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PubMed1980

[Scleroderma and Rendu-Osler disease. A casual association? (author's transl)].

An uncommon association of scleroderma and Rendu-Osler disease in the same patient is reported. The authors have not found similar descriptions after an extense bibliographic review. A possible relationship is suggested on the basis of the etiopathogenesis of both disorders; Rendu-Osler syndrome is an hereditary disease of unknown etiology with mesoblastic involvement (embryonic tissue which gives origin to the connective tissue), and scleroderma is an immune disease characterized by diffuse sclerosis of the connective tissue. Rendu-Osler disease was diagnosed because of the presence of angiomatous telangiectasias since childhood, recurrent mucosal hemorrhages, and the possible existence of the syndrome in a familial member. The diagnosis of scleroderma was supported by the demonstration of: 1) cutaneous infiltration, 2) Raynaud's phenomenon, 3) functional esophageal alterations with clinical dysphagia, 4) resorption of the distal phalanges, and 5) conclusive skin biopsy.

Aged↗
PubMedDate not supplied

Eosinophilic fasciitis: a distinct clinical entity?

Eosinophilic Fasciitis is a syndrome characterized by exertion related scleroderma-like skin changes, peripheral eosinophilia, hypergammaglobulinemia and diffuse faciitis. Controversy exists as to the precise classification of the syndrome, i.e., whether it is a distinct entity or a variant of scleroderma. We describe a patient with eosinophilic faciitis but with several unique features: 1) progressive skin changes unresponsive to corticosteroid therapy; 2) elevated anti-DNA antibodies; 3) hypocomplementemia; and 4) a followup biopsy showing sclerodermatoid skin changes. These features and others relating to the controversial aspects of classification of eosinophilic fasciitis are discussed.

Diagnosis, Differential↗
PubMed1996

Multilobated B cell lymphoma (Pinkus variant) with isolated muscular localization in a patient with systemic sclerosis: a case report.

The association of systemic sclerosis (SSc) and non-Hodgkin's lymphoma (NHL) is a rare event whose pathogenesis remains to be clarified. We describe a case of NHL in a patient with SSc, in whom the NHL was particular in terms of clinical presentation (primitive muscle and bone involvement) and histologic type (large multilobated B-cell variant). The lymphoma was clinically aggressive but responded well to therapy. Surveillance for the development of "atypical" NHL is suggested in SSc cases.

Aged↗
PubMed1996

Electrophysiologic abnormalities of cardiac function in progressive systemic sclerosis.

The heart has been generally recognized as a target organ in progressive systemic sclerosis. Noninvasive studies have assessed the incidence and prognostic importance of cardiac arrhythmias in these patients. However, detailed exploration of the function of impulse formation and the conduction system of the heart in these patients has never been reported. Therefore, invasive electrophysiologic studies were performed in 30 patients with systemic sclerosis, all of whom had neither obvious cardiac involvement nor cardiac arrhythmias, and in 32 subjects with no evidence of heart disease, who served as a control group. Corrected sinus node recovery time in patients with systemic sclerosis was significantly longer (P < .001) than in the control group, as was the HV interval (P < .05). Of the 30 patients with systemic sclerosis, 10 had an HV interval of 60 ms or longer. In four patients with systemic sclerosis, the recorded AH interval exceeded 125 ms. The intra-atrial conduction time tended to increase to a significant degree (P < .05) in patients with systemic sclerosis. The interatrial conduction time was much longer (P < .001), and the maximal conduction delay to the atrioventricular junction and to the distal coronary sinus was much greater in the patients with systemic sclerosis than in the control group (P < .001 for both). Supraventricular tachyarrhythmias were induced in 15 patients with systemic sclerosis versus 3 control group subjects (P < .001). With respect to corrected sinus node recovery time, AH and HV intervals, atrial vulnerability, and ventricular tachycardia, 3 of the 30 patients with systemic sclerosis had abnormal findings in one of these parameters and 14 had abnormalities in more than one. These results suggest that a broad spectrum of electrophysiologic abnormalities is present in patients with systemic sclerosis, which can be revealed only by invasive studies. Furthermore, this study provides additional support for the hypothesis that diffuse myocardial involvement is characteristic of scleroderma patients, since a number of these patients showed more than one electrophysiologic defect.

Adolescent↗
PubMed2001

Evaluating the cutaneous involvement in scleroderma: torsional stiffness revisited.

OBJECTIVE: The pace, progression and extent of the skin lesions in scleroderma may parallel the risk of new internal organ involvement and the progression of existing internal lesions. Accurate assessment of cutaneous change permits an evaluation of patient prognosis and the response to therapy. The aim of this study was to assess a simple device for measuring skin stiffness for its ability to measure sclerodermatous skin in a quantitative and reproducible manner. Materials and methods. Torsional skin stiffness was measured in 56 normal subjects and 42 scleroderma patients (31 of whom had the limited form and nine the diffuse form, and two had mixed connective tissue disease). Data for the scleroderma patients were compared with data obtained by the use of the modified Rodnan clinical skin scoring technique. Intraclass correlation coefficients (ICCs) were calculated as a measure of intraobserver and interobserver variability. RESULTS: For the left and right hands respectively, the ICCs for intraobserver variability were 0.908 and 0.906 and those for interobserver variability were 0.871 and 0.628. There was a significant difference in mean angular rotation obtained by normal subjects compared with scleroderma patients (15.1 vs 11.3 degrees, P<0.001). There was a significant difference in the angular rotation with increasing severity of skin involvement (skin score 0, median rotation 16.3 degrees; score 1, 10.5 degrees; score 2, 8.5 degrees; score 3, 8.0 degrees; P<0.00001). CONCLUSIONS: The measurements obtained with the skin stiffness device are highly reproducible and are consistent with the current clinical method of assessment of skin involvement. The significant difference in angular rotation obtained by normal subjects and scleroderma patients indicates that the device can distinguish normal from sclerodermatous skin. The torsional stiffness measurements derived from the device may also be useful in longitudinal studies.

Adult↗
PubMed2001

Classification of oesophageal motility abnormalities.

Manometric examination of the oesophagus frequently reveals abnormalities whose cause is unknown and whose physiological importance is not clear. A large body of literature dealing with oesophageal motility abnormalities has evolved over the past few decades but comparisons among studies have been compromised by the lack of a widely accepted system for classifying the abnormal motility patterns, and by the lack of uniform diagnostic criteria for the putative disorders. Based on an extensive review and analysis of the literature, this report suggests an operational scheme to be used for the general classification of oesophageal motility abnormalities, and proposes standardised manometric criteria for the putative oesophageal motility disorders. By applying the guidelines proposed in this report, clinicians and researchers can determine if their patients fulfil the manometric criteria for a putative motility disorder. This should facilitate and improve comparisons among patients and studies. However, it is important to emphasise that fulfilment of the proposed criteria does not establish the clinical importance of the motility abnormalities.

Adult↗
PubMed1991

The eosinophilia-myalgia syndrome and related disorders.

The recognition of the eosinophilia-myalgia syndrome associated with L-tryptophan in the United States during 1989 as a disorder resembling the previously described 1981 toxic oil syndrome of Spain has established an increased level of consciousness regarding drug and toxin associated diseases. Both of these disorders were characterized by the development of acute and chronic multisystem features that parallel many idiopathic connective tissue diseases. Common manifestations have included generalized myalgias, fever, transient pulmonary infiltrates, and xerostomia during the early months followed by late stage neuromuscular and cutaneous disease. The most conspicuous laboratory abnormality was a peripheral eosinophilia. One of the most striking clinical findings has been scleroderma-like skin disease manifesting as diffuse fasciitis or hidebound induration. A sensory neuropathy and proximal myopathy in association with skin thickening have established these syndromes as chronic disabling diseases for many of their victims. Mononuclear perimysial and epineurial infiltrates have been distinctive pathological findings. Although the etiology of the eosinophilia-myalgia syndrome and the toxic oil syndrome are unknown, there is epidemiologic evidence to support the presence of contaminants in L-tryptophan and rapeseed oil, respectively, as the causative agents. No therapy has been demonstrated to arrest the evolution of the chronic sequelae in either disorder.

Eosinophilia↗
PubMed1982

Clinical aspects of autonomic nerve dysfunction of the gut.

Autonomic dysfunction leads to a variety of clinical disorders involving all parts of the gut. These neural disorders are distinct from the four other recognised categories of disorders involving myogenic function, myoelectric activity, hormonal regulation and abnormal humoral factors. Criteria for establishing that a disorder has a neurogenic aetiology vary in different diseases. Absence of a neural mediated response with intact muscle function has been the major criterion used in most studies. Neural mediated responses of peristalsis, sphincteric relaxation and intestinal contraction following distension or feeding are the major parameters of assessment. Abnormalities in neural function have been demonstrated in achalasia, symptomatic diffuse oesophageal spasm, diabetes mellitus, amyloidosis, scleroderma and chronic idiopathic intestinal pseudoobstruction. The anatomical site and type of gut neurological disorder varies in each condition. Morphological studies have been helpful in demonstrating specific intranuclear inclusion bodies in some pseudoobstruction patients, and vagal and ganglionic lesions in achalasia. Intact muscle ad myoelectric function as well as normal responsiveness to drugs acting directly upon muscle may be established by morphological study. Advancement in basic technology should provide a rewarding area for future study of the pathogenesis and treatment of the gut neurological disorders.

Amyloidosis↗
PubMed2003

[Scleroderma-like skin changes following administration of paclitaxel for the treatment of ovarian carcinoma].

A 61-year-old women with ovarian carcinoma was treated with surgery followed by 6 cycles of paclitaxel (Taxol) and carboplatin, producing a complete remission. During the course of therapy, she developed diffuse cutaneous sclerosis and thickening along with edema, weight gain and malaise. While antinuclear antibodies were elevated, specific antibodies against Scl 70 were not found. Other routine laboratory investigations were normal or negative. 20 MHz sonography showed a thickened, echo-rich dermis, while the Cutometer documented decreased cutaneous elasticity. Esophageal manometry identified abnormal motility. Additional screening and imaging studies showed no further evidence of systemic involvement. A skin biopsy showed dermal sclerosis. Treatment with immunosuppressive agents - prednisolone, azathioprine, cyclophosphamide- and phototherapy, including both UVA-1 and PUVA combined with penicillin, produced no definite improvement. One year after the administration of paclitaxel, the sclerosis and malaise are only minimal improved. This case is the second documented association of scleroderma-like cutaneous changes with paclitaxel therapy. Similar cases have been more often described following treatment with the analog substance docetaxel (Taxotere).

Diagnosis, Differential↗
PubMed1995

Correlation of manometric and radiographic diagnosis in esophageal motility disorders.

BACKGROUND: Radiography and manometry are complementary investigations in the diagnosis of esophageal motility disorders. In most centers, however, manometry is not available and diagnosis is based on radiography alone. AIMS: To correlate the findings on radiography in patients with esophageal motility disorders in whom a manometric diagnosis was available. METHODS: Retrospective analysis of esophageal manometry and barium contrast studies of 138 patients, done for suspected motility disorders, over a period of two years. RESULTS: Manometry was abnormal in 111 (80.4%) cases [achalasia 87, scleroderma 6, non specific esophageal motility disorders (NEMD) 13, diffuse esophageal spasm (DES) 5]. Radiology was abnormal in 106 cases; the overall radiographic sensitivity was 73.9% (achalasia 87.4%, scleroderma 83.3%, NEMD 38.5%) and the positive predictive value was 75.5% (achalasia 95%, scleroderma 100%, NEMD 29.4%). CONCLUSION: Radiography has a high sensitivity and positive predictive value in esophageal motility disorders like achalasia and scleroderma but in DES and NEMD the two investigative modalities (radiography and manometry) do not correlate well.

Adult↗
PubMed1979

Pemphigus vulgaris and lymphoma in a patient with scleroderma.

The coexistence of scleroderma and malignancy is well documented. The case presented here is unique, however, as it is the first report of the occurrence of diffuse histiocytic lymphoma and pemphigus vulgaris in a patient with scleroderma. Because of this unusual triad of diseases, the possibility of a common pathogenetic basis was examined. A review of the literature confirms that each disorder does have immunologic abnormalities. It is suggested that an aberrant immune response, specifically impaired T-cell function, is responsible for the emergence of disease of this patient.

Female↗
PubMed2006

Systemic and localized scleroderma in children: current and future treatment options.

Scleroderma is a group of rare and complex diseases with varied clinical manifestations. The most obvious manifestation of the diseases is skin hardening and sclerosis. Scleroderma can be divided into two main subgroups: systemic and localized. The systemic form, also known as systemic sclerosis, involves diffuse skin involvement and potentially severe visceral involvement. Localized scleroderma on the other hand is more common in children and usually confined to a specific region of the body with no internal organ involvement. The juvenile forms of systemic sclerosis and localized scleroderma are important conditions in children because of the clinical severity and substantial mortality of systemic scleroderma and the major growth defects associated with childhood-onset localized disease even if the active disease itself is self-limited. The pathogenic pathways of the various forms of scleroderma are only partially defined, but the main defect in scleroderma is abnormal collagen deposition leading to eventual fibrosis in the skin as well as multiple organ systems such as the heart and lungs in juvenile systemic sclerosis. Therapeutics are divided into three main subgroups for systemic sclerosis: antifibrotics, anti-inflammatories, and vasodilators. For localized disease, anti-inflammatories, vitamin D analogs, and UV irradiation have been investigated. However, the infrequency of scleroderma in the pediatric population plus the fact that this disease is very often self-limiting makes randomized controlled trials very difficult. It is for this reason that most data on treatment modalities for this disease have been extrapolated from studies in adult patients. There is no one therapy for systemic sclerosis or localized scleroderma that has proven to be very effective or significantly disease modifying. However, current therapeutic strategies must be initiated early in the disease course for maximum beneficial clinical effects. New interventions such as autologous stem cell transplant and cytokine-directed therapies are under investigation as potential treatments for this complex disease.

Child↗
PubMedDate not supplied

Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases.

OBJECTIVE: Patients with systemic rheumatic disease constitute a small percentage of admissions to the medical intensive care units (ICUs). Systemic sclerosis (SSc) is one of the rheumatic diseases that together with secondary complications may lead to a critical illness requiring hospitalization in the ICU. We present the features, clinical course and outcome of critically ill patients with scleroderma that were admitted to the ICU. METHODS: The medical records of nine patients with diagnosis of scleroderma (8 female, 1 male), admitted to the intensive care unit of Sheba Medical Center during the 11-year interval between 1991 and 2002, were reviewed. RESULTS: The mean age of the patients at the time of admission to the ICU was 48 +/- 13 [SD] years. The mean duration of SSc from diagnosis to the ICU admission was 8 +/- 8 years. Six patients had diffuse SSc, two patients had limited SSc and one patient had juvenile diffuse morphea. The main reasons for admission to the ICU were: infection/ septic syndrome (n = 4), scleroderma renal crisis (SRC) with pulmonary congestion (n = 2), acute renal failure associated with diffuse alveolar hemorrhage namely scleroderma- pulmonary - renal syndrome (SPRS) (n = 1), iatrogenic pericardial tamponade (n = 1), mesenteric ischemia (n = 1). The patients had high severity illness score (mean APACHE II 25 +/- 3). Eight out of nine patients (89%) that were admitted to the ICU died during the hospitalization, six (66.6%) of them died in the ICU. Septic complications as the main cause of death were determined in five patients (62.5%), while four of them had pneumonia and acute respiratory failure along with underlying severe pulmonary fibrosis. Lungs and kidneys were the most common severely affected organs by SSc in our patients. CONCLUSION: The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. Although infections are treatable, the high mortality rate for this group of patients was dependent on the severity of the underlying visceral organ involvement, particularly severe pulmonary fibrosis. The severity of this involvement is a poor outcome predictor. An early diagnosis and an appropriate treatment of such complications may help to reduce the mortality in scleroderma patients.

Adult↗
PubMed1989

The scleroderma neck sign.

The scleroderma neck sign, as described by Barnett, is a visible and palpable tight band over platysma in the hyperextended neck. A recent survey of 76 patients with scleroderma revealed that more than 90% had the scleroderma neck sign. Our study was performed using 15 patients with scleroderma and 30 controls including 3 with primary Raynaud's disease to examine the specificity of the scleroderma neck sign, and to look for a correlation between the presence of the scleroderma neck sign and histological changes of scleroderma in the skin overlying platysma. The scleroderma neck sign was present in 12 of the 15 patients with scleroderma but in none of the 30 controls. It was found both in patients with diffuse (5 out of 5) and limited (7 out of 10) scleroderma. In 10 of the 12 cases where the scleroderma neck sign was positive, there were characteristic histological changes of scleroderma on biopsy of the skin overlying platysma, in 1 there were nondiagnostic abnormalities, and in 1 the biopsy was unsatisfactory. The 3 patients with scleroderma in whom the scleroderma neck sign was absent had either nondiagnostic changes (1) or normal biopsies (2). The 3 patients with Raynaud's disease had normal skin biopsies. The scleroderma neck sign appears to be produced by scleroderma changes in the skin of the neck. In limited or early scleroderma where these changes are otherwise clinically inapparent, the scleroderma neck sign may be diagnostically useful.

Adult↗
PubMed1989

[Respiratory-hemodynamic features in patients with systemic scleroderma].

The authors provide the results of the clinical and functional studies in 25 patients with systemic scleroderma. The patients were examined for the diffusion lung capacity separated into the constituent components (membrane and blood ones), for the uniformity of alveolar ventilation, for pulmonary artery pressure and pulmonary blood flow. They also underwent echocardiography. The use of the combination of non-invasive research methods makes it possible to obtain quantitative and quantitative information pertaining to the involvement of the lungs at the level of the alveolocapillary membranes and microvascular bed, myocardium and to their interrelations at the early stages of systematic scleroderma. In patients with the pronounced clinical picture of the disease, of paramount importance is the use of these methods for proper interpretation of certain symptoms in the estimation of lung and heart injuries and of their prevailing significance in the general clinical picture of the disease. The new trends in the present work are related to the demonstration of the compensatory and pathological changes in the respiratory and hemodynamic system in sclerodermia systematica at the early enough stages of the illness.

Adult↗
PubMed1995

Autoantibodies to topoisomerase I in a patient with systemic lupus erythematosus without features of scleroderma.

We report a woman with systemic lupus erythematosus (SLE) with diffuse proliferative glomerulonephritis and anti-dsDNA antibodies whose serum contained autoantibodies specific for the phosphorylated form of RNA polymerase II (RNAP IIO), Su and ribosomal P antigen, as well as anti-topoisomerase I antibodies, a marker for scleroderma (SSc). Over 6 years, the patient exhibited clinical manifestations consistent with SLE without clinical evidence of scleroderma. The reactivity of her serum autoantibodies with the phosphoproteins ribosomal P, topoisomerase I, and RNAP IIO is consistent with recognition of autoepitopes comprised in part of phosphate groups. This may explain the unexpected coexistence of marker autoantibodies for SLE and scleroderma, possibly with implications for the mechanisms of autoantibody generation.

Adult↗