulmonary system is needed,especially in earlier stage and diffuse type.Systemic sclerosis-related autoantibodies (anti-Scl 70 antibody,anti-centromere antibody)are less prevalent than the results of other studies.
Autoantibodies ; Cough ; Dyspnea ; Female ; Humans ; Korea* ; Lung Diseases ; Medical Records ; Respiratory System ; Rheumatic Diseases ; Scleroderma, Diffuse* ; Scleroderma, Systemic

Extensive tumoral calcinosis affecting a large joint is uncommon in patients with systemic sclerosis. We report the case of a 52-year-old female patient referred for a growing calcified mass in the shoulder. She was diagnosed with interstitial lung disease and progressive systemic sclerosis. Although the pain and disability associated with the affected joint was not severe, the patient underwent surgical excision because the mass continued to grow and was likely to produce shoulder dysfunction and skin ulceration. The patient appeared well 10 months after surgery with no signs of recurrence. This report highlights the timing and indication of surgical excision in similar cases.
Calcinosis* ; Female ; Humans ; Joints ; Lung Diseases, Interstitial ; Middle Aged ; Quality of Life ; Recurrence ; Scleroderma, Diffuse ; Scleroderma, Systemic* ; Shoulder ; Skin Ulcer

Autoimmune polyendocrinopathy syndrome is a heterogeneous group of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can be affected. We present a case of autoimmune polyendocrinopathy syndrome type 2 in a 42-year-old woman with Guillain-Barre syndrome and scleroderma. This combination of syndromes has not been reported and warrants further investigation.
Adult ; Female ; Guillain-Barre Syndrome ; complications ; diagnosis ; Humans ; Polyendocrinopathies, Autoimmune ; diagnosis ; Scleroderma, Diffuse ; complications ; diagnosis

Sjogren's syndrome(SS) is a chronic inflammatory disorder characterized by lymphocytic infiltration of lacrimal and sailvary glands, which results in dry eyes and dry mouth. SS may exist as a secondary condition or as a secondary condition in association with connective tissue disease such as rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. We experienced a patient with primary SS who developed multiple bullae, nodular type of pulmonary amyloidosis and lymphocytic interstitial peumonitis. We believe this to be the first reported case of SS acompanied by these three types pulmonary manifestations at the same time.
Amyloidosis* ; Arthritis, Rheumatoid ; Bronchioles* ; Connective Tissue Diseases ; Humans ; Lupus Erythematosus, Systemic ; Mouth ; Scleroderma, Diffuse ; Sjogren's Syndrome*

Scleroderma is a systemic disorder with multiorgan involvement. About 90% of patients with scleroderma has Raynaud's phenomenon and microvascular involvement is well recognized in scleroderma, but macrovascular involvement is not recognized. We experienced 2 cases of scleroderma with peripheral vascular occlusions that had been diagnosed by fernoral angiography. The one patient with limited scleroderma had anticentromere antibody and angiography of both fernoral arteries showed nonvisualization of posterior tibial artery. The other patient with diffuse scleroderma had anti-Scl-70 antibody and angiography of right fernoral artery showed occlusion of both anterior and posterior tibial arteries and stenosis of distal portion of peronial artery. Both patients didn't have the risk factors of atherosclerosis such as hypertension, hypercholesterolemia, obesity, and smoking. In recent years, there have been reports that scleroderma is associated with macrovascular disease. In the future, the study on the freguency, prevention and treatment of macrovasular disease in scleroderma is necessary. We report 2 cases of scleroderma with peripheral vascular occlusions that had been diagnosed by fernoral angiography with review of literatures.
Angiography ; Arteries ; Atherosclerosis ; Constriction, Pathologic ; Humans ; Hypercholesterolemia ; Hypertension ; Obesity ; Peripheral Vascular Diseases* ; Risk Factors ; Scleroderma, Diffuse ; Scleroderma, Limited ; Smoke ; Smoking ; Tibial Arteries