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PubMed1984

[Clinical significance of antinuclear antibodies in progressive systemic sclerosis].

Indirect immunofluorescence (IIF) was used to detect antinuclear antibodies (ANA) in 42 clinical cases. In each case cryostatic rat kidney slices and cultivated HEp-2 cells were used as substrates. Clinical diagnoses were as follow: Progressive Systemic Sclerosis (PSS) 25 cases, of which 8 were acrosclerotic, 8 diffuse, 5 CREST syndrome, 1 overlap PSS + Systemic Lupus Erythematosus (SLE) and 3 PSS + myopathy; Localised scleroderma (morphea): 3 cases; Mixed Connective Tissue Disease (MCTD): 3 cases; "Idiopathic" Raynaud's Disease (RD): 4 cases; Dermatomyositis (DM): 2 cases (1 paraneoplastic); SLE: 1 case; Unclassifiable Connective Tissue Disease (UCTD): 4 cases. The ANA-positive cases identified by the traditional technique were divided according to pattern into 4 categories: homogeneous, peripheral, speckled, nucleolar. In contrast those identified using HEp-2 cells were divided into 9 pattern groups: (nuclear type) centromere, fine speckled, coarse speckled, diffusely grainy, homogeneous: (nucleolar type) speckled, clumpy, homogeneous. The results demonstrated a higher general incidence of positivity with HEp-2 cells and confirmed the close connection between Anticentromere ANA and CREST syndrome. A similarly close connection was noted between MCTD and both nuclear diffusely grainy and nucleolar speckled patterns. A fairly clear connection was also noted between acrosclerotic or diffuse SSP and a fine speckled nuclear pattern. It is felt that ANA tests using IFI on HEp-2 cells should lead to significant progress in the field of diagnosis and prognosis and the study of PSS subsets.

Antibodies, Antinuclear↗
PubMedDate not supplied

[Anti-cardiolipin antibodies and other immunological disorders in patients with systemic scleroderma].

A total of 104 patients with scleroderma were examined. Anticardiolipin antibodies were detected in 37.5 per cent of the patients with systemic scleroderma and in 3 per cent of healthy individuals; they were more often detected in 46.8 per cent of the patients with diffuse affections of the skin, atherosclerosis, Raynaud's syndrome accompanied by ulcero-necrotic affections of the skin as compared to patients with restricted affections of the skin (sclerodactylia and focal scleroderma)--29.8 per cent. No significant changes in the frequency of detecting a rheumatic factor, antibodies to Scl-70 were revealed in subgroups of patients with scleroderma, positive and negative anticardiolipin antibodies. Of the greatest interest is a significant difference in levels of C-reactive protein which were high in half of the patients with anticardiolipin antibodies. Anticentromere antibodies were detected twice as more often in patients without anticardiolipin antibodies that corresponded to systemic sclerodermia with minimum involvement of the skin into the pathological process. It is suggested that ulcero-necrotic affection of the skin in systemic sclerodermia is associated with C-reactive protein but it is not of an immunocomplex nature.

Autoantibodies↗
PubMed1979

Decreased blood flow in a papaverine-induced passive vascular bed in sclerodermal skin.

Blood flow was measured in the form of 133Xenon washout rate constants in a cutaneous vascular bed made passive by intracutaneous injection of a 133Xenon-papaverine mixture. Experiments were performed using injection volumes of 0.005, 0.02, 0.1 and 0.2 ml in 7 normals and 15 patients suffering from generalized scleroderma. Blood flow was closely related to the volume of fluid injected, probably reflecting dilution of tissues and increased diffusion distances when injection volumes were increased. When using injection volumes of 0.005 and 0.02 ml, blood flow was significantly reduced in the patients, as compared with normals, probably because of a decreased capillary density in cutaneous tissue in generalized scleroderma.

Adult↗
PubMed1988

Scleromyxedema: a scleroderma-like disorder with systemic manifestations.

Scleromyxedema is a rare fibromucinous connective tissue disorder characterized by papular skin lesions associated with sclerosis and a serum monoclonal gammopathy. Little is known about either the natural history or the systemic manifestations of this disease. We reviewed the medical records of 19 patients with biopsy-proven scleromyxedema seen from 1950 to 1985 for evidence of systemic disease. There were 10 males and 9 females with a median age at diagnosis of 53 years. Monoclonal gammopathy was present in 13 patients. Eight patients complained of dysphagia; 3 had proximal esophageal dysfunction and 1 had total esophageal aperistalsis on barium swallow. Proximal muscle weakness was noted in 5, with an inflammatory myopathy in 3. Six patients complained of dyspnea on exertion. Of these, 5 had reduced diffusing capacity, 3 had reduced volumes, and 2 developed cor pulmonale. Pathologic changes characteristic of "scleroderma kidney" were demonstrated in 1 patient at postmortem. One patient had Raynaud's phenomenon and 2 had arthralgias/arthritis with noninflammatory synovial fluids. Although 8 of 12 patients treated with melphalan noted regression of their skin changes, no consistent improvement in the extracutaneous manifestations was demonstrated. Furthermore, 2 patients died of sepsis related to melphalan-induced myelosuppression, and 4 developed hematological malignancies following melphalan therapy. In conclusion, systemic manifestations in scleromyxedema are more prevalent than previously recognized, and can resemble those of scleroderma. Significant toxicity occurred with the use of alkylating agents in these patients, with treatment-related complications developing in 45% of patients treated with melphalan. The lack of definitive data regarding the natural history of this disease complicates the question of optimal therapy, but the use of alkylating agents should be reserved for those patients with severe debilitating skin disease.

Adult↗
PubMed2001

Interstitial lung diseases in collagen vascular diseases.

In this review, a clinical update is presented of the most important collagen vascular diseases (CVDs) and the different types of interstitial lung disease (ILD) encountered in these CVDs. These CVDs represent a heterogenous group of immunologically mediated inflammatory disorders with a large variety of affected organs besides the lungs. The frequency, clinical presentation, prognosis and response to therapy vary depending on the histological pattern (usual interstitial pneumonia, desquamative interstitial pneumonia, organising pneumonia, diffuse alveolar damage, nodular lesions, etc.), as well as on the underlying CVD (scleroderma, rheumatoid arthritis, systemic lupus erythematosus, dermatopolymyositis, Sjogren's syndrome or mixed connective tissue disease). The diagnosis of most of these CVDs is based on a number of criteria; in several of these, however, lung involvement is not part of the diagnostic criteria. In addition, there may be overlaps between several of these CVDs. Optimal treatment varies depending on the type of collagen vascular disease and the presence of interstitial lung disease, although in many cases, a combination of corticosteroids and cytostatic drugs are given.

Arthritis, Rheumatoid↗
PubMed1976

The spotted nephrogram of renal scleroderma.

The renal angiographic findings in our two patients with scleroderma and recent onset of hypertension included minor changes in the distal interlobar and arcuate arteries and a nephrogram displaying diffuse, spotty lucencies. Although the spotted nephrogram is occasionally seen in cases of severe nephrosclerosis, we believe that, in the absence of major arterial changes in the arcuate and distal interlobar arteries, it is virtually diagnostic of renal scleroderma.

Adult↗
PubMed1993

Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease.

Fourteen patients with systemic sclerosis (SSc, scleroderma) and interstitial lung disease were treated with oral cyclophosphamide (1-2 mg/kg/day) and low dose prednisone (< 10 mg/day). There was a significant improvement in FVC after 6 months compared to entry values (2.21 +/- 0.19 l vs. 2.03 +/- 0.15 l, p < 0.02). Improvement was maintained at 12 months (2.27 +/- 0.27 l, p < 0.05) and 18-24 months (2.60 +/- 0.28 l, p < 0.001). In 12 cases followed for 18-24 months, FVC was stable or improved. No significant improvement or decline was noted for the DLCO. Side effects included cytopenia (2), infection (1), and hemorrhagic cystitis (2), and one possible related malignancy. A controlled prospective trial of cyclophosphamide is warranted in patients with SSc and active interstitial lung disease.

Adult↗
PubMed1988

[Respiratory disorders in Sjögren's syndrome: its comparison with scleroderma and normal state].

The respiratory system was evaluated in 40 patients with primary Sjogren's syndrome (pSS) and its involvement was compared with that of 26 patients with secondary Sjogren's syndrome (sSS), 40 with rheumatoid arthritis (RA), 30 with scleroderma and 100 matched healthy controls using questionnaire, physical examination and functional criteria (spirometry, flow/volume curve, total lung capacity, diffusion). The commonest clinical manifestation in pSS patients was dry cough either alone (xerotrachea) or in combination with dyspnea (indicative of diffuse interstitial lung disease). Functional analysis revealed that 38% of pSS patients had impaired diffusion and 25% impaired flow/volume curve (indicative of small airways disease). Interstitial lung involvement was more common in extraglandular pSS (52% versus 21% in glandular). However no pSS patient was incapacitated from pulmonary disease and when pulmonary function was compared with that of scleroderma and healthy controls it was shown that the dysfunction in primary Sjogren's syndrome was much less serious than in scleroderma and not much different from that of healthy controls. The respiratory involvement in sSS differed since it was more suggestive of obstructive lung disease (19%) in a way very similar to that of rheumatoid arthritis. The results indicate that pulmonary involvement in pSS although frequent is not clinically important. They also suggest that sSS does not add pulmonary manifestations to RA. The differences in pulmonary involvement between pSS and sSS further support the thesis that they are different disease entities.

Humans↗
PubMed1991

[The total proteolytic and collagenolytic activity in the blood cells of patients with systemic scleroderma].

Collagenolytic (CA) and neutral caseinolytic activity (NCA) was studied in extracts from blood cells (neutrophils, mononuclear cells and platelets) of patients suffering from systemic scleroderma (SSD). 23 persons were examined. Of these, 15 had local skin lesions, 8 diffuse lesions. CA, both specific and per 10(6) cells was found to be dramatically decreased (2-4-fold) in all blood cells, whereas NCA was increased in neutrophils and mononuclear cells; in platelets, it remained within normal. The amount of protein in neutrophils and mononuclear cells was lowered 1.2 and 2-fold respectively. In diffuse skin lesions, the amount of protein in cells was lower than in local lesions. It should be noted that in SSD patients examined, the inflammatory process was unmarked. Therefore, the data on substantial changes in proteolytic activity cannot be related to the inflammatory process. A reverse correlation was established between CA in neutrophils and circulating immune complexes as was a reverse correlation between CA in mononuclear cells and blood antinuclear factor. The data presented indicate that proteinases are an important factor of the pathogenesis in SSD. Apparently, SSD is characterized not only by enhanced synthesis of collagen but also by a dramatic reduction of the activity of enzymes that specifically hydrolyze this group of proteins, which leads to a decrease of collagen catabolism and hence, to the development of fibrosis in tissues.

Adult↗
PubMed1993

Clinical aspects of systemic and localized sclerosis.

Although pulmonary disease is now the main cause of mortality in systemic sclerosis, there is a subset of patients with early rapidly progressive disease and a high risk of renal and cardiac failure and death. Recognition of risk factors (clinical and laboratory, including autoantibody profiles and tissue types) will help identify patients whose disease outcome may be improved with early and more aggressive treatment. The frequency of cancer has increased in scleroderma patients. Serious cardiac disease may be present in the absence of symptoms, whereas not all lung manifestations (reduced diffusion capacity) are necessarily progressive. More detailed descriptions of various manifestations and disease associations have been published. The development of noninvasive procedures will allow us to evaluate and follow up pulmonary and cardiac manifestations more objectively in such patients. The evolution of eosinophilic fasciitis into local scleroderma in children is of interest.

Adult↗
PubMed2004

Nailfold digital capillaroscopy in 447 patients with connective tissue disease and Raynaud's disease.

The presence of megacapillaries and a decreased capillary density are the hallmarks of the scleroderma capillary pattern, which can be detected by nailfold capillarmicroscopy. One hundred and eighty-six patients with Raynaud's phenomenon, 65 cases with undifferentiated connective tissue disease (UCTD), 47 patients with systemic lupus erythematosus (SLE), 26 patients with dermato/polymyositis, 14 with rheumatoid arthritis, seven cases with primary Sjögren's syndrome and 102 patients with systemic sclerosis (SSc) were investigated. Of the 16 patients with diffuse cutaneous SSc and the 86 limited cutaneous SSc cases, 14 (87.5%) and 53 (61.6%) showed the scleroderma capillary pattern, respectively. Nine of the 65 (13.8%) cases with UCTD and 24 of the 186 (12.9%) cases with Raynaud's phenomenon also exhibited the same pattern. Four of the 47 (8.5%) with SLE and seven of the 26 (26.9%) with dermato/polymyositis, and no patients with rheumatoid arthritis or Sjögren's syndrome, exhibited the scleroderma capillary pattern. The conclusion is that the scleroderma capillary pattern is often present in SSc and dermato/polymyositis. Furthermore, patients with Raynaud's phenomenon and UCTD may also occasionally exhibit this pattern. Therefore, capillarmicroscopy seems to be a useful tool for the early selection of those patients who are potential candidates for developing scleroderma spectrum disorders.

Adult↗
PubMedDate not supplied

Isolated right ventricular failure in scleroderma heart disease.

Scleroderma is reported to have numerous cardiac manifestations. Right ventricular failure (RVF) is a well-recognized cardiac complication of scleroderma and most often is related to underlying pulmonary hypertension (PH). Causes of PH include both interstitial lung disease and pulmonary artery vasculopathy. Direct involvement of the ventricle by myocardial fibrosis or coronary vasospasm could also cause a diffuse bilateral cardiomyopathy. We describe a case of predominant RVF in the absence of significant PH in a patient with longstanding scleroderma.

Adult↗
PubMed1997

Pulmonary function in patients with systemic sclerosis.

The lungs are frequently affected in systemic sclerosis (SSc), a generalized connective tissue disorder. We evaluated the prevalence of respiratory functional abnormalities and their correlation with symptoms and radiograph features in a group of 34 patients who fulfilled the American Rheumatism Association criteria for the diagnosis of systemic sclerosis. Patients were submitted to a specific respiratory questionnaire and to lung function tests. Measurements were performed according to the European Coal and Steel Community (ECSC) recommendations and results expressed as a SD score, an accurate method that, taking into account the dispersion of the parameters in the reference population, allows precise definition of pathological subjects. Of the patients examined, 38% reported dyspnoea at rest or on exertion. No other respiratory symptoms were reported. Fifty percent had a normal chest radiograph. This study documents the high prevalence of respiratory functional abnormalities in patients with SSc. A restrictive pattern was found in 41% and an isolated diffusion impairment in 18%. No significant relationship was found between the isolated impairment of transfer factor of the lungs for carbon monoxide (TL,CO) and the mean duration of the scleroderma: thus, it does not seem to represent an early sign of severe restrictive disease. No bronchial or bronchiolar obstructive patterns were observed: it can be stated that small airways dysfunction is not a characteristic manifestation of SSc as considered previously. A significant association was found between the group of subjects with chest radiographic abnormalities and that with a restrictive pattern or isolated TL,CO alteration (p = 0.018). Chest radiographic abnormalities were also found in 29% and dyspnoea in 35% of the patients with normal respiratory function. The mean duration of scleroderma was not significantly different between the groups with and without abnormalities on chest radiography, between the groups with and without a restrictive pattern or isolated diffusion impairment, and between the groups of patients with and without dyspnoea. In conclusion, an accurate evaluation of respiratory function is recommended in the assessment of patients with systemic sclerosis, since the functional involvement of the lung cannot be predicted on the basis of the chest radiograph and the respiratory symptoms.

Dyspnea↗
PubMed1992

Bronchiolitis obliterans organizing pneumonia and scleroderma.

Pulmonary involvement in scleroderma is characterized by interstitial fibrosis and pulmonary hypertension. Although bronchiolitis obliterans organizing pneumonia (BOOP) may be associated with a variety of connective tissue diseases and their treatment, there are only rare reports of bronchiolitis associated with scleroderma. We describe 2 patients with scleroderma and rapidly evolving pulmonary infiltrates, which upon biopsy showed histologic findings of BOOP. Each patient had severe restrictive lung disease and markedly diminished diffusion capacity. Treatment with high dose prednisone showed a good response in one patient. The rapid course of pulmonary findings in these patients differs from the usual course of pulmonary fibrosis in scleroderma. Although BOOP is a rare finding in scleroderma, our findings suggest that rapid pulmonary decompensation or atypical findings for pulmonary fibrosis should prompt consideration for an open lung biopsy. Finding a potentially steroid responsive disorder in an otherwise steroid unresponsive disorder has important clinical implications.

Adult↗
PubMed1978

Scleroderma (progressive systemic sclerosis): progress and course based on a personal series of 118 cases.

A follow-up study has been made of a personal series of 118 cases of patients with scleroderma (progressive systemic sclerosis) followed for periods up to 25 years. These were classified according to the early distribution of skin changes as Type 1 (skin changes in fingers only), 48 cases; Type 2 (skin changes beyond the fingers but mainly in extremities), 47 cases; and Type 3 (diffuse), 19 cases, and atypical, four cases. Eighty per cent of the total series have survived five years, and 68% have survived 10 years. Survival is related to the type of scleroderma, being much better for Types 1 and 2 (acrosclerotic) than for Type 3 (diffuse). Actual compared with expected survival rate was better for younger than for older patients and for females than for males. Although the disease type gives an indication of survival in general, the caution must be used in the individual cases, as the course of the disease is very variable even in patients of the same type, and is subject to remission and relapse.

Adolescent↗
PubMed1985

The effect of D-penicillamine on pulmonary findings in systemic sclerosis.

D-penicillamine has been used for the treatment of systemic sclerosis for 2 decades. Forty-four systemic sclerosis patients who received a mean dose of 636 mg of D-penicillamine for 2.3 years and 48 untreated patients, who had repeat pulmonary function tests performed after a mean of 3.5 and 4.8 years, respectively, were evaluated. There were no significant changes in the vital capacity or the forced expiratory volumes during this time in either group. However, the diffusing capacity for carbon monoxide improved from 76% of predicted to 87% of predicted in D-penicillamine-treated patients. Untreated patients changed slightly, from 73% of predicted to 76% of predicted. When multiple logistic regression analysis was used to account for factors which could have biased treatment comparisons, the improvement related to use of the D-penicillamine was significant (P less than 0.05). This improvement in the D-penicillamine-treated patients was associated with no further progression of dyspnea or fibrosis on chest radiograph, as well as reduced skin thickening. These data suggest that D-penicillamine may be useful in the treatment of systemic sclerosis that involves the lung; however, further studies are needed.

Colchicine↗
PubMed1994

Increase of pulmonary diffusing capacity in systemic sclerosis.

Pulmonary function tests and chest radiographs of 29 non-smoking systemic sclerosis (SSc) patients were analysed, featuring an apparently paradoxic finding of an increased diffusing lung capacity for carbon monoxide (DLCO). Twenty-one patients (72%) had abnormal pulmonary function, 11 of them had restrictive disease (38%), six (21%) had isolated DLCO increase, four (14%) had isolated DLCO reduction, while two patients had obstructive disease (7%). Chest X-ray revealed interstitial abnormalities consistent with pulmonary fibrosis in all four patients with isolated DLCO reduction, in one obstructive patient and in six restrictive patients. In patients with DLCO increased steroid treatment significantly reduced DLCO (P < 0.05) and membrane DLCO component (Dm) (P < 0.05). Hitherto unobserved finding of DLCO increase in SSc patients was associated with shorter duration of SSc (P < 0.05), normal lung mechanics and roentgenogram (P < 0.05) and absence of pulmonary symptoms (P < 0.05). The findings that in some SSc patients DLCO increases suggest that DLCO might prove to be an early and sensitive indicator of acute pulmonary involvement.

Adult↗