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PubMed1997

Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography.

OBJECTIVE: Thin-section computed tomography (CT) provides a sensitive and reproducible method of quantifying the morphologic extent of disease in the clinical management of fibrosing alveolitis associated with systemic sclerosis (FASSc). The aim of this study was to determine which indices of lung function best reflect the extent of disease on CT in FASSc, and to determine the independent influences of smoking history, extent of fibrosing alveolitis, demographic features, and concurrent treatment upon functional impairment in FASSc. METHODS: Sixty-four patients with FASSc were studied using CT and static and exercise lung function testing. Statistical relationships were determined by multiple regression analyses. RESULTS: Five patients with overt pulmonary hypertension were characterized by severe impairment in 3 indices of lung function: diffusing capacity for carbon monoxide (DLCO), DLCO adjusted for alveolar volume (KCO), and arterial partial pressure of oxygen. On multiple regression analysis, the major determinant of functional impairment was the extent of fibrosing alveolitis on CT. A history of smoking was independently associated with preservation of total lung capacity and depression of KCO, but did not otherwise influence functional-morphologic correlations. The percent predicted DLCO correlated better with extent of disease on CT (r = -0.70) than did oxygen desaturation on exercise (r = 0.55), the physiologic component of the clinical-radiographic-physiologic score (CRP index) (r = 0.52), or other indices of lung function. Lung volume measures correlated poorly with disease extent on CT. CONCLUSION: The percent predicted DLCO best reflects the extent of fibrosing alveolitis in FASSc, and therefore should be measured in routine evaluations. Exercise testing may also have a useful role in staging the severity of pulmonary fibrosis, but the CRP index offers no additional advantage over the DLCO and exercise testing.

Carbon Monoxide↗
PubMed1997

Clinical setting of patients with systemic sclerosis by serum autoantibodies.

Associations of antinuclear (ANA) and anticardiolipin (aCL) antibodies with clinical manifestations were analyzed in patients with systemic sclerosis (SSc). We studied 105 SSc patients: 28 had limited cutaneous SSc (lcSSc) involving fingers; 36 had intermediate cutaneous SSc involving limbs and face; 33 had diffuse cutaneous SSc (dcSSc) involving the trunk; 8 had a sclerosis sine scleroderma. Clinical manifestations and instrumental and laboratory findings were considered to calculate a disease score. Serum anticentromere (ACA), anti-topoisomerase I (anti-topo I) antibodies, and aCL (of IgG/IgA/IgM classes) were investigated by conventional methods. ACA positive patients (n=18), compared to ACA negative, showed higher prevalence of IcSSc (p < 0.001), lower prevalence of restrictive ventilatory defect (p=0.006), and lower disease score (p=0.008). Anti-topo I positive patients (n= 70) showed lower prevalence of lcSSc (p =0.001) compared to anti-topo I negative. In aCL positive patients (n=27) widespread skin and visceral involvement occurred more frequently than in aCL negative. The association with myocardial ischemia or necrosis (p=0.010) was significant. Occurrence of ACA excluded the coexistence of anti-topo I (p < 0.001), and aCL (p=0.037). aCL positive patients showed higher disease score in comparison with ACA positive patients (p=0.003). In conclusion ACA recognize patients with a mild disease. aCL in contrast to ACA are better than anti-topo I in recognizing the most severe pictures of SSc.

Adult↗
PubMed1992

Palmoplantar keratoderma with sclerodactyly (Huriez syndrome).

A syndrome characterized by palmoplantar keratoderma, sclerodactyly, and skin cancer was first described in two families by Huriez et al. The pattern of inheritance was compatible with that of an autosomal dominant disorder. We report a patient with this condition and review the literature.

Fingers↗
PubMed1997

Functional impairment in lone cryptogenic fibrosing alveolitis and fibrosing alveolitis associated with systemic sclerosis: a comparison.

Lone cryptogenic fibrosing alveolitis (CFA) is histologically identical to fibrosing alveolitis associated with systemic sclerosis (FASSc), but it has a much worse prognosis after matching for disease severity at presentation. Thin-section CT scanning (CT) provides a reproducible method of quantifying the morphologic extent of fibrosing alveolitis. The aim of this study was to gain insights into contrasting pathophysiologic mechanisms in the two diseases by comparing patterns of functional impairment after matching for extent of disease on CT, demographic factors, smoking history, and concurrent treatment. Patients with emphysema on CT (n = 16) and patients with FASSc with overt pulmonary hypertension (n = 5) were excluded; 111 patients were studied (CFA, n = 54; FASSc, n = 57). Patients with CFA were distinguished by more severe functional impairment and more extensive disease on CT (40.1 versus 22.1%, p < 0.00005). On multivariate analysis, patients with CFA had greater reduction in arterial P(O2) (p < 0.0005), wider AaP(O2) (p < 0.0005), greater oxygen desaturation on maximal exercise (p < 0.03), and higher dyspnea scores (p < 0.02) than did patients with FASSc after controlling for extent of disease on CT and other covariates. Measures of lung volume and gas transfer did not differ independently between CFA and FASSc. These findings persisted in subanalyses of patients with limited disease, extensive disease, histologic confirmation of fibrosing alveolitis, and with the reinclusion of patients with emphysema and pulmonary hypertension. The patterns of functional impairment were indicative of more severe ventilation-perfusion mismatch or anatomic shunting in CFA after adjustment for disease extent; we speculate that perfusion of poorly ventilated lung parenchyma in CFA occurs through new vessels formed in areas of intense inflammation. This mechanism may contribute to the greater mortality of patients with CFA than of patients with FASSc because of the deleterious effects of hypoxia on concurrent cardiac disease.

Female↗
PubMedDate not supplied

Effects of long-term cyclic iloprost therapy in systemic sclerosis with Raynaud's phenomenon. A randomized, controlled study.

OBJECTIVE: Iloprost is a stable prostacyclin analogue which has been shown to be effective in the short-term symptomatic treatment of Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). The aim of this study was to evaluate the effects of long-term cyclic therapy with iloprost in comparison with nifedipine on the skin score, pulmonary function and Raynaud's severity score in patients with SSc and RP. METHODS: We conducted a 12-month prospective, randomised, parallel-group, blind-observer trial to compare the effects of intravenously infused iloprost (2 ng/kg/min on 5 consecutive days over a period of 8 hours/day and subsequently for 8 hours on one day every 6 weeks) with those of conventional vasodilating therapy with nifedipine (40 mg/day for os) in 46 patients with SSc and RP. RESULTS: At 12 months, iloprost but not nifedipine reduced the skin score (iloprost: from 13.26 +/- 2.05 to 9.26 +/- 1.32, p = 0.002; nifedipine: from 10.83 +/- 2.09 to 12.17 +/- 3.02, p = n.s.; iloprost vs nifedipine: p = 0.016) and the RP severity score (iloprost: from 2.17 +/- 0.2 to 1.22 +/- 0.13, p = 0.02 vs baseline; nifedipine: from 2.08 +/- 0.34 to 1.33 +/- 0.22, p = n.s.). Carbon monoxide diffusing capacity (DLCO), expressed as % of the predicted normal value, worsened significantly in the nifedipine group (from 69.6 +/- 7.4% to 61.5 +/- 6.5%, p = 0.044) and remained stable in patients treated with iloprost (from 53.2 +/- 4.8 to 56.0 +/- 4.6%, iloprost vs nifedipine: p = 0.026). CONCLUSION: In SSc patients, cyclic intravenous iloprost infusion is able to control vasospastic disease. Our results suggest that it might also act as a disease-modifying agent, as it seems to improve the course of the disease. Further studies principally focused on organ involvement and the natural history of the disease are needed to confirm our results.

Adult↗
PubMed2002

[Fibrotic diseases].

Fibrosis is a pathologic process, which includes scar formation and over production of extracellular matrix, by the connective tissue, as a response to tissue damage. The molecular process is not different from normal formation of connective tissue and extracellular matrix in the normal organs. The context, the environment and the over production make the difference. Fibrosis formation includes interaction between many cell types and cytokines, and when the balance becomes profibrotic, there is fibrosis formation. Major profibrotic agents are type 2 CD4 positive lymphocytes, CD40 receptor and ligand interaction, and the following cytokines: IL-4, transforming growth factor b, platelet derived growth factor. The major antifibrotic agent is interferon gamma. Pathologies include: in the skin pathologic scarring as colloid and hypertrophic scar, cirrhosis of liver and gallbladder, in the heart and the kidneys, pulmonary and bone-marrow fibrosis, and scleroderma. Scleroderma is chronic connective tissue disease, expressed clinically by systemic sclerosis and diffuses fibrosis of the skin and viscera. This is a progressive degenerative disorder of the blood vessels, skin, lungs, kidneys, heart and GI tract and for this reason this disease plays a major role in fibrosis research. Fibrosis is considered an irreversible process, at least clinically, and is usually treated by anti-inflammatory and immunosuppressive agents. This kind of therapy was not proven successful and sometimes it harms more than cures. Many patients suffer from fibrotic diseases and the aim is to develop anti-fibrotic agents, targeted to the pathologic molecular process. Progressing step by step in this issue has direct clinic affect.

Cytokines↗
PubMed2006

[Pulmonary arterial hypertension in connective tissue disease].

INTRODUCTION: Pulmonary arterial hypertension (PAH) is one of the most severe complications of connective tissue diseases. PAH is defined by mean arterial pulmonary pressure > 25 mmHg at rest (or 30 mmHg during exercise) during right heart catheterisation. STATE OF THE ART: About 10% of cases of PAH are related to connective tissue diseases. PAH may be observed in any of the connective tissue diseases; it is mostly encountered in systemic sclerosis (prevalence estimated to 8%), and is more frequent in the limited form of the disease with anticentromeres antibodies, in the presence of Raynaud's phenomenon, of antinuclear antibodies, decreased CO diffusion capacity, and during the perimenopausal period. Histopathological lesions are similar to that of idiopathic PAH (with plexogenic lesions being more rare); mutations have not been described. Venous thromboembolic disease (especially in lupus) and hypoxemia related to pulmonary fibrosis should be investigated. Screening for PAH is recommended in systemic sclerosis (by echocardiography every year and in case of dyspnea). Few clinical trials have been conducted specifically in PAH related to connective tissue diseases, therefore the treatment approach is similar to that of idiopathic PAH, with a poorer prognosis, including treatment with bosentan, epoprostenol and other prostacyclin analogs, and sildenafil. Immunosuppressive therapy may occasionally improve PAH in patients with systemic erythematosus lupus or mixed connective tissue disease. PERSPECTIVES: Respective indications of drugs and treatment associations need to be precised. CONCLUSION: The availability of efficacious treatments of PAH justifies its screening and early diagnosis in connective tissue diseases.

Antihypertensive Agents↗
PubMed1981

Eosinophilic fasciitis: report of 15 cases.

Clinical and histopathologic features of 15 cases of diffuse fasciitis with eosinophilia are reviewed. The patients experienced an inflammatory scleroderma-like illness without Raynaud's phenomenon or internal organ involvement. Polyarthritis and carpal tunnel syndrome were observed. The clinical courses demonstrated the potential for spontaneous remission, relapse, and late recurrence. Histopathologic features are discussed, as is their relationship to other forms of localized scleroderma. None of the 12 patients treated with corticosteroids has had complete resolution of the skin changes after a median follow-up of 10 months. Unusual associated diseases--acute myelomonocytic leukemia and an evolving myeloproliferative disorder--were observed in two patients.

Adult↗
PubMed1991

Epidemiological study of patients with systemic sclerosis in Tokyo.

We conducted an epidemiological study of systemic sclerosis in the city of Tokyo using the records of patients who had been registered to receive free medical service for intractable diseases. A total of 636 patients were registered as having systemic sclerosis in 1987, and we sent questionnaires to the doctor of each patient. The contents of the questionnaires included the patient's name, sex, age, occupation, major symptoms, therapy and laboratory findings. We received 357 completed replies, and were able to analyse them. Our study estimated that at 1 January 1988 the prevalence rate in Japan was between 2.1 and 5.3 per 100,000. The male/female ratio was 14:1. The ages of the patients when surveyed ranged from 17 to 82 years, with a mean age of 51 years, peaking with the most numerous group being 50-59 years. The characteristic signs of systemic sclerosis were as follows: proximal scleroderma, 75%; sclerodactyly, 91%; pitting scars, 49%; short sublingual frenulum, 49%; pulmonary fibrosis, 45%; diffuse pigmentation, 45%; and phalangeal contracture, 35%. Raynaud's phenomenon was present in 93% of patients, and was the initial symptom in 59% of cases. With respect to specific antinuclear antibodies, anticentromere antibody was present in 19% and antitopoisomerase I antibody was present in 27%.

Adolescent↗
PubMedDate not supplied

[Pulmonary arterial hypertension and generalized scleroderma. A propos of 10 cases].

Whereas pulmonary fibrosis is a very frequent manifestation of scleroderma, pulmonary hypertension of the precapillary type is a much rarer occurrence in this affection. Two cases of scleroderma with subsequent development of a pulmonary hypertension are reported. Various hypotheses have been advanced to explain the origin of this disorder during the course of scleroderma. Rather than a direct consequence of the sclerodermic pulmonary fibrosis it probably arises secondary to the specific, diffuse, primary pulmonary vascular lesions.

Aged↗
PubMed1986

Role of inflammation in the lung disease of systemic sclerosis: comparison with idiopathic pulmonary fibrosis.

Alveolar inflammation is thought to underlie the development of pulmonary fibrosis in several forms of diffuse lung disease including the connective tissue diseases. The relationship between inflammation and the clinical manifestations of systemic sclerosis (scleroderma), such as skin and lung involvement, is less clear. We therefore evaluated 14 never-smoking patients with systemic sclerosis with pulmonary involvement by bronchoalveolar lavage (BAL) and compared the results with those found in eight nonsmoking patients with idiopathic pulmonary fibrosis (IPF) and eight normal subjects. The patients with scleroderma also underwent gallium citrate Ga 67 scanning. We found that patients with scleroderma and pulmonary involvement have alveolitis that appears to wane with time. In addition, patients with systemic sclerosis have a cellular profile in lavage fluid that appears to differ from that of patients with IPF. Finally, we found a significant correlation between BAL cellular recovery and the single-breath carbon monoxide diffusing capacity in patients with systemic sclerosis but not in patients with IPF. We conclude that inflammation may play an important role in the pathogenesis of the pulmonary disease of scleroderma and that different mechanisms may lead to fibrosis in IPF and scleroderma.

Adult↗
PubMed1992

Systemic sclerosis.

Systemic Sclerosis is a multisystemic disease characterized by sclerosis of the skin and visceral organs, vasculopathy (Raynaud's phenomenon) and autoantibodies. The criteria for the classification of the disease requires either proximal scleroderma (major criteria) or the presence of 2 of the 3 minor features namely sclerodactyly, digital pitting scars and bibasilar pulmonary fibrosis. There are 3 subsets of this condition--diffuse variant, limited variant (CREST syndrome) and Overlap Syndrome (where patients have features of other rheumatic diseases). There are localized forms of scleroderma and pseudoscleroderma states. The presenting features of Systemic Sclerosis are usually Raynaud's, skin changes and arthralgia. Systemic complaints like breathlessness, dyspepsia, etc depending on the organ involved may be present. Management starts with patient education regarding the disease, skin care, exercises and regular medical check-up. There is no miracle cure but much can be done to improve the quality of life of the patient. Nifedepine and other drugs may improve Raynaud's phenomenon. Drugs can be used to treat other complications. Various medication have been tested as disease modifying drugs for scleroderma. These include drugs which inhibit collagen like D-penicillamine, colchicine, and immunosupressive drugs like cyclosporin. Ketotifen, a mast cell stabilizer has been reported to be effective in scleroderma. As it is a relatively safe drug, clinical trials are underway.

Age Factors↗
PubMed1991

Current options for the treatment of systemic scleroderma.

The epidemiology, pathology, diagnosis, and clinical manifestations of systemic scleroderma (SSc) are described, and therapeutic options are discussed. SSc is a rare condition of unknown etiology that occurs in a subset of scleroderma patients. It is distinguished by involvement of the small arteries, microvessels, and diffuse connective tissue. The degree of internal organ involvement is the main determinant of morbidity and mortality. Management of SSc may entail supportive, palliative, remittive, and immunosuppressive therapies. Supportive therapy involves maintaining the affected extremities at warm temperatures and the use of emollient creams. Results of palliative treatment are mixed. Toxic reactions may be associated with many of these medications. Dermatologic manifestations have been treated with nonsteroidal anti-inflammatory agents, low-dose corticosteroids, dimethyl sulfoxide, and edetate disodium; peripheral and internal-organ vascular obstruction, with alpha-adrenergic blockers, angiotensin-converting-enzyme inhibitors, and calcium-channel blockers. Antacids with alginic acid, histamine H2-receptor antagonists, sucralfate, and cholinergic-acting agents may be used to relieve GI symptoms. Lung infections should be treated promptly with antibiotics. No drug therapy has been successful in reducing the incidence of fatal cardiac arrhythmias or in preventing cardiac fibrosis. Captopril and enalapril are essential in the control of SSc renal crisis. Penicillamine may hold promise as a remittive therapy. The immunosuppressive agents fluorouracil, cyclosporine, and methotrexate, which have shown limited effectiveness in preliminary studies, merit further investigation. No therapeutic agent has yet been shown to alter the course of SSc on a consistent or long-term basis. Toxicity and drug interactions remain a major concern in patient management, and aggressive monitoring is essential.

Adult↗
PubMed1989

Retrospective studies in scleroderma: pulmonary findings and effect of potassium p-aminobenzoate on vital capacity.

The principal clinical pulmonary findings were extracted from University of Michigan Hospital records of 390 patients with scleroderma. Dyspnea was the most frequent symptom and strongly correlated with pulmonary fibrosis and with decreased vital capacity (FVC) and CO diffusing capacity (DLCO). The mean value for FVC was 84% of the predicted normal for 326 patients, and that of the initial DLCO 56.8% of the predicted normal (323 patients). Pulmonary fibrosis was diagnosed on first chest X-ray in 80 of 382 patients. An additional 48 patients developed fibrosis detected on subsequent X-rays. Analyses were performed to determine whether the deterioration of pulmonary function over time was less for scleroderma patients who were adequately treated with potassium p-aminobenzoate (KPAB) than for those inadequately or never treated with KPAB, The average decrease for both FVC and DLCO was found to be less for KPAB-treated patients. However, only in the case of vital capacity was the difference significant. In the presence of radiological evidence of pulmonary fibrosis FVC decreased more rapidly (p = 0.002), but the decline in DLCO was not affected. When adjusting for the presence or absence of fibrosis the average slopes of the logarithm of vital capacity were significantly less negative (p = 0.003) for patients on KPAB.

4-Aminobenzoic Acid↗