Multiple sclerosis in the course of systemic sclerosis.
Explore the source record for details and available documents.
Adult↗
Biomedical subjects
A P Andonopoulos
Publications and source records attributed to A P Andonopoulos.
Dermatomyositis in a patient with adenocarcinoma of the gall bladder.
Explore the source record for details and available documents.
Adenocarcinoma↗
Is a purified protein derivative skin test and subsequent antituberculous chemoprophylaxis really necessary in systemic rheumatic disease patients receiving corticosteroids?
Our objective was to determine whether a purified protein derivative (PPD) skin test and subsequent isoniazid administration to patients with systemic rheumatic disease, who react positively and are about to receive corticosteroids, is necessary. For this purpose, 451 unselected patients with systemic rheumatic diseases, such as rheumatoid arthritis, giant cell arteritis, polymyalgia rheumatica, systemic lupus erythematosus, scleroderma, poly- and dermatomyositis, mixed connective tissue disease, eosinophilic fasciitis, systemic necrotising vasculitis and Behçet's disease, were observed over a 6-year period. All patients had been started on steroids after commencement of the study and had received the drug for at least 1 year by the end of the study. A chest X-ray was performed before entry, every 6 months for the first year and yearly thereafter. A PPD skin test had been performed in 40 patients by other physicians, but it was our policy to omit the test. We divided our patients into age groups by decades. Steroid dosage varied according to diagnosis and severity. An initial chest X-ray revealed old inactive tuberculosis in 65 patients. During the follow-up period, none of the patients exhibited clinical or radiological evidence of reactivations of tuberculosis. However, at least 184 of the patients would have had a positive PPD skin test reaction, if tested. This figure is derived from the results of several studies on Greek army recruits whose current ages correspond to those of our patients. In conclusion, our results suggest that screening with a PPD and isoniazid prophylaxis, with all the potential risks for those who test positive, may not be necessary in patients with systemic rheumatic disease who will receive steroids.
Adrenal Cortex Hormones↗
Multiple spontaneous intracerebral hemorrhages in a patient with progressive systemic sclerosis.
A 64-year-old woman with a two-year history of diffuse scleroderma responsible for severe interstitial lung disease was admitted for recurrent loss of consciousness. Her treatment at the time consisted of a corticosteroid and monthly cyclophosphamide pulses. Computed tomography and magnetic resonance imaging of the brain revealed two hemorrhagic lesions in the left frontal and temporal lobes, respectively. Amyloidosis and/or vasculitis may have contributed to these lesions.
Aged↗
Autonomic cardiovascular neuropathy in Sjögren's syndrome. A controlled study.
OBJECTIVE: To test patients with primary Sjögren's syndrome (SS) for evidence of autonomic neuropathy. METHODS: Thirty-two patients with primary SS and 22 age and sex matched healthy individuals were asked specific questions about symptoms suggestive of autonomic neuropathy, and were subjected to a battery of 5 cardiovascular tests: response of blood pressure to sustained hand grip, Valsalva maneuver, heart rate response to deep breathing, and heart rate and blood pressure response to standing up. The chi-squared test with Yates' correction and 95% confidence intervals were used for statistical analysis of the results. RESULTS: Sixteen patients (50%) had symptoms of autonomic neuropathy when specifically asked versus none of the controls (p < 0.0005). The frequency of abnormal responses to the tests was 68.8% in patients and 12.7% in controls (p < 0.0001). Severe autonomic cardiovascular neuropathy was found in 87.5% of the patients but in none of the healthy individuals (p < 0.0001). CONCLUSION: Our results suggest that autonomic neuropathy is a feature of a significant portion of the SS population, and such patients should have appropriate evaluation. Similarly, patients with unexplained autonomic neuropathy should be investigated for evidence of SS.
Adult↗
Intrahepatic bile duct injury and nodular regenerative hyperplasia of the liver in a patient with polyarteritis nodosa.
Although involvement of the hepatic vasculature in patients with polyarteritis nodosa is not unusual, biliary manifestations are very rare. We describe a patient with polyarteritis nodosa presenting with a febrile cholestatic anicteric syndrome. Histological examination of the liver revealed necrotizing arteritis of small hepatic arteries associated with significant lesions of intrahepatic bile ducts of the sclerosing cholangitis type, i.e. fibrous collar around the ducts, periductal inflammation and ductal proliferation. Concomitant nodular regenerative hyperplasia was found, a condition which has rarely been described in association with polyarteritis nodosa. We think that hepatic arteritis compromising arterial blood flow to the liver was responsible for the most likely ischemic nature of the bile duct injury and the nodular regenerative hyperplasia seen in our patient.
Aged↗
Sjögren's syndrome in patients with newly diagnosed untreated non-Hodgkin's lymphoma.
The purpose of the study was to detect cases of Sjögren's syndrome among newly diagnosed untreated patients with non-Hodgkin's lymphoma and, furthermore, to identify in such cases clinical and serologic features known to occur more frequently in Sjögren's syndrome patients who evolve into lymphoma. Accordingly, thirty-three cases of newly diagnosed non-Hodgkin's lymphoma, prior to any treatment administration, were thoroughly studied for evidence of Sjögren's syndrome. Immunophenotyping for T and B cells and kappa and lambda light chains was concomitantly performed on both lymphomatous tissues and minor salivary glands. There were 5 patients with T cell and 28 with B cell lymphoma of various histologic subtypes and grades. Two of the latter (7.1%) had a positive for Sjögren's syndrome minor labial salivary gland biopsy, positive responses to the specific questionnaires for both eye and mouth dryness and abnormal Schirmer's and rose Bengal eye tests, substantiating the diagnosis of Sjögren's syndrome. Both were male with lung and stomach non-Hodgkin's lymphoma respectively, enlargement of the lacrimal glands, monoclonal gammapathy of the IgM kappa type and, one of them had high titer and of fine speckled pattern positive antinuclear antibodies and anti-Ro(SSA) and anti-La(SSB) antibodies in his serum. A monotypic infiltrate with kappa light chain restriction, identical to that in the lymphomatous tissue of these two patients, was present in their minor salivary gland biopsy as well. Such a finding was not encountered in any of the remaining patients. Although our sample is relatively small, our results confirm the relationship between Sjögren's syndrome and non-Hodgkin's lymphoma, looked at from the opposite direction. Obviously, studies involving larger populations would be more definitive, regarding the issue of what percentage of this lymphoma patients originates from Sjögren's syndrome.
Adult↗
Thyroid function and immune profile in rheumatoid arthritis. A controlled study.
The aim of our study was to determine the prevalence of thyroid dysfunction and autoimmune abnormalities in rheumatoid arthritis (RA) and to further investigate the possible association between D-penicillamine and autoimmune thyroiditis. For this purpose, one hundred and one unselected consecutive patients with RA and 70 age and sex matched controls were studied prospectively. Evaluation included a complete history and physical examination with special attention to symptoms suggestive of thyroid pathology, routine laboratory and serologic immune profile, plus determination of serum levels of thyroxine (T4), triiodothyronine (T3), thyroid stimulating hormone (TSH), antibodies to thyroid peroxidase (AbTPO) and TSH receptor antibodies (TRAB). Serum thyroxine binding globulin (TBG) was measured in all subjects with high thyroid hormone levels, whereas free T3 and T4 concentrations were determined in all individuals with abnormal T3, T4, TSH or TBG. Six patients with hyperhyroidism, 3 with hypothyroidism and 1 with the euthyroid hyperthyroxinemia (EH) syndrome were found, whereas four of the controls had hyperthyroidism. Thirteen patients and 6 controls had high AbTPO levels whereas no one had high TRAB. No association was detected between thyroid abnormalities and any serologic RA finding. Furthermore, no correlation between thyroid dysfunction and elevated AbTPO's was found. A relatively high prevalence of thyroid dysfunction (9,9%) and subclinical autoimmune thyroiditis (12,9%), the latter indicated by elevated AbTPO's, was found in our RA patients. These figures were higher than those in the control group (5,7% and 8,6% respectively), but the difference did not reach statistical significance. Of further interest may be our finding that, despite anecdotal reports blaming D-penicillamine for cases of autoimmune thyroiditis, the incidence of the latter was similar among recipients and nonrecipients of the drug. Similarly, TRAB were not detected in any patient treated with D-penicillamine.
Adult↗
Autonomic cardiovascular neuropathy in primary Sjögren's syndrome.
The case of a 65-year-old female with primary Sjögren's syndrome and severe autonomic cardiovascular neuropathy, manifested by incapacitating postural hypotension, is presented. It is postulated that the patient's Sjögren's syndrome was responsible for the autonomic neuropathy, probably via a vasculitic mechanism. Treatment with hydrocortisone resulted in improvement in the patient's symptoms but it did not significantly alter the objective findings of autonomic dysfunction. We suggest that it is probably worth studying Sjögren's patients for evidence of autonomic neuropathy.
Aged↗
Superior sagittal sinus thrombosis in a patient with Behçet's disease and the Mayer-Rokitansky-Küster-Hauser syndrome.
Explore the source record for details and available documents.
Abnormalities, Multiple↗
D-penicillamine induced myasthenia gravis in rheumatoid arthritis: an unpredictable common occurrence?
Five patients out of 71 with rheumatoid arthritis (RA), who received D-penicillamine, developed myasthenia gravis (MG) within a two-year period. They all responded promptly to discontinuation of the drug and pyridostigmine administration. None of the patients had anti-Ro(SSA) antibodies or features of Sjögren's syndrome, whereas three of the five had the HLA-DR1 phenotype. The relatively high frequency of MG observed in our population, along with its unpredictability and potentially serious sequelae, necessitates its inclusion in the list of side effects of D-penicillamine routinely discussed with the patient, prior to initiation of the treatment. Full alertness of both the patient and the physician to even minor initial myasthenic symptoms, that dictate immediate discontinuation of the drug, is of obvious importance.
Aged↗
Peripheral blood mononuclear cells from patients with rheumatoid arthritis suppress erythropoiesis in vitro via the production of tumor necrosis factor alpha.
Anemia is a frequent manifestation of rheumatoid arthritis, with a probably multifactorial etiology. We investigated the effect of peripheral blood mononuclear cell supernatants (PBMCS) from rheumatoid arthritis (RA) patients on hematopoietic colony formation in vitro, by using a methylcellulose assay. PBMCS from patients suppress in vitro erythroid (BFU-E), mixed-lineage (CFU-GEMM) and to a lesser degree granulocyte-macrophage (CFU-GM) progenitors. PBMCS from anemic RA patients were more suppressive for BFU-E than those from non-anemic patients. Addition of antibodies to tumor-necrosis factor alpha (TNF alpha) almost completely reversed the inhibition of BFU-E and CFU-GEMM, but had little effect on the CFU-GM colony formation. Antibodies to interferon-gamma (IFN gamma) and interleukin-1 (IL-1) were not effective. The above data suggest that PBMCS from RA patients suppress in vitro erythropoiesis via the production of TNF alpha; a pathogenetic role for TNF alpha in the anemia of RA can be implied.
Adult↗
Excessive fibrosis of supraclavicular lymph-node granulomas in a patient with progressive systemic sclerosis.
The case of a 29-year-old white female with a 7-year history of typical scleroderma is presented who developed excessive fibrosis of the supraclavicular lymph nodes. After 3 years of disease, firm right supraclavicular lymphadenopathy appeared, accompanied by a high fever. Biopsy revealed non-caseating granulomas and short-term antituberculous therapy was ineffective. The symptoms finally responded to steroids, but adenopathy persisted. A second biopsy, 40 days after the first, disclosed a similar picture with some degree of fibrosis of the granulomas. Four years later, with stony hard right supraclavicular adenopathy persisting, a third biopsy showed excessive fibrosis of the granulomas within the node and destruction of its architecture. It is postulated that the primary disease of this patient might be responsible for this clinical picture. The present seems to be the first report of such a case in the literature.
Adult↗
Subcutaneous edema: an "unrecognized" feature of acute polymyositis.
The case of a man with acute onset of muscle pain, weakness, anasarca, severe dysphagia and dysphonia, and biochemical, electromyographic and histologic evidence of polymyositis is presented. The literature on the occurrence of subcutaneous edema in polymyositis was reviewed. It is concluded that this particular symptom, with no other apparent cause, including heart failure from the underlying disease, is a rare but definite feature of polymyositis itself. A correlation of that with severe bulbar muscle involvement is also suggested.
Acute Disease↗
Light microscopy of dried saliva in the evaluation of xerostomia of the sicca syndrome. A preliminary report.
Dried, freshly produced saliva from 21 patients with xerostomia related to the sicca syndrome [15 with primary Sjögren's syndrome (pSS), 3 with rheumatoid arthritis (RA) and secondary Sjögren's syndrome (sSS), and 3 with Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE)] and 21 age and sex matched controls, was examined by light microscopy. A typical fern-like pattern was demonstrated by the crystallized mucus of the healthy individuals. In contrast, much thicker, shorter, irregular and densely arranged branches of crystallized mucus, sometimes giving a reindeer horn appearance, were observed in the patients' saliva. Given the lack of a reliable clinical measure for the objective evaluation of xerostomia, light salivary microscopy, simple and easy as it is, may fill this deficit, if its sensitivity and specificity are documented.
Aged↗
Sarcoidosis in a patient with myasthenia gravis. Case report and review of the literature.
The case of a 38-year-old white female, with a long history of myasthenia gravis, who developed sarcoidosis is presented. The diagnosis of myasthenia gravis had been established on the basis of typical clinical symptoms with severe myastenic crises necessitating respiratory support, characteristic electromyographic findings, positive anti-acetylcholine receptor antibodies and response to appropriate medications. After 9 years of disease and while in apparent remission, she presented with ankle arthritis and bilateral hilar adenopathy. A transbronchial lung biopsy showed noncaseating granuloma, typical of sarcoidosis. To our knowledge, the combination of myasthenia gravis and sarcoidosis is extremely rare and emphasizes the intriguing tendency of some immunologic disorders to appear together in certain individuals.
Adult↗
Adult respiratory distress syndrome: an unrecognized premortem event in systemic lupus erythematosus.
A retrospective study of the medical records of 46 patients, comprising our SLE population over a 6-year period, noted that mortality (7 of 46:15.2%) was directly related to the adult respiratory distress syndrome. In five, a concomitant severe infection was detected. Overall, 17 of our 46 patients presented with infectious episodes. The only statistically significant factor predisposing to both infection and the adult respiratory distress syndrome was high dose corticosteroid administration. Our results suggest that adult respiratory distress syndrome is a clinical condition related to the death of lupus patients and commonly complicates infection.
Adult↗
Methotrexate therapy in rheumatoid arthritis. A two year prospective follow-up.
One hundred and thirty seven rheumatoid arthritis (RA) patients refractory to D-penicillamine and some of them (15%) refractory to other slow active drugs were treated with oral methotrexate (MTX) (10-15 mg weekly). After 12-24 months of treatment, 94 and 74 patients respectively showed a significant improvement as judged by duration of morning stiffness (p less than 0.0001), grip strength (p less than 0.0001), degree of joint swelling (p less than 0.01) and tenderness (p less than 0.0001) compared to pre-treatment values. This clinical improvement was also associated with a decrease of erythrocyte sedimentation rate (p less than 0.001), decrease of C-reactive protein (p less than 0.0001) and with improvement of anaemia (p less than 0.05). No changes were seen in rheumatoid factor titres. Seventy-four of the patients were followed for up to 24 months. Thirty-one of them (23%) had complete remission and 43 (31%) had an excellent response. Adverse drug reaction during MTX therapy included: elevated liver enzymes in 34 patients, mucosal ulcers in 21, nausea and vomiting in 8, diarrhoea in 4, leukopenia in 2, interstitial pneumonitis in one, intestinal bleeding in one and finally septic arthritis in another patient. The majority of these side effects were resolved without sequelae. However, 15 patients (11%) with adverse drug reactions had to discontinue the treatment. Forty-one of our patients who received a cumulative mean dose of MTX of 1550.5 +/- 235.5 mg underwent a percutaneous liver biopsy. Ten patients had normal tissue, 12 had minimal changes, 13 nonspecific changes and 6 patients had mild fibrosis.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗