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ID PMID Title PublicationDate abstract
10599067 [Efficacy of radiation synovectomy in degenerative inflammatory and chronic inflammatory j 1999 AIM: Effect of radiosynovectomy (RS) should be evaluated both by subjective and objective parameters in patients with osteoarthritis and in patients with inflammatory joint disorders not caused by rheumatoid arthritis. METHODS: A total of 98 joints in 61 patients were investigated. Patients were divided into two groups. The first group included 35 patients with therapy-resistant effusions caused by severe osteoarthritis (46 joints). The second group consisted of 26 patients (52 joints) with ankylosing spondylitis, reactive arthritis, undifferentiated spondylarthropathy, psoriatic arthritis, pigmented villo-nodular synovitis, and recurrent synovitis following surgery. Effect of RS was evaluated by a standardized questionnaire and quantified by T/B-ratios derived from blood pool images prior to and after RS. RESULTS: Within the first patient group suffering from osteoarthritis, 40% showed a good or excellent improvement of clinical symptoms, 51% were unchanged, and in 9% symptoms worsened. Similar results were found in the second patient group. The majority of unchanged results were small finger joints. In contrast, wrist and knee joints showed a better improvement. Good correlation between results of bone scan and patients subjective impression was found in 38% and 67% in the first and the second patient group, respectively. CONCLUSION: Radiosynovectomy might be an effective treatment in osteoarthritis and inflammatory joint disorders not caused by rheumatoid arthritis.
9725091 Undifferentiated arthritis and reactive arthritis. 1998 Jul The terms undifferentiated arthritis and undifferentiated characterize arthritides that do not fit into well-known clinical disease categories (e.g., seronegative rheumatoid arthritis and reactive arthritis) and that are an early stage or forme fruste of a definite rheumatic disease, an overlap syndrome between such diseases, or an unknown, etiologically undefined disease that remains to be differentiated from other types of arthritis or spondylarthritis. Undifferentiated arthritis and undifferentiated spondylarthritis share some clinical features with reactive arthritides. Recent data suggest that, at least in Chlamydia-induced reactive arthritis, the triggering bacteria persist in affected joints for some time during the course of the disease in a viable but nonreplicative state, providing an antigenic stimulus for a bacteria-specific immune reaction in the joint. The clinical manifestations of reactive arthritis include not only Reiter's syndrome or clinically suspected postinfectious arthritis but also undifferentiated oligoarthritis and spondylarthritis. The optimal treatment remains to be defined, but there is increasing data that antibiotic therapy is not as effective in cases of well-established reactive arthritis as has been suggested.
15881745 [Sjögren syndrome associated with renal tubular acidosis type I]. 2000 Primary Sjögren's Syndrome complicated with a renal tubular acidosis type 1 and hypocalcemic paralysis, as the principal clinical manifestation, is uncommon. Although the initial manifestations of the nephropathy are not well understood, it is believed that the invasion of mononuclear cells and the high level of circulating antibodies, play an important role in the pathogenesis of the disease. We present a patient with hypocalcemic paralysis as an initial manifestation of a latent Sjögren's disease. The glandular biopsy was normal, suggesting a mayor participation of an immunological humoral factor in the renal lesion.
11147760 Heart rate variability in patients with Sjögren's syndrome. 2000 Heart rate variability (HRV) gives information about sympathetic parasympathetic autonomic balance. Our purpose was to determine whether HRV is abnormal in patients with Sjögren's syndrome. In 16 patients with Sjögren's syndrome and 30 matched controls, a short time analysis of HRV was performed for both the frequency and the time domain. In the time domain, patients tended to display a slower heart rate, greater R-R variability and higher standard deviation of the mean (SDNN) than did healthy subjects, but the differences were not statistically significant. In the frequency domain the spectral measures of HRV showed a slight reduction of LF and an increase of HF; as a result, the ratio between high and low frequencies, representative of sympathovagal modulation, was significantly reduced. Our data suggest an increase in the parasympathetic control of heart rate in patients with Sjögren's syndrome. This predominance in vagal tone could exert a protective and antiarrhythmic role in patients with primary Sjögren's syndrome, and may be relevant with reference to the lower incidence of sudden death in this disorder compared to other major autoimmune diseases.
10503565 Bone scintigraphy and magnetic resonance imaging in adult-onset Still's disease. 1999 Adult-onset Still's disease (AOSD) is an acute systemic inflammatory disorder of unknown origin. We report a patient whose AOSD presented with the commonly accepted diagnostic clinical signs and laboratory parameters. The painful joints distinctly demonstrated increased uptake of 99mTc-methylene diphosphonate in scintigraphy and areas of increased gadolinium-enhanced signal in MRI. Biopsies indicated bone marrow edema. AOSD in association with bone marrow edema had not been previously demonstrated. AOSD is often diagnosed after a considerable delay, bone scintigraphy, and magnetic resonance imaging may offer new imaging techniques for early diagnosis and successful therapy in follow-up examinations.
9893303 Subclinical Sjögren's syndrome: a significant 67gallium accumulation in the orbits and pa 1998 Dec An 8-year-old girl with hypergammaglobulinemia showed an abnormal 67gallium accumulation in the orbits and parotid glands. Although she did not have any subjective siccant complaints, reported typical histopathological and sialographic changes suggesting Sjögren's syndrome (SjS) were observed in the salivary glands. Gallium scintigram might be a valuable and non-invasive diagnostic tool in the diagnosis of children with SjS without sicca symptoms.
9726334 Coexisting Sjögren's syndrome and sarcoidosis in the lung. 1998 Aug CONTEXT: Sjögren's syndrome (SS) and sarcoidosis are diseases of unknown origin that are considered to result from abnormal regulation of the immune system. Pulmonary involvement by SS and sarcoidosis may have similar clinical and radiographic manifestations, making it difficult for the clinician to distinguish between these diseases. OBJECTIVES: This study was undertaken to analyze the characteristics of SS and sarcoidosis in the lung to identify distinguishing features that may assist clinicians in the differentiation of these conditions. DESIGN: We present two cases with severe pulmonary impairment in which the distinction between SS and sarcoidosis required lung tissue biopsy. The literature regarding the pulmonary manifestations of these diseases is reviewed. RESULTS: The clinical, pathological, radiographic, and physiological characteristics of lung disease in the setting of SS and sarcoidosis can be very similar, preventing a diagnosis solely on clinical grounds. This is exemplified in the two cases reported. In one patient who carried the diagnosis of sarcoidosis, examination of lung tissue revealed lymphocytic interstitial pneumonitis consistent with SS. In the other patient, who had previously been diagnosed with SS on clinical grounds, examination of lung tissue showed lymphocytic interstitial pneumonitis with scattered noncaseating granulomas, suggesting the possibility of coexisting SS and sarcoidosis. A literature review indicated that lung involvement by SS may be difficult to distinguish from that of sarcoidosis. Furthermore, several cases have been reported in which both diseases coexisted. CONCLUSIONS: Because SS and sarcoidosis may coexist and present with similar pulmonary manifestations, aggressive evaluation including tissue biopsy may be required. However, even tissue biopsy may not distinguish between these entities unless noncaseating granulomas are seen (in the case of sarcoidosis) or isolated lymphocytic interstitial pneumonitis is detected (in the case of SS). When both features (ie; noncaseating granuloma and lymphocytic interstitial pneumonitis) are encountered in the same organ, we believe these diseases are coexisting. Distinguishing both conditions may have prognostic implications, because sarcoidosis may present as an autolimiting process and frequently resolves spontaneously without significant residual functional impairment. In contrast, pulmonary involvement with SS often leads to permanent defects and may progress to incapacitating disease.
9301147 A case of multicentric reticulohistiocytosis, systemic sclerosis and Sjögren syndrome. 1997 Aug We report the case of a 42-year-old Japanese woman who developed multicentric reticulohistiocytosis (MR) complicated by systemic sclerosis (SSc) and Sjögren syndrome (SS). The patient complained of tender nodules on the left hand, polyarthralgia in the finger joints and knees, and xerostomia. The skin nodules were distributed mainly on her hands and fingers with skin sclerosis. The serum anti-nuclear test revealed anti-centromere antibody and the discrete speckled pattern of anti-nuclear antibody. The biopsy specimens from the finger nodule and the sclerotic finger skin showed a perivascular infiltration of multinucleated giant cells with ground-glass cytoplasm and dermal thick collagen proliferation, respectively. The lip biopsy and sialography specimens showed periductal lymphocyte infiltration and apple tree-like changes. Systemic corticosteroid treatment improved the polyarthritis, xerostomia, and skin sclerosis rapidly but suppressed the nodular lesions only gradually. This is the first report of a combined case of MR, SSc and SS. This multiple autoimmune complication suggests the involvement of an immunological disturbance in the pathogenesis of MR.
11822094 [Primary Sjögren's syndrome. A longitudinal study of 68 patients]. 2001 Dec There are only few studies, regarding primary Sjögren's syndrome (pSS) long-term clinical course. Moreover, it has been often studied in a restricted number of patients, employing different recruitment and diagnostic criteria. During a 10 years follow-up, we longitudinally evaluate clinical course as well as severe complications and mortality rates in 68 patients with pSS, diagnosed according to the Fox's criteria. Patients were divided into 2 groups according to the autoantibodies pattern detected at the diagnosis: anti-Ro and/or La positive and anti-Ro La negative. Glandular manifestations of pSS were distinctively present in the majority of patients already at time of the diagnosis and serological findings remained typically constant during the whole follow-up. Increased IgG, IgA and ESR as well as low C4 serum levels were significantly prevalent in the Ro and/or La positive group. Finally, we did not found any significant increase in the mortality rate of pSS patients in comparison with the general population.
11819900 An update on the management for the dental patient with Sjögren's syndrome and xerostomia 1999 Jul Sjögren's syndrome is a chronic and uncomfortable inflammatory condition for the individuals who suffer from it. There are many and varied systemic and oral complications associated with Sjögren's syndrome. It is also a complex and challenging condition for the dentist to diagnose and manage. The key concepts are early recognition and intervention to prevent the secondary complications from hyposalivation. To the degree possible, salivary flow should be restored by either artificial salivas or stimulated by secretogogues or both, which is usually the case. Atrophy and secondary infections of the oral mucosa should be properly identified, effectively treated, and frequently monitored. Pilocarpine HCl (Salagen) has been shown to be effective in increasing salivary flow in patients with SS. Preventive and supportive therapy including supplemental fluorides, dietary assessment, and frequent professional recalls are imperative to maintaining the oral health of the patient with SS.
11078154 Comparison of the anti-Ro/SSA autoantibody profile between patients with primary and secon 2000 Sep OBJECTIVE: To measure serum levels of anti-Ro52-kD/SSA, anti-Ro60-kD/SSA and anti-La/SSB autoantibodies in patients with primary and secondary Sjögren's syndrome. To examine if there is any connection between the disease and the subtype-spectrum of these antibodies. METHODS: We measured serum levels of anti-Ro52-kD/SSA, anti-Ro60-kD/SSA and anti-La/SSB autoantibodies by ELISA, in the sera of patients with primary Sjögren's syndrome with or without extraglandular manifestations and with or without anti-La/SSB positivity and of patients with systemic lupus erythematosus/Sjögren's syndrome overlapping disease with or without anti-La/SSB positivity. RESULTS: Differences of the distribution of the anti-Ro52-kD/SSA and the anti-Ro60-kD/SSA were found between the primary and secondary Sjögren's syndrome patients' groups; when Sjögren's syndrome is accompanied by systemic lupus erythematosus, the occurrence of anti-Ro60-kD/SSA autoantibodies is significantly higher than in primary Sjögren'syndrome. CONCLUSION: Our results suggest that there is a possible connection between the distribution of the subtypes of the anti-Ro/SSA autoantibodies and the disease type in primary/secondary Sjögren's syndrome.
10084165 Validity of the saliva ferning test for the diagnosis of dry mouth in Sjögren's syndrome. 1999 Feb OBJECTIVE: To study the validity of saliva ferning patterns as a diagnostic test for dry mouth in primary or secondary Sjogren's Syndrome (SS). METHODS: Salivary smears were collected from 25 patients with Sjögren's syndrome in the fasting and nonfasting state. All 25 patients had symptomatic xerostomia and xerophthalmia and a positive Shirmer's test. Smears were taken from four sites, the cheek, lower lip, tongue, and saliva. Tests were done for rheumatoid factor, antinuclear antibodies, and anti-Ro(SS-A) antibodies. The salivary smears were air-dried and examined under a light and a polarizing microscope. Smears from 25 healthy subjects were also examined as controls. RESULTS: Three patterns of salivary secretion were identified, namely normal geometric ferning, reindeer antler ferning, and thick branching ferning. All Sjögren's syndrome patients had abnormal salivary smears, usually with a combination of reindeer antler ferning, thick branching ferning, and mucosal squames. This combination was seen in six of the 25 fasting specimens (24%); most of the remaining fasting samples showed the reindeer antler ferning. Reindeer antler ferning alone was found in five fasting and four nonfasting samples: this pattern was absent from five fasting and five nonfasting samples in which mucosal squames were the only abnormal finding. All nonfasting control samples exhibited normal geometric ferning. Smears from the cheek and saliva provided the most illustrative findings. CONCLUSION: The saliva ferning test is a simple, reproducible, and useful diagnostic aid in autoimmune xerostomia, approximately equivalent to Shirmer's test in xerophthalmia.
11301610 [Diagnosis of primary Sjogren's syndrome]. 2001 Feb 20 BACKGROUND: The diagnosis of primary Sjögren's syndrome largely depends on pathological findings at lower lip biopsy, or the presence of anti SSA and/or anti SSB antibodies. The present study evaluated which clinical and laboratory features among patients with sicca symptoms could predict a positive biopsy. MATERIAL AND METHODS: All 217 patients evaluated for sicca symptoms at Aust-Agder Central Hospital, Arendal, Norway from 1989 through 1998 were retrospectively reviewed. RESULTS: 136 biopsies were performed. 59 patients were diagnosed with primary Sjögren's syndrome. A reduced Schirmer I test combined with either an elevated ESR, positive ANA or elevated serum gammaglobulin had a high positive predictive value for primary Sjögren's syndrome. INTERPRETATION: Among patients with sicca symptoms, those with laboratory evidence of inflammation, autoimmunity or exocrine dysfunction should be subjected to a lower lip biopsy for a final diagnosis of primary Sjögren's syndrome.
9385738 The uncertain role of immunosuppressive agents in Sjögren's syndrome. 1997 Nov Although Sjögren's syndrome is an autoimmune disorder, immunosuppressive agents have yielded disappointing results in clinical trials. In this paper we summarize our experience using immunosuppressive and cytotoxic agents to treat Sjögren's syndrome, and present an illustrative case history.
10928197 Is it juvenile rheumatoid arthritis or fibromyalgia? 2000 Jul For the clinician evaluating adolescents with chronic musculoskeletal pain and fatigue, the distinctions between JRA and FS are clear based on physical examination findings. The two conditions can coexist. For the patient with an initial diagnosis of either JRA or FS whose clinical response to therapy is not in keeping with expectations or physical examination findings or whose clinical course worsens without explanation, reevaluation to determine if FS in the JRA patient has developed or JRA in the FS patient has emerged is warranted. Until clinicians have a better understanding of the intricacies of the neurohormonal and immunologic systems and how they affect somatic symptoms, they can continue to provide patients with a treatment plan based on current knowledge that should minimize patients' discomfort and allow them to have appropriately functional lives.
11819063 Mega os trigonum in progressive pseudorheumatoid dysplasia. 2002 Jan BACKGROUND: Progressive pseudorheumatoid dysplasia (PPRD), a noninflammatory condition, needs to be differentiated diagnostically from juvenile rheumatoid arthritis (JRA). OBJECTIVE: Demonstration of an unusually large and often early-appearing os trigonum helps distinguish PPRD from JRA. MATERIALS AND METHODS: Ankle images in four children with PPRD were reviewed. RESULTS: The os trigonum was abnormally enlarged in all PPRD subjects and was shown to have appeared or fused earlier than normal in two subjects. CONCLUSION: A large and early os trigonum ossification helps differentiate PPRD from JRA.
11122038 Bilateral upper limb lymphoedema associated with psoriatic arthritis: a case report and re 2000 Dec Lymphoedema is an unusual extra-articular feature of rheumatoid arthritis and has rarely been described in psoriatic arthritis. We report a 41-year-old man with psoriasis and psoriatic arthritis who developed bilateral lymphoedema of the upper extremities. Lymphoscintigraphy showed absent lymphatic drainage in the right arm and a subnormal increase in lymphatic flow under manual exertion in both arms. Colour Doppler ultrasound studies did not reveal venous or arterial abnormalities. Conservative management and therapy with cyclosporin (for worsening arthritis) resulted in partial resolution of the lymphoedema and improvement of flow parameters on the right side upon repeat lymphoscintigraphy.
9035001 Idiopathic limb edema in children with chronic arthritis: a multicenter report of 12 cases 1997 Feb Lymphedema, a well known extraarticular manifestation of rheumatoid arthritis, has been rarely described in children with idiopathic chronic arthritis. We describe 12 cases of lymphedema and idiopathic arthritis of childhood seen at 4 different pediatric rheumatology centers. Eight patients were girls, 4 boys; the age at appearance of lymphedema ranged from 2.3 to 17 years. In all patients except one, lymphedema was localized to the lower limbs. The outcome of lymphedema was variable, but not always related to the arthritis course, and was mostly independent of any specific therapy. Lymphography was performed in only one patient, and revealed lack of lymphatic drainage in the affected leg. We conclude that the association of lymphedema and idiopathic arthritis of childhood is not rare; this association is unlikely to be coincidental, even though the pathogenetic mechanisms are currently not well understood.
9108852 Infantile-onset multisystem inflammatory disease: a differential diagnosis of systemic juv 1997 Apr We describe four unrelated children with neonatal maculopapular rash, fever, arthritis, hepatosplenomegaly, lymphadenopathy, eye involvement, and neurologic symptoms. Radiographs of the joints were surprisingly similar, showing an abnormal epiphyseal and metaphyseal appearance. These clinical and radiologic findings allowed us to include these children in a very peculiar syndrome described as infantile-onset multisystemic inflammatory disease. A chondrosarcoma developed in one of our patients.
11580301 Management of hepatitis C virus-related arthritis. 2001 Hepatitis C virus (HCV) infection is often associated with extrahepatic manifestations among which arthropathy is common, affecting up to 20% of HCV-infected individuals. This arthropathy is to be distinguished from the more superficially prominent myalgias and fatigue. HCV-related arthritis is commonly presented as rheumatoid-like, symmetrical inflammatory polyarthritis involving mainly small joints, or, less commonly, as mono- or oligoarthritis, usually of the large joints. HCV arthritis usually runs a relatively benign course that, in contrast to 'true' rheumatoid arthritis (RA), is typically non-deforming and is not associated with articular bony erosions. In addition, unlike 'classic' RA, erythrocyte sedimentation rate is elevated only in about half of the patients and subcutaneous nodules are absent. In about two-thirds of the affected individuals morning stiffness may be severe, resolving after more than an hour. Several pathogenetic mechanisms may be involved: HCV arthritis may be part of the syndrome of mixed cryoglobulinaemia, or may be directly or indirectly mediated by HCV. Such possible, but yet not proven, mechanisms include direct invasion of synovial cells by the virus eliciting local inflammatory response, cytokine-induced disease or immune complex disease, particularly in genetically susceptible individuals. The diagnosis of HCV arthritis in patients with positive rheumatoid factor and chronic inflammatory polyarthritis may be difficult. Positive HCV antibody and HCV RNA, and the absence of bony erosions, subcutaneous nodules and antikeratin antibodies, may be useful in distinguishing between HCV-related arthritis and RA. The optimal treatment of HCV-related arthritis has not yet been established. Concerns may be raised regarding the use of immunosuppressive or potentially hepatotoxic drugs. However, it may be suggested that once the diagnosis of HCV-associated arthritis is made, combination antiviral treatment with interferon-alpha and ribavirin should be initiated as part of the therapeutic armamentarium. Low dose oral corticosteroids, nonsteroidal anti-inflammatory drugs, hydroxychloroquine or sulfasalazine in addition to the antiviral therapy can be used to control arthritis-related symptoms. Some patients may need long term anti-inflammatory treatment in various combinations, along with antiviral therapy. In patients with severe, disabling or life-threatening cryoglobulinaemia-related symptoms refractory to antiviral or anti-inflammatory treatment, high dose corticosteroids (including pulse therapy) and/or plasmapheresis may be needed.