Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antiphospholipid syndrome (APS) is a disorder characterized by antiphospholipid antibody positivity, arterial or venous thrombosis, and fetal loss. In APS, renal as well as vascular and glomerular involvement is observed. Systemic lupus erythematosus and other connective tissue diseases should be excluded to diagnose primary APS. Immunoglobulin M (IgM) nephropathy is characterized by single or dominant IgM deposition in glomerular mesangium. It often presents with hematuria and proteinuria. In a 45-year-old female patient admitted to our clinic with diabetes mellitus and proteinuria, fundus examination did not reveal diabetic retinopathy but a high anticardiolipin IgM and venous thrombosis in the upper extremity were observed. Renal biopsy revealed IgM nephropathy. The patient was diagnosed with primary APS and IgM nephropathy. Cyclophosphamide and steroid treatment was started. Her proteinuria decreased as a result of the treatment. Although, it is reported in the literature that primary APS coexists with other glomerulonephritis, we did not detect coexistence of primary APS and IgM nephropathy.
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PMID:Upper extremity venous thrombosis associated with primary antiphospholipid syndrome and immunoglobulin M nephropathy in diabetes mellitus type II. 3018 66

The use of human intravenous immunoglobulins (IVIg) in systemic lupus erythematosus (SLE) currently relies on evidence from small case series and is mainly regarded as an off-label strategy in cases that are refractory to conventional therapies or poorly controlled with high doses of corticosteroids. Standard dosage regimens typically entail the administration of a total amount of 2 g/kg of IVIg divided into five consecutive days in order to minimize the risk of severe adverse events. We herein describe the case of a 28-year-old woman with a known history of antiphospholipid syndrome (APS) who was admitted to our hospital following fulminant onset of SLE in spite of ongoing immunosuppressive therapy. Acute renal insufficiency with nephrotic-range proteinuria, central nervous system involvement, severe thrombocytopenia, malar rash, pancreatic injury and moderate-severe aortic valve steno-insufficiency were the most prominent clinical manifestations, along with high titres of anti-dsDNA antibodies. Pulses of methyl-prednisolone followed by high-dose corticosteroids proved ineffective. Strikingly, IVIg therapy delivered at unconventional doses (1.2 g/kg) due to the presence of multiple risk factors for adverse events resulted in a significant, comprehensive clinical improvement. Although large-scale randomized double-blind studies are needed, the use of IVIg might constitute a valuable therapeutic modality as a last-resort strategy in cases of fulminant SLE. The total dose of immunoglobulins should be dictated by the clinical response as well as the presence of pre-existing risk factors for adverse events.
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PMID:Use of Intravenous Immunoglobulin Therapy at Unconventional Doses in Refractory Fulminant Systemic Lupus Erythematosus. 3075 66

A 42-year-old woman was referred from a primary care centre for severe hypertension, stage 3A chronic kidney disease and proteinuria. This was associated with a significant obstetric history of pre-eclampsia during her previous two pregnancies. Secondary hypertension was suspected and autoimmune workup was positive for anticardiolipin IgG and lupus anticoagulant. A renal biopsy showed evidence of chronic thrombotic microangiopathy, with electron microscopy features suggestive of fibrillar glomerulonephritis. The diagnosis of antiphospholipid syndrome with antiphospholipid-associated nephropathy was made. She was started on anticoagulation with warfarin, and her hypertension was controlled with lisinopril and amlodipine with subsequent improvement in proteinuria. She remains on regular follow-up to monitor for possible development of malignancy or connective tissue disease.
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PMID:Chronic thrombotic microangiopathy secondary to antiphospholipid syndrome, presenting with severe hypertension and chronic renal impairment. 3152 21


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