ABSTRACT
PURPOSE: To describe an unusual manifestation of sarcoidosis as a large tumor of the iris and ciliary body without any other involvement of the body. METHODS: We describe a 20-year-old female presenting with a granulomatous tumor of the right iris and ciliary body and concomitant uveitis. RESULTS: Extensive ocular and systemic workup revealed the tumor to be a large solitary sarcoid granuloma. As systemic steroids were not able to control the activity of the uveitis and granuloma, only the initiation of immunosuppressive therapy with cyclosporine A achieved a lasting remission. CONCLUSION: The possibility of an exclusively ocular sarcoidosis should always be kept in mind despite negative regular screening tests. In these cases, a biopsy should be considered and immunosuppressive agents like cyclosporine A should be evaluated in cases not responding to first-line treatment with systemic steroids.
Subject(s)
Granuloma/diagnosis , Iris Diseases/diagnosis , Panuveitis/diagnosis , Sarcoidosis/diagnosis , Adult , Female , Glucocorticoids/therapeutic use , Granuloma/diagnostic imaging , Granuloma/drug therapy , Humans , Iris Diseases/diagnostic imaging , Iris Diseases/drug therapy , Methylprednisolone/therapeutic use , Panuveitis/drug therapy , Sarcoidosis/diagnostic imaging , Sarcoidosis/drug therapy , UltrasonographyABSTRACT
Thrombocytopenia is a common phenomenon in patients suffering from systemic lupus erythematosus (SLE). The cause of thrombocytopenia in SLE, however, is poorly understood. In this study, 100 patients with SLE were evaluated for serum thrombopoietin levels, anti-thrombopoietin antibodies and routine laboratory parameters such as peripheral blood counts, parameters of blood chemistry and immunologic parameters of SLE. The median platelet count of SLE patients was 230 g/l and 19 were thrombocytopenic (range 8-148 g/l). Thrombopoietin levels in SLE patients were found to be significantly higher than in healthy controls (n = 96; median, 117 pg/ml vs 64 pg/ml, P < 0.01). When excluding thrombocytopenic SLE patients, thrombopoietin levels in SLE were still above controls (111 pg/ml, P < 0.01). The thrombopoietin levels were correlated to erythrocyte sedimentation rate and ECLAM score of disease activity, and inversely correlated to complement factor C4, but not to the platelet count. Anti-thrombopoietin antibody reactivity was found in 23% of SLE patients. Interestingly, these patients had lower platelet counts than SLE patients without anti-thrombopoietin antibodies (median 174 g/l and 253 g/l, respectively, P < 0.01), but thrombopoietin levels were not significantly different. Taken together, thrombopoietin levels are significantly higher in the sera of SLE patients than in healthy controls and anti-thrombopoietin antibodies are frequently found.
Subject(s)
Lupus Erythematosus, Systemic/blood , Lupus Erythematosus, Systemic/immunology , Thrombopoietin/blood , Thrombopoietin/immunology , Adolescent , Adult , Aged , Female , Humans , Immunoenzyme Techniques , Male , Middle Aged , Platelet CountABSTRACT
OBJECTIVE: This study was performed to assess erythropoietin levels and anti-erythropoietin antibodies in patients with systemic lupus erythematosus (SLE). METHODS: The sera of 100 patients with SLE were investigated for serum erythropoietin levels and the presence of anti-erythropoietin antibodies by ELISA. Routine laboratory parameters such as peripheral blood count, relevant parameters of blood chemistry, and immunological parameters of SLE were recorded. RESULTS: Erythropoietin levels were significantly decreased in SLE patients when related to individual haemoglobin and haematocrit values (P<0.001), suggesting an inadequate erythropoietin response in SLE. Anti-erythropoietin antibodies were found in 46% of SLE patients, and erythropoietin levels (but not haemoglobin or haematocrit values) were significantly decreased in these patients compared with patients without anti-erythropoietin antibodies. Serum erythropoietin concentration as determined by ELISA was reduced in the presence of anti-erythropoietin antibodies. Furthermore, anti-erythropoietin antibodies also correlated with younger age, decreased serum levels of complement factors C3 and C4 and elevated anti-double-stranded DNA antibodies. CONCLUSIONS: We conclude that the anaemia of SLE is characterized by an inadequate erythropoietin response. Anti-erythropoietin antibodies are frequently present in SLE and interfere with the measurement of serum erythropoietin level. However, these antibodies are not associated with increased severity of SLE-associated anaemia.
