Long-term treatment of antiphospholipid syndrome-associated cerebral arterial thromboses with intravenous immunoglobulin: a case report.

We report a now 13-year-old male with trisomy 21, hypothyroidism, and insulin-dependent diabetes who developed acute hemiplegia due to the antiphospholipid antibody syndrome (APS) at age four. The risks of long-term anticoagulation were initially considered to be high; hence, he was treated with monthly infusions of intravenous immunoglobulin (IVIG) at 2 g/kg for 2 years and then every other month for 7 years. Antiphospholipid antibodies were no longer detectable within 6 months and have continued to be negative. There was no clinical deterioration or further changes on magnetic resonance arteriography over 7 years. IVIG may be an alternative therapeutic choice for children with APS who are not candidates for conventional anticoagulation.

Nonsteroidal anti-inflammatory intolerance. An anaphylactic reaction to tolmetin.

A case of an anaphylactic reaction to tolmetin sodium was proven by rechallenge. The nature of allergic-like reactions to nonsteroidal anti-inflammatory drugs is unclear. Caution is advised in treating a patient experiencing such a reaction.

Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporine A.

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.

Neonatal rheumatic disorders. View of the pediatrician.

Neonatal lupus is a unique clinical entity characterized primarily by cutaneous or cardiac injury. Dermatitis usually resolves without significant residual effects but heart block may be irreversible and life threatening during the neonatal period. SS-A/Ro and/or SS-B/La antibodies of maternal origin are present in the serum of the mother and affected infant and are markers for this syndrome. For many mothers breast feeding is the preferred choice for infant nutrition. With proper guidance, lactating mothers may safely use several antirheumatic medications such as ibuprofen, piroxicam, flurbiprofen, diclofenac, mefenamic acid, prednisone, sulfasalazine, and methotrexate.

Juvenile arthritis in Turner's syndrome: a multicenter study.

OBJECTIVE: Turner's syndrome (TS) is a disorder associated with characteristic defects in the X chromosome. Autoimmune conditions such as thyroiditis, inflammatory bowel diseases and diabetes have been described in association with TS. METHODS: We have studied the association between TS and juvenile arthritis (JA) by using a survey in which 28 pediatric rheumatology centers (15 in the USA, 10 in Europe, and 3 in Canada) participated. RESULTS: Eighteen cases of TS in a population of approximately 15,000 JRA patients have been found. Two different patterns of arthritis were present: polyarticular (7) and oligoarticular (11). Children with polyarticular disease had early onset, seronegative, progressively deforming arthritis and growth retardation. Those with oligoarticular arthritis had a benign course and were ANA+ (8/11). The oligoarticular children had varying karyotypes whereas almost all of the polyarthritic patients shared the same 45X0 karyotype (6/7). The light and electron microscopic studies of synovium performed in two patients showed chronic inflammation and hyperplasia of the synovial lining cells, vascular proliferation and infiltration with lymphocytes, plasma cells and mononuclear phagocytes. CONCLUSION: Juvenile arthritis is a new autoimmune condition association with Turner's syndrome. The prevalence seems to be at least six times greater than would be expected if the two conditions were only randomly associated. This is the first description of the synovium in Turner's syndrome; no differences from other forms of juvenile rheumatoid arthritis were found.

Lyme arthritis in children. An orthopaedic perspective.

The cases of forty-three children with clinical and serological evidence of Lyme arthritis that was diagnosed between August 1983 and July 1985 were evaluated. The mean length of follow-up was twenty months, with a range of five to thirty months. All of the children lived in or had visited an area where the disease was known to be endemic. Arthritis was the presenting feature in more than half of the children, and half of the children had initially consulted an orthopaedic surgeon, none of whom made the correct diagnosis. Only twenty patients had a history of erythema chronicum migrans, the characteristic rash that precedes the arthritis, and for only nineteen children was there any recollection of having been bitten by a tick. Three patients had Bell palsy and one had a popliteal cyst in conjunction with the arthritis. All of the patients had oligoarticular involvement. The knee was involved in all but two patients. Recurrent attacks of synovitis were common. Effusion was the only radiographic abnormality that was observed, and it was found in thirty-two patients. The sedimentation rate was elevated in thirty of thirty-six patients. Immunofluorescent serology for Lyme disease, which is sensitive and specific, was uniformly positive. Of thirty-three patients who were treated with oral administration of penicillin or tetracycline alone, thirty-one responded, while two patients who had recurrent attacks of the disease responded to parenteral administration of antibiotics. The remaining ten patients responded to combinations of orally and parenterally administered antibiotics. Longer follow-up is needed to further document the apparently low rate of relapse after antibiotic therapy in this young population.

Atherogenesis in swine fed several types of lipid-cholesterol diets.

Eighteen-month-old Nebraska strain minipigs were fed diets containing 2% cholesterol and 20% corn oil, lard, or coconut oil for 12 to 18 months. Concentrations of serum total lipid, total cholesterol, and total phospholipid increased 200 to 300% with each diet. Changes in serum concentrations of Sf 20+ and Sf 0-20 lipoproteins varied with diets fed. Serum concentration of high density lipoprotein was increased in all cases. Intima concentration of Sf 0-20 lipoprotein fraction was elevated by feeding the corn oil diet. There was no development of atherosclerosis as a result of feeding the corn oil-cholesterol diet, but there was an increase in atherosclerosis as a result of feeding the lard or coconut oil diet. There were no correlations between fatty acid patterns of several lipid fractions from serum and corresponding lipid fractions from aortic intima of corn oil fed animals.

Differentiation of systemic juvenile rheumatoid arthritis from acute leukemia near the onset of disease.

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.

Pyogenic arthritis caused by capnocytophaga gingivalis in an immunocompetent three-year-old male.

Capnocytophaga gingivalis is most often isolated as normal oral flora or with periodontal disease. This organism is also associated with sepsis usually in immunocompromised hosts. We identified pyogenic arthritis caused by C. gingivalis in a 3-year-old immunocompetent male, whose clinical course closely resembled monoarticular onset pauciarticular juvenile rheumatoid arthritis. This is the first report of C. gingivalis septic arthritis in the world literature, but there are increasing reports of infections with this carbon dioxide-loving organism at other sites in non-immunocompromised individuals. The subacute presentation of the monoarthritis with this organism of low virulence led to a long delay in diagnosis and treatment. Any monoarthritis must continue to raise concern about infection.

Recurrent reflex sympathetic dystrophy as a manifestation of systemic lupus erythematosus.

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.

Rheumatic symptoms associated with hypothyroidism in children.

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.

Measurement of health status in children with juvenile rheumatoid arthritis.

OBJECTIVE: To develop and validate a self- or parent-administered instrument for measuring functional status in children with juvenile rheumatoid arthritis (JRA). METHODS: We adapted the Stanford Health Assessment Questionnaire (HAQ) for use in children ages 1-19 years, by adding several new questions, such that for each functional area, there was at least 1 question relevant to children of all ages. The face validity of the instrument was evaluated by a group of 20 health professionals and parents of 22 healthy children. The questionnaire was then administered to parents of 72 JRA patients (mean age 9.1 years, onset type systemic in 16, polyarticular in 21, pauciarticular in 35). RESULTS: The instrument showed excellent internal reliability (Cronbach's alpha = 0.94), with a mean inter-item correlation of 0.6. The convergent validity was demonstrated by strong correlations of the Disability Index (average of scores on all functional areas) with Steinbrocker functional class (Kendall's tau b = 0.77, P < 0.0001), number of involved joints (Kendall's tau b = 0.67, P < 0.0001), and morning stiffness (Kendall's tau b = 0.54, P < 0.0001). Spearman's correlation coefficient between Disability Index scores from questionnaires administered to parents and those from questionnaires administered to older children (> 8 years) was 0.84 (n = 29; P < 0.001), showing that parents can accurately report for their children. The test-retest reliability, studied at a 2-week interval, revealed virtually identical Disability Index scores measured on the 2 occasions (0.96 versus 0.96; P > 0.9 by paired t-test; Spearman's correlation coefficient = 0.8, P < 0.002). CONCLUSION: The Childhood HAQ, which takes less than 10 minutes to complete, is a valid, reliable, and sensitive instrument for measuring functional status in children with JRA.

Clinical characteristics of antiphospholipid antibody syndrome in children.

OBJECTIVE: To evaluate the clinical features and outcome of antiphospholipid syndrome (APS) in children. STUDY DESIGN: Retrospective chart review of patients seen at the Children's Hospital of Philadelphia and Children's Seashore House Pediatric Rheumatology Center between 1988 and 1993. RESULTS: Nine patients with ages ranging from 8 months to 17 years are presented. Clinical features of five patients with primary APS, described in detail, were digital ischemia, stroke, chorea, Addison disease, and pulmonary vaso-occlusive disease. The four children with secondary APS had systemic lupus erythematosus. Clinical features of these patients include livedo reticularis, deep venous thrombosis, and pulmonary hypertension. Antiphospholipid titers, results of coagulation studies, and serologic findings did not predict outcome. CONCLUSION: APS in children has diverse clinical features similar to those in adults and should be considered in cases of unexplained vaso-occlusive disease.

Hypercalcemia during the resolution of calcinosis universalis in juvenile dermatomyositis.

Dystrophic calcification is seen in more than 50% of children with juvenile dermatomyositis and tends to resolve spontaneously in some patients. Calcinosis universalis is the least common type of calcification seen and rarely regresses. We describe a boy with juvenile dermatomyositis and calcinosis universalis who developed hypercalcemia during spontaneous regression of dystrophic calcification. The treatment and possible mechanisms of this complication are discussed.

Lymphedema associated with juvenile rheumatoid arthritis.

We describe 3 children with lymphedema associated with polyarticular juvenile rheumatoid arthritis (JRA). We review the occurrence of lymphedema in adult rheumatoid arthritis (RA) as well as other associations.

Cutaneous manifestations of cystic fibrosis: possible role of cryoglobulins.

A 13-year-old girl with cystic fibrosis (CF), severe lung disease, purpura on the lower extremities and with elevated serum mixed cryoglobulins is described. The possible relationship of the purpura in CF with cryoglobulinaemia is discussed.

Early onset sarcoidosis with aortitis--"juvenile systemic granulomatosis?".

A syndrome of granulomatous arthritis-uveitis-rash has been described in children. Since the clinical features of arthritis in this syndrome closely resemble those seen in early onset sarcoidosis, and since large vessel vasculitis has been seen in children with sarcoidosis, it is possible that early onset sarcoidosis and granulomatous arthritis-uveitis-rash syndrome are part of the same clinical spectrum. Clinical features of a child with sarcoidosis and Takayasu arteritis described in this report support this hypothesis.

Nuclear imaging and clinical features of childhood reflex neurovascular dystrophy: comparison with adults.

Reflex neurovascular dystrophy (RND) is less common in children than in adults, and differences in onset, clinical course, response to treatment, and degree of disability suggest a different pathogenesis. We have assessed the usefulness of nuclear imaging in 15 children with RND who were evaluated from March 1983 to September 1985. Abnormal findings on 3-phase bone scans were observed in 14 children, with diffusely decreased bone uptake at the symptomatic site being the most common observation. This contrasts sharply with previous reports of diffusely increased uptake in most adults with RND.