Biomedical subjects
A Deodhar
Publications and source records attributed to A Deodhar.
Miller Fisher syndrome in adult onset Still's disease: case report and review of the literature of other neurological manifestations.
Adult-onset Still's disease (AOSD) is a multi-system inflammatory disorder characterized by high spiking fevers, evanescent salmon-coloured rash, arthralgias or arthritis, hepatosplenomegaly, lymphadenopathy and sore throat. There is no specific test or combination of tests that can establish the diagnosis of AOSD and patients may present with other systemic involvement including neurological manifestations in 7-12% of cases. We present a complex case of a patient with AOSD who developed the Miller-Fisher variant of Guillain-Barré syndrome. This immunological disorder of the nervous system has not been described in association with AOSD before. We also review the literature on other neurological manifestations in AOSD. AOSD mimics different disease processes and its multi-system manifestations may complicate the picture further.
Henoch-Schönlein purpura: a possible complication of hepatitis C related liver cirrhosis.
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Neutrophil CD64 expression: distinguishing acute inflammatory autoimmune disease from systemic infections.
BACKGROUND: Common bacterial and opportunistic infections are a major cause of mortality in patients who are immunosuppressed owing to treatment with corticosteroids or cytotoxic drugs. Common laboratory tests for infection lack sensitivity and specificity. One of the new generation of tests to detect early systemic infections measures the up regulation of an Fc receptor (Fcgamma R1, or CD64) on neutrophils. The Fc receptors on white blood cells are very important for effective phagocytosis of bacteria and are up regulated during an infection. OBJECTIVE: To measure the clinical usefulness of quantitative CD64 measurements to differentiate between systemic infection and active autoimmune inflammation in an ongoing study. METHODS: Patients with systemic infection (n=27), active autoimmune inflammatory disease (n=44), vasculitis (n=5), and controls (n=20) were studied for neutrophil CD64 expression using monoclonal antibodies and flow cytometry. RESULTS: The median (interquartile range (IQR)) CD64 expression in patients with active inflammatory disease and systemic infection was 907.5 (586-1550) and 3647 (2380-6642), respectively (p<0.0001). The median (IQR) CD64 expression in control patients (osteoarthritis and fibromyalgia) was 505 (359-599). The sensitivity and specificity of CD64 expression on neutrophils to diagnose systemic infection (using a cut off value of 2000) was 85% and 91%, respectively. CONCLUSION: These results indicate that quantitative measurement of CD64 can distinguish between systemic infection and the flare of autoimmune diseases.
Treatment of lupus-induced thrombocytopenia with recombinant human interleukin-11.
Autoimmune thrombocytopenia can be a serious manifestation of systemic lupus erythematosus (SLE) which necessitates treatment with immunosuppressive agents and platelet transfusions. Interleukin-11 (IL-11) is a unique thrombopoietic growth factor which causes proliferation of megakaryocyte progenitors as well as induces megakaryocytic maturation. To our knowledge, this agent has not been used in the treatment of autoimmune thrombocytopenia, since theoretically there is a danger of IL-11 stimulating the immune system by up-regulating the lymphoid stem cells. We describe a 36-year-old splenectomized woman with known SLE who presented with pulmonary hemorrhage, acute renal failure, change in mental status, and severe thrombocytopenia (platelet count 2,000/mm3). Her pulmonary, renal, and central nervous system complications responded to intensive therapy with intravenous (IV) pulse methylprednisone and cyclophosphamide along with hemodialysis. The thrombocytopenia remained refractory to the above treatment plus daily multiple platelet transfusions and IV immunoglobulin. Treatment with recombinant human IL-11 (25 microg/kg/day subcutaneously) was initiated and continued for 5 days. Her platelet count improved to 25,000/mm3 within 48 hours, and she experienced no adverse effects.
Synovial neoformations and tumours.
An important group of soft tissue tumours and tumour-like lesions originates from the synovium of the joints, bursae and tendon sheaths. These include synovial chondromatosis, diffuse articular lipomatosis (lipoma arborescens), villonodular synovitis, synovial haemangioma, synovial chondroma and fibroma (intracapsular and peri-articular), primary chondrosarcoma originating from the synovium and synovial sarcoma. The main clinical symptoms of these tumours, such as pain, swelling, effusion and joint locking, are not specific, so the diagnosis can easily be missed in clinical practice. The most important clinical characteristics and the differential diagnostic clues for synovial tumours and tumour-like lesions are described in this chapter. In addition, the new results of genetic and histological studies are discussed, together with a summary of the available evidence-base for therapy.
The association of systemic lupus erythematosus and Lambert-Eaton myasthenic syndrome.
The association of systemic lupus erythematosus (SLE) and myasthenia gravis is well known. However, the association between SLE and Lambert-Eaton myasthenic syndrome (LEMS) is described in only 2 cases. We describe the case of a 31-year-old woman with SLE who presented with severe, predominantly proximal muscle weakness of subacute onset. The diagnosis of LEMS was based on her distinctive clinical and electrophysiological findings. She was treated successfully with plasmapheresis and oral prednisone. She remains in remission 6 months after this episode.
Arthritis as a complication of intravesical BCG vaccine.
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