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PubMed2005

Bilateral primary orbital non-Hodgkin's lymphoma in a patient with scleroderma: a case report.

Orbital presentation of non-Hodgkin's Lymphoma (NHL) is uncommon but occurs both as the only site of disease and as a site of recurrence. Primary orbital NHLs are usually low-grade, and mostly extranodal marginal zone/mucosa associated lymphoid tissue B-cell neoplasms. They are commonly associated with chronic inflammatory conditions. The issue of bilaterality in orbital lymphoma is not very common. The onset of malignant lymphoproliferation may precede, follow, or exist simultaneously with scleroderma. Different treatment modalities were applied in orbital NHL such as surgery, radiation therapy, chemotherapy or both. We report a 57-year-old man with scleroderma presenting with eyelid hernias who had biopsy-proven marginal zone NHL, successfully treated with radiotherapy and combined chemotherapy, and also review the literature.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Effectiveness of a treatment based on melatonin in five patients with systemic sclerosis.

Systemic sclerosis (SSc) is a multisystem connective disorder in which endothelial damage leads to fibrotic reactions through platelet hyperaggregability and inappropriate release of platelet-derived growth factors. With other colleagues, I have previously reported that melatonin seems to have a direct favorable effect on endothelium (stopping of bleeding symptoms in thrombocytopenic patients). Furthermore, melatonin has been described as performing an antiaggregating activity, and ACTH and vitamin E have been described as increasing melatonin effects. This provided the rationale to treat 5 SSc patients with melatonin plus ACTH and vitamin E. Patients received the treatment for 1 month. The therapy was continued for 2 additional months in patients with stable or responding disease. After 3 months, the stable or responding patients continued the therapy for 2 months and longer. All patients had a partial response after 1 month. Continuing with the treatment, none of the 5 patients had disease progression (average follow-up time of 16.6 months; range, 7-44 months). Toxicity was lacking, with the only side effect being drowsiness. Our experience suggests that the combination of melatonin-ACTH-vitamin E may be safe and effective in patients with SSc.

Adrenocorticotropic Hormone↗
PubMed2005

Factors related to oral hygiene in persons with scleroderma.

OBJECTIVES: The purpose of this study was to assess potential risk factors for the development of poor oral hygiene and increased dental caries in individuals with scleroderma. METHODS: Twenty-two subjects with scleroderma participated in this study. All subjects were examined with the Patient Hygiene Performance (PHP) Index, which assesses the extent of plaque and debris over a tooth surface. In addition, oral aperature, xerostomia and dominant upper extremity strength, motion, skin thickness, and dexterity were measured. RESULTS: None of the subjects had good or excellent hygiene. Fifteen subjects had fair oral hygiene and seven subjects had poor oral hygiene as measured by the PHP Index. Significant differences were seen between these two groups for oral aperture, percentage of teeth with caries, skin thickness, dexterity and finger flexion. Dexterity and joint motion correlated significantly with the number of decayed surfaces and number of caries. Oral aperture correlated with the PHP Index and the number of dental caries. CONCLUSIONS: Subjects with poor oral hygiene required more time to complete manual dexterity task and had decreased hand strength and joint motion. Moreover, these same subjects had more contractures, scars, ulcers, and higher skin scores (more disease activity) than subjects in the fair hygiene group. In addition to frequent dental exam and routine cleaning, dental personnel may want to consider exercises to increase oral aperature and investigate toothbrush and dental floss selection used by persons with scleroderma.

Dental Caries↗
PubMed1995

Autoimmune vasculitis preceding aspirin-induced asthma.

Two female patients have been described in whom peripheral vasculitis with cold sensitivity preceded by about 5 years typical symptoms of aspirin-induced asthma. Vasculitis was immunologically mediated and took the form of either limited cutaneous scleroderma or perniosis. Antinuclear autoantibodies of SS-B specificity were present in the serum. Challenge with aspirin provoked asthma and distinct blood circulation disturbance in the hands. It is speculated that in these patients vasculitis and asthma were causally linked, and leukotrienes mediated bronchial and vascular reactions that occurred simultaneously following aspirin administration.

Adult↗
PubMed1999

Is anti-topoisomerase I a serum marker of pulmonary involvement in systemic sclerosis?

STUDY OBJECTIVE: To determine the value of the level of anti-topoisomerase I (anti-topo I) to evaluate lung involvement defined by abnormal high-resolution computed tomography (HRCT) score and pulmonary function tests (PFTs) in systemic sclerosis (SS). PATIENTS: Forty-eight patients with SS, 20 with lung involvement and 28 with no lung involvement. DESIGN: PFT measurement, HRCT scoring of lung involvement, and anti-topo I assay by enzyme-linked immunosorbent assay. Normal anti-topo I level was defined as < 30. RESULTS: There was a significant association between cutaneous extent and anti-topo I level (6.5% of patients with limited cutaneous scleroderma had abnormal anti-topo I levels vs 70.6% of patients with diffuse cutaneous scleroderma, p = 0.0001). In patients with diffuse cutaneous scleroderma, pulmonary involvement was associated with a higher percentage of abnormal anti-topo I level: 91.7% vs 20% (p = 0.010). In patients with diffuse cutaneous scleroderma, a significant association was found between the class of anti-topoII level and total lung capacity (median, 69 in patients with abnormal anti-topo I level vs 87 in patients with normal anti-topo I level, p = 0.010), between the class of anti-topo I level and HRCT score (median, 12 in patients with abnormal anti-topo I level vs 5 in patients with normal anti-topo I level, p = 0.05). CONCLUSION: Anti-topo I can be considered as a marker of lung involvement in patients with diffuse cutaneous scleroderma.

Adult↗
PubMed2006

Reduced exercise capacity and stress-induced pulmonary hypertension in patients with scleroderma.

OBJECTIVES: We sought to determine the incidence of stress-induced pulmonary artery (PA) systolic hypertension in a referral population of patients with scleroderma, and to examine the relation between stress-induced pulmonary systolic hypertension and exercise capacity in this population. BACKGROUND: Early detection of patients with scleroderma at risk for pulmonary hypertension (PHTN) could lead to more timely intervention and thus reduce morbidity and improve mortality. The change in PA systolic pressure (PASP) with exercise provides a possible tool for such detection. METHODS: Sixty-five patients with scleroderma (9 men and 56 women; mean age 51 +/- 12 years [SD]), normal resting PASP, and normal resting left ventricular function underwent exercise Doppler echocardiography using a standard Bruce protocol. Tricuspid regurgitation velocity was measured before and after exercise. Exercise variables including workload achieved in metabolic equivalents (METS), total exercise time, percentage of target heart rate achieved, and PASP at rest and within 60 s after exercise were recorded. RESULTS: Thirty patients (46%) demonstrated an increase in PASP to > 35 mm Hg plus an estimated right atrial pressure of 5 mm Hg. Postexercise PASP inversely correlated to both the maximum workload achieved (r = - 0.34, p = 0.006) and exercise time (r = - 0.31, p = 0.01). In women, the correlation was more significant (r = - 0.38, p = 0.003). Patients in the lowest quartile of exercise time, with the least cardiac workload achieved, produced the highest postexercise PASP. CONCLUSION: Stress-induced PHTN is common in patients with scleroderma, even when resting PASP is normal. Stress Doppler echocardiography identifies scleroderma patients with an abnormal rise in PASP during exertion. Peak PASP is linearly related to exercise time and maximum workload achieved. Measurement of PASP during exercise may prove to be a useful tool for the identification of future resting PHTN.

Blood Pressure↗
PubMed2003

[Pulmonary veno-occlusive disease in a patient with scleroderma and the CREST syndrome].

BACKGROUND: Pulmonary veno-occlusive disease is a rare and poorly understood condition that affects the postcapillary pulmonary vasculature, posing diagnostic problems and treatment dilemmas. MATERIALS AND METHODS: We present a patient with veno-occlusive disease and give a short review on the disease. RESULTS: The patient was a 54-year-old female with a history of the CREST variant of scleroderma. Admitted with dyspnoea, she was treated with epoprostenol in addition to oxygen, diuretics and warfarin. Epoprostenol improved her condition initially; her symptoms grew worse during further medical escalation. With an attempt to stop epoprostenol, however, she became even more dyspnoeic and tolerated best an intermediate dose. She died after three months of treatment with signs of progressive right heart failure. INTERPRETATION: Veno-occlusive disease may be difficult to diagnose and treat. Clinical signs of pulmonary hypertension without evidence of left ventricular failure may give rise to suspicion of the disease, and high-resolution CT of the lungs with relatively specific findings can be helpful. The prognosis is poor and lung transplantation is the only form of effective treatment. Vasodilators as a bridge to transplantation must be used with caution because of the risk of intolerance and development of pulmonary oedema.

Antihypertensive Agents↗
PubMed2005

HLA markers for susceptibility and expression in scleroderma.

OBJECTIVE: Reported associations between HLA alleles and both susceptibility to and features of scleroderma have been conflicting. Our objective was (1) to determine the role of HLA alleles in the susceptibility to scleroderma; and (2) to determine the role of HLA alleles in various aspects of disease expression. METHODS: Consecutive patients were followed in the scleroderma clinic between 1996 and 1998. Clinical data were obtained through chart review. Healthy volunteers as well as cadaveric donors served as controls. Molecular HLA typing was performed (polymerase chain reaction/sequence-specific oligonucleotides). Statistical analysis included Fisher's exact test and multivariate analyses, using logistic and linear regression models. RESULTS: Ninety-five Caucasian patients (75 women, 20 men, age 43.9 yrs, disease duration 11.9 yrs) with scleroderma and 416 controls were studied. HLA-DRB1*01 and HLA-DRB1*11 were associated with susceptibility to scleroderma, whereas HLA-DRB1*07 was protective. HLA-A*30 and HLA-A*32 were also associated with susceptibility to scleroderma, while HLA-B*57 and HLA-Cw*14 were protective. HLA-B*62 and HLA-DRB1*07 had a significant correlation with the presence of diffuse skin involvement in both univariate and multivariate analyses. HLA-DRB1*11 was associated with high skin score values, while lower values were related to the presence of HLA-Cw*14 and HLA-DQB1*06. Both alleles retained significance in a linear regression model. High skin score values were related to the absence of anticentromere antibodies. Pulmonary fibrosis was associated with HLA-B*62 and HLA-Cw*0602, whereas pulmonary hypertension was associated with HLA-B*13 and HLA-B*65. CONCLUSION: HLA alleles play a role in susceptibility to scleroderma and its disease expression.

Adult↗
PubMed2006

Functional hyperprolactinemia and hypophyseal microadenoma in systemic sclerosis.

OBJECTIVE: Hyperprolactinemia (HPRL) has been identified in more than half of patients with systemic sclerosis (SSc). However, the association with pituitary adenoma and the status of hypothalamic dopaminergic tone using metoclopramide (MTC) test has not been studied. We investigated the prevalence of prolactin (PRL)-secreting pituitary adenoma and evaluated production of PRL by dynamic testing with MTC in SSc. METHODS: We studied 30 patients with SSc (mean age 38 +/- 10 yrs) and 20 healthy controls (mean age 37 +/- 11 yrs). Serum PRL concentrations were determined by radioimmunoassay in all subjects, and PRL response was measured 30, 60, 90, and 120 min after injection of 10 mg of MTC. Computed tomography (CT) of the sella turcica was performed. RESULTS: The mean basal serum PRL levels before and after stimulation with MTC in SSc patients versus controls were: basal 18.2 +/- 5.4 versus 8.7 +/- 1.6 ng/ml, p = NS; 30 min: 175.0 +/- 5.4 versus 61.0 +/- 42 ng/ml, p < 0.001; 60 min: 160 +/- 64 versus 52 +/- 30 ng/ml, p < 0.001; 90 min: 125 +/- 57 versus 42 +/- 21.0 ng/ml, p < 0.05; 120 min: 108.0 +/- 57 versus 30.0 +/- 10 ng/ml, p < 0.005. CT scan showed microadenomas in 24/30 SSc patients and 1/20 controls (p = 0.001). CONCLUSION: Our study suggests that a group of patients with SSc have a high prevalence of HPRL with increased central dopaminergic tone, and microadenomas. PRL may have a role in the pathogenesis of SSc. Further studies are necessary to confirm our results.

Adenoma↗
PubMed2006

Ischemic scleroderma wounds successfully treated with hyperbaric oxygen therapy.

Hyperbaric oxygen therapy (HBOT) has been used to treat refractory wounds for the last several decades, with the majority of research focusing on wounds secondary to arterial insufficiency. We describe 2 patients with scleroderma with intractable bilateral extremity ulcers. Local ischemia was identified using transcutaneous oximetry. Each patient then underwent 30 treatments of HBOT at a relative depth of 2.4 ATA with resulting wound healing. This is the first reported successful use of HBOT to treat scleroderma ulcers, and may represent an unrecognized treatment option for these notoriously difficult chronic wounds.

Adult↗
PubMed2006

Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension.

OBJECTIVE: Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). We describe a series of 4 patients with systemic sclerosis (SSc), concomitant PH, and biopsy-proven/presumed PVOD. We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect.

Fatal Outcome↗
PubMed1997

Sjögren's syndrome with anticentromere antibodies.

Clinical and laboratory findings in 10 women with Sjogren's syndrome and positive anticentromere antibodies were compared to those in 50 patients with Sjögren's syndrome and typical serologic features. The anticentromere antibody-positive patients were more likely to have Raynaud's phenomenon and less likely to have leukopenia polyclonal hypergammaglobulinemia, rheumatoid factor, and anti-SSA/Ro antibody. Four anticentromere antibody-positive patients developed limited cutaneous scleroderma during follow-up, but none had lymphoma; some of the other patients in this group exhibited stable symptoms typical for Sjögren's syndrome. Among our overall population of Sjogren's syndrome patients who met diagnostic criteria for primary Sjogren's syndrome at the first evaluation, 16.6% tested positive for anticentromere antibodies.

Adult↗
PubMed1998

Clinical manifestations of systemic sclerosis.

Systemic sclerosis is a multisystem disease characterized by inflammation and fibrosis of many organs. There are two major subsets, limited cutaneous (the old CREST syndrome) and diffuse cutaneous scleroderma. The major difference is the pace of disease. Limited scleroderma patients often have a long history of Raynaud's phenomenon before other symptoms. They have skin thickening limited to hands and frequently have problems with digital ulcers and esophageal dysmotility. Although generally a milder form than diffuse scleroderma, they can have life-threatening complications from small intestine hypomotility and pulmonary hypertension. Diffuse scleroderma patients have a much more acute onset, with many constitutional symptoms, arthritis, carpal tunnel syndrome, and marked swelling of hands and legs. They get widespread skin thickening, progressing from their fingers to their trunk. Internal organ problems, including gastrointestinal and pulmonary fibrosis, are common, but severe life-threatening involvement of the heart and kidneys occurs. Understanding the type of disease that occurs in these two subsets will enable the physician to anticipate problems, aggressively treat those that can be treated, and give the patient a better understanding of their disease.

Adult↗