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Results for “Scleroderma, Diffuse”

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At least 1,009 records · Page 56Linked to original sources
PubMed1992

Isolated diffusing capacity reduction in systemic sclerosis.

OBJECTIVE: To determine the long-term outcome of patients with systemic sclerosis (SSc) and an isolated reduction in the diffusing capacity for carbon monoxide (DLCO) at the time of initial evaluation. METHODS: Patients with an isolated reduction in DLCO (i.e., normal forced vital capacity [FVC] and normal ratio of the forced expiratory volume in one second [FEV1] to the FVC) on initial evaluation were identified from among 815 patients with SSc who were carefully followed up throughout their illness. We requested that patients have repeat pulmonary function testing (PFT), and the outcomes of these tests, as well as cardiopulmonary and survival outcomes, were determined. RESULTS: An isolated reduction in DLCO, with a normal FVC was detected in 152 (19%) of the 815 patients. A subset of those with an isolated reduction in DLCO (11%) developed isolated pulmonary hypertension and had severely reduced survival rates. Pulmonary hypertension was strongly associated with an initial DLCO of less than 55% of predicted normal and a FVC (% predicted)/DLCO (% predicted) ratio of greater than 1.4. Among all patients in whom this ratio was greater than 1.4, 22% developed isolated pulmonary hypertension, compared with only 2% of those whose ratio was less than 1.4 (P less than 0.01). Of the 152 patients with isolated DLCO reduction, 73 (48%) underwent PFTs a mean of 5.4 years (range 2.0-13.2) after the initial PFT. Only 6 (8%) of these 73 patients ever had serious pulmonary disease: 5 had isolated pulmonary hypertension, and 1 had severe pulmonary fibrosis. Half of the patients with a low initial DLCO demonstrated a significant improvement (greater than 20%) at followup testing that could not be explained by the demographic, clinical, or laboratory findings at the first visit. CONCLUSION: Isolated reduction in DLCO is a frequent abnormality in SSc. Overall, it is associated with a good prognosis for survival and for pulmonary morbidity. A small subset of patients (11%) who have a very low DLCO (less than 55% of predicted) have developed isolated pulmonary hypertension, all of whom had limited scleroderma.

Carbon Monoxide↗
PubMedDate not supplied

Epidemiology of systemic sclerosis.

Systemic sclerosis is a family of disorders most appropriately considered in the category of the connective tissue diseases. Two major forms are recognized (diffuse cutaneous and limited cutaneous involvement subtypes), each with distinctive clinical and serologic findings as well as natural history. Scleroderma is characterized epidemiologically by several distinctive features. From a demographic viewpoint, the disease spares children and its incidence increases steadily with age among adults. If occurs much more frequently in women, especially during the child-bearing years, and most often and most severely in young black women, though there is no overall prominent racial predilection. The annual incidence approaches 20 per million population, and may be considerably underestimated. Both incidence and mortality have increased during the past several decades, but these changes are most likely a result of improved case detection rather than a true increase in incidence. Prevalence studies have not been undertaken, but 500 per million population may be a reasonable estimate. Family and genetic studies suggest a weak genetic predisposition, but several strong HLA associations with scleroderma-specific serum autoantibodies are evident. Certain environmental agents may be implicated in pathogenesis of scleroderma and closely related systemic illnesses associated with cutaneous fibrosis. The widespread pathologic process in systemic sclerosis leads to vascular insufficiency and fibrosis, which diminishes the reserve function of many organ systems. The result is considerable disability, especially affecting hand function, and a significant reduction in life span, with an overall 10-year survival from first physician diagnosis of under 70%. Further epidemiologic studies should take full advantage of established and newly proposed subsets of patients with homogeneous clinical, laboratory, serologic, and natural history features. The environment-host interactions noted here must be fully explored, especially in early untreated disease, where primary rather than secondary mechanisms are most likely to be operative.

Adolescent↗
PubMed2003

Misdiagnosis of silent sinus syndrome.

PURPOSE: To review cases of spontaneous enophthalmos erroneously diagnosed as silent sinus syndrome to identify other inflammatory disorders with a similar clinical presentation. METHODS: Retrospective observational case series. Chart reviews were performed covering encounters over a 5-year period, searching for patients referred to two physicians with an incorrect diagnosis of silent sinus syndrome. Only cases of acquired enophthalmos of reported nontraumatic or nonneoplastic cause were included. Particular attention was directed toward noting clinical features of upper eyelid position and periocular atrophy. RESULTS: Nineteen cases of erroneous referral for silent sinus syndrome were identified. Fourteen of these cases were due to tumor, trauma, congenital facial asymmetry, or diffuse facial lipodystrophy. Among the remaining cases, four were diagnosed as Parry-Romberg syndrome and one as linear scleroderma. CONCLUSIONS: Parry-Romberg syndrome and linear scleroderma must be distinguished from silent sinus syndrome as causes of inflammatory-mediated, spontaneous enophthalmos.

Adult↗
PubMed2004

Fibrosing alveolitis in systemic sclerosis: the need for early screening and treatment.

Abnormalities in lung function occur in 70% of patients with systemic sclerosis (SSc). Fibrosing alveolitis in SSc (FASSc) is more commonly seen in the diffuse cutaneous form of SSc, particularly in the presence of antitopoisomerase antibodies (Scl70), and with the decreasing incidence of scleroderma renal crisis it is now the major cause of mortality in this patient population. Screening of patients recently diagnosed with SSc by pulmonary function tests and the performance of high resolution computed tomography when physiological abnormalities are identified has resulted in the identification of significant numbers of patients with early, asymptomatic FASSc. Whether these patients should be further investigated with a surgical lung biopsy or receive immunosuppression is unclear, because it cannot yet be reliably predicted who will develop progressive disease and the evidence to support the efficacy of treatment is not strong. The objective of the present article was to review the evidence to support the use of immunosuppressive therapy in FASSc and, based on these data, to propose an algorithm for the investigation and management of this difficult clinical problem.

Bronchoalveolar Lavage Fluid↗
PubMed1978

Isolated trigeminal sensory neuropathy: early manifestation of mixed connective tissue disease.

A young woman with mixed connective tissue disease (MCTD) had an isolated trigeminal sensory neuropathy as an early manifestation of the disease. Raynaud phenomenon occurred almost synchronously with the onset of trigeminal neuropathy and was followed by myositis, diffuse hand swelling, synovitis, and increased ribonucleoprotein antibody. Mixed connective tissue disease has overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by high-titer antibody to ribonucleoprotein.

Adult↗
PubMedDate not supplied

[Eosinophilic fasciitis (Shulman's syndrome)].

The method of optic and electron microscopy was used to examine the skin, muscle and fascia in 8 patients with eosinophilic fasciitis aged from 20 to 57 years with the disease duration from 6 months to 3.5 years. When examining biopsy samples the authors paid special attention to the state of collagenic structure, cellular elements of the skin connective tissue, fascia and intermuscular layers, changes in the cross-striated muscle adjacent to the fascia, composition of cellular infiltrates as well as to the state of vascular microcirculation. It has been shown that with the given disease one can observe exudative-infiltrative processes with alteration of the vascular bed in the fascia and epimysium and the reaction of the microcirculatory bed of the skin and muscle with subsequent outcome into sclerosis at later periods of the disease. But the morphological data allow exclusion of the diffuse nature of microcirculation derangement in eosinophilic fasciitis. Morphogenesis of vascular lesions in this disease differs from such in scleroderma that suggests two different diseases.

Adult↗
PubMed1984

[1st results of functional RES scintigraphy with 99mTc-labeled human serum albumin millimicrospheres in progressive scleroderma: possibility of early diagnosis of lung involvement?].

Functional RES scintigraphy with 99mTc-labeled human serum albumin millimicrospheres (99mTc-HSA-MM) was conducted in 11 female patients suffering from progressive scleroderma. The RES scan revealed abnormalities in the bone marrow in eight patients as well as pathological changes of the liver in 2 cases. 7 patients showed diffusely enhanced concentrations of 99mTc-HSA-MM in the lung, whereas X-ray picture and pulmonary function test revealed pathological findings in only 4 patients, respectively. Humoral inflammatory and immunologic parameters, too, indicated abnormalities less frequently than the lung scan. Thus functional RES scintigraphy seems to be a very sensitive approach to the assessment and possibly to the early diagnosis of lung involvement in progressive scleroderma.

Adult↗
PubMed1977

Mixed connective tissue disease: the spectrum of radiographic manifestations.

Mixed connective tissue disease (MCTD) is a serologically distinct entity defined by a ribonuclease-sensitive extractable nuclear antigen. This unusual overlap syndrome has clinical features of scleroderma, systemic lupus erythematosus, polymyositis, and rheumatoid arthritis. In order to define the radiographic changes in MCTD, radiographs of the hands of 17 patients were studied, utilizing a fine-detail technique. Diffuse and periarticular osteopenia were found in 8 and 10 patients, respectively; soft-tissue swelling in 11; erosive changes in 9; joint-space narrowing in 7; tuft resorption and soft-tissue atrophy in 6; and subluxations in 2. In individual cases radiographs may appear normal or exhibit features of scleroderma, systemic lupus erythematosus or rheumatoid arthritis, thereby mirroring the clinical diversity of this entity.

Adolescent↗
PubMed1986

Hiatus hernia and esophageal contraction abnormalities.

The relationship of hiatus hernia to esophageal motility pattern was examined in patients referred for evaluation of esophageal symptoms. Results from standard esophageal motility studies were compared with findings on barium radiography of the upper gastrointestinal tract. Of 169 patients without radiographic evidence of esophagitis, 53 (31 percent) had normal motility of the esophageal body, whereas 116 (69 percent) demonstrated esophageal contraction abnormalities, a classification that includes the pattern of diffuse esophageal spasm at the severest extreme. Hiatus hernia was significantly more common in those with contraction abnormalities (25 percent) than in those with normal patterns (8 percent) (p = 0.01). Hiatus hernia increased in prevalence with increasing severity of contraction abnormalities, such that hiatus hernia was present in nearly half of patients with the pattern typifying diffuse esophageal spasm. Thus, hiatus hernia and esophageal contraction abnormalities are associated once other associations with hiatus hernia (e.g., esophagitis and scleroderma) have been excluded. These findings may help explain the recognized relationship of esophageal symptoms to hiatus hernia in patients without significant gastroesophageal reflux.

Esophageal Diseases↗
PubMed1986

Respiratory involvement in patients with Sjögren's syndrome: is it a problem?

We examined the pulmonary involvement in 40 patients with primary Sjögren's syndrome (1 degree SS), and 26 patients with secondary SS (2 degrees SS) and compared them with 100 healthy matched controls, 40 patients with rheumatoid arthritis (RA) without evidence of SS and 30 patients with scleroderma. The respiratory evaluation included questionnaire, physical examination, chest roentgenogram and pulmonary function studies (spirometry, flow/volume curve, total lung capacity and diffusion: DLCO). The most common symptom in 1 degree SS was dyspnea (25%). Xerotrachea was present only in 1 degree SS (12.5%) while pleurisy only in 2 degrees SS and RA (7.7% and 2.5% respectively). The most common functional defect in 1 degree SS was interstitial lung disease (30%) and in 2 degrees SS and RA airways obstruction (46% and 32.5% respectively). Finally, small airways disease was frequent in all patient groups, as well as the control group. Comparison between patients with glandular and extraglandular SS revealed that xerotrachea was a manifestation of glandular and diffuse interstitial lung disease of extraglandular SS. Finally, comparison of patient groups and controls revealed statistical significant differences in: FVC and DLCO between extraglandular 1 degree SS/controls, FEV1/FVC ratio between 2 degrees SS/controls and the same between RA/controls, FVC, TLC, DLCO between scleroderma/controls, DLCO between scleroderma/SS and separately between scleroderma/1 degree SS and scleroderma/extraglandular 1 degree SS (which were the subgroups of SS with lowest DLCO). Diffusion impairment correlated with the presence of cryoglobulins and low complement levels, suggesting immune complex deposition as a mechanism of the interstitial lung disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Dyspnea↗
PubMedDate not supplied

An uncommon cause of scleroderma.

The human immunodeficiency virus (HIV) pandemic is nearly 20 years old. HIV infection is characterized by profound immunodeficiency resulting in an increased incidence of opportunistic infections and neoplasms. However, the greatest paradox is the occurrence of certain autoimmune disorders in the setting of HIV. These include diffuse interstitial lymphocytosis syndrome (DILS), reactive arthritis, systemic lupus erythematosus (SLE), and rheumatoid arthritis (RA). It has also been seen that even in the absence of these well-defined diseases, various rheumatological manifestations such as arthralgias, arthritis, myopathy, vasculitis, and sicca syndrome are commonly associated with HIV. To the best of our knowledge, the association of HIV with scleroderma has not previously been reported.

Acquired Immunodeficiency Syndrome↗
PubMed1996

Scleroderma epidemiology.

The overall incidence and prevalence rates for scleroderma in the United States appear to be stable over the past 2 decades. Age-specific incidence rates are higher in black women than in white women, and diffuse disease is more common in the black population. Risk factors for disease development include female gender and may include HLA-DQ type. Diffuse disease, older age at onset, and early internal organ involvement are risk factors for reduced survival. Genetic factors appear to play a permissive role, whereas as yet undefined environmental factors play a more direct role in disease causation.

Ethnicity↗