Beneficial effect of etanercept on rheumatoid lymphedema.
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Biomedical subjects
Publications and source records attributed to B E Ostrov.
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Infectious diseases commonly cause illnesses that mimic rheumatic diseases. Both Lyme disease and Parvovirus B19 infections produce arthritis, rashes, and a systemic illness that may be thought to represent a chronic rheumatic disease. In the case presented, a child with both infections simultaneously exhibited arthralgias, aseptic meningitis, and a facial rash. The features of Lyme disease and Parvovirus B19 infection that may mimic systemic lupus erythematosus include a facial rash, often in a malar distribution, hematologic abnormalities, arthritis, neurologic disorders, and autoantibody positivity. Given the proper season and geographical location, one must consider the possibility of co-infection with these two organisms, especially in those with atypical rheumatic complaints.
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OBJECTIVE: To describe and review the autoimmune features and typical manifestations of Wiskott-Aldrich syndrome (WAS). DESIGN: Case series and review of the literature. SETTING: Tertiary care medical center and pediatric referral center. PATIENTS: The presentation, diagnosis, and management of two cases are reported. In addition to the typical features of WAS, the first patient had hemolytic anemia, arthritis, leukocytoclastic vasculitis, and colitis. The second patient had colitis and arthralgias. Detailed review of features and therapeutic options in WAS as exemplified by these two patients are presented. Both patients had bone marrow transplantation, the only definitive treatment for WAS. CONCLUSIONS: WAS has variable clinical and autoimmune manifestations. Diagnosis must be suspected in a boy with small, decreased number of platelets and autoimmune problems or infections. Bone marrow transplantation is the only successful mode of treatment for all aspects of WAS.
Juvenile rheumatoid arthritis (JRA) is an inflammatory condition that affects children under 16 years of age. The cases of 7 patients with isolated finger swelling as the initial manifestation of JRA are reviewed. In these children, the diagnosis was significantly delayed (t-test, p < .0076) for up to 14 months (mean, 8 months) when compared to a mean of 3.4 months in the JRA patients with a typical onset. Of the patients with the initial presentation of isolated digital swelling, +/7 (57%) had disease that became polyarticular, whereas only 15% of the patients with the initial presentation of large-joint disease experienced progression to polyarticular disease (Fisher's exact test, p < .0307). This presentation of JRA should be recognized so that appropriate management can be instituted promptly.
OBJECTIVE: To determine the prevalence and outcome of chronic uveitis in patients with juvenile rheumatoid arthritis (JRA). METHODS: A retrospective analysis of 760 patients with JRA followed in 4 pediatric rheumatology centers. Patients with chronic uveitis were identified and their medical and ophthalmologic records were reviewed. RESULTS: Seventy-four patients with uveitis were identified. The prevalence of uveitis was 9.3%. The mean interval from the onset of JRA to the onset of uveitis was 21 months, and 90% of the patients who developed uveitis did so within the first 4 years of their disease. Visual complications (synechiae, band keratopathy, cataract, or glaucoma) developed in 31% of the patients with uveitis. Complications were more common in patients who presented with uveitis early in the course of their JRA. Complications were also more common in antinuclear antibody (ANA) negative than in ANA positive patients. Visual loss to 20/50 or worse occurred in only 11% of patients with uveitis, and no patient became blind. CONCLUSION: In a very large cohort of patients with JRA, uveitis was uncommon and poor visual outcome was rare. Visual complications did not necessarily result in a poor outcome.
Cytophagic histiocytic panniculitis (CHP) can be a severe variant of Weber-Christian disease characterized by the histopathologic appearance of lobular panniculitis infiltrated by histiocytes containing blood cell fragments and by a clinical course with marked systemic features including multiorgan failure, hypertriglyceridemia, and coagulopathy, which may lead to death. Therapy of CHP includes standard treatment for panniculitis, such as antimalarials, plus immunosuppressives for more severe cases. The response to treatment, however, is unpredictable. In several recent reports, cyclosporine A has been successfully used to treat panniculitis. We report a patient and review the literature on CHP and the use of cyclosporine A as therapy. Published reports reveal that in instances of severe CHP when cyclosporine A was not given, 19 of 27 patients died (70% mortality). When severe CHP was treated with cyclosporine A, rapid remission was achieved in our patient and all five previously published cases (0% mortality). We believe cyclosporine A is the drug of choice in severe CHP and should be considered in all such patients.
Relapsing polychondritis (RP), while relatively rare, presents a characteristic clinical picture. Based upon a symptom complex of auricular, nasal, and respiratory chondritis associated with ocular and otic complaints, diagnosis can frequently be made with confidence in the absence of histologic confirmation. We present a case where a therapeutic intervention was required without sufficient criteria for diagnosis. Magnetic resonance imaging (MRI) proved useful in initial evaluation and allowed follow-up imaging demonstrating a gratifying response to steroid treatment. We believe that MRI has an important role in the diagnosis and subsequent evaluation of patients with proven or suspected RP, particularly those with tracheal involvement.
Diabetic muscle infarction (DMI) is a painful and potentially serious complication in patients with poorly controlled diabetes mellitus. The incidence of DMI is likely much greater than reports in the literature suggest, perhaps secondary to the difficulty in making the diagnosis and excluding other more serious etiologies. This paper describes the role of MRI in the evaluation of a diabetic patient with a painful, swollen limb. Early application of MRI can more accurately classify the disease process and focus the differential diagnosis, thus avoiding the hazards of medical therapy associated with other etiologies such as deep venous thrombosis, cellulitis, or osteomyelitis. This paper describes the evaluation and diagnostic pitfalls encountered in two patients. MRI techniques and applications are presented with a discussion of clinical and radiological differential diagnoses.
Several syndromes involving antiphospholipid antibodies have been described in the literature. Although the varied clinical manifestations have been well delineated, the vascular pathophysiology in patients with these antibodies remains unclear. Vascular damage is often described as a vasculopathy; however, several case reports have described an associated vasculitis. We report two patients with manifestations of antiphospholipid antibody syndrome (APLS) and concurrent vasculitis. The first patient, a 42-year-old man, presented with abdominal pain and fevers. The second patient, a 39-year-old man, presented with fever and testicular pain. Both were ultimately felt to have polyarteritis nodosa associated with APLS. Their complicated hospital courses and difficulties we encountered in diagnosing and treating them are discussed. The literature describing other cases of vasculitis associated with antiphospholipid antibody syndrome is reviewed. Whether the presence of antiphospholipid antibodies favors the development of vasculitis or vice versa is not clear. Further studies are needed to address this question and to determine optimal therapeutic regimens in these critically ill patients.
We describe five children with varied rheumatic manifestations, including fibromyalgia and arthralgias, ultimately proved to be associated with hypothyroidism. All musculoskeletal symptoms improved after thyroid replacement therapy. We conclude that rheumatic manifestations of hypothyroidism can be as varied in children as in adults.
Many children with acute leukemia have musculoskeletal complaints and non-specific hematologic abnormalities. A total of 10 children with acute leukemia and 10 with systemic juvenile rheumatoid arthritis were compared to delineate which early features could differentiate these diagnoses. Attention to evolving hematologic abnormalities and musculoskeletal findings may expedite diagnosis of these diseases.
Reflex sympathetic dystrophy (RSDS) is a syndrome of hyperesthesia and vasomotor disturbance of an extremity, often following trauma or an inflammatory process. We describe a patient with systemic lupus erythematosus (SLE) and recurrent RSDS involving all 4 extremities at various times during her illness. Treatments, including corticosteroid use, physical therapy and at times sympathetic nerve blockade led to the resolution of each episode. We suggest the diagnosis of RSDS be considered in patients with connective tissue diseases who present with atypical pain syndromes not attributable to their primary illness.
Musculoskeletal disorders may be the primary manifestations of scleroderma. The case is reported of a patient admitted for an evaluation of severe knee flexion contractures who was diagnosed as having scleroderma but lacked the typical presenting features of the disease. Radiographic, serological, and histological support for this diagnosis are presented. Scleroderma should be considered in the diagnostic evaluation of joint contractures.
Growth abnormalities are common in pediatric rheumatic diseases. Studies suggest that effects of inflammation such as anorexia, and adipose and muscle tissue breakdown contribute to the nutritional and growth aberrations. The effects of the various cytokines produced during inflammation create a vicious cycle of anorexia and increased catabolism, and may be responsible for the nutritional deficits seen. In future, specific treatment with anticytokine monoclonal antibodies may block these effects, minimizing the nutritional consequences of inflammation.