1/12. IgA myeloma presenting as Henoch-Schonlein purpura with nephritis.IgA nephropathy (IgAN) and Henoch-Schonlein purpura (HSP) are both characterized by IgA-mediated tissue injury, including mesangial proliferative glomerulonephritis. Abnormalities of IgA1 glycosylation are described in IgA nephropathy and HSP nephritis. IgA-antineutrophil cytoplasmic antibodies (ANCA) have been inconsistently described in the serum of patients with HSP. In IgA myeloma, the paraprotein-mediated renal lesion is typically cast nephropathy; IgAN or HSP have only rarely been reported in myeloma even when an IgA paraprotein is circulating in large concentrations. We report the case of a 50-year-old man with IgA myeloma who presented with HSP including nephritis and rapidly progressive renal failure. His IgA1 had altered O-glycosylation in the pattern seen in IgAN and also contained an IgA-ANCA. This case adds further weight to the evidence that IgA1 O-glycosylation abnormalities predispose to mesangial IgA deposition and also that IgA-ANCA may have a pathogenic role in the development of HSP.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
2/12. Cerebral vasculitis in Henoch-Schonlein purpura: a case report with sequential magnetic resonance imaging changes and treated with plasmapheresis alone.Neurological complications are rare during the course of Henoch-Schonlein purpura (HSP). We report a 7-year-old girl with HSP who presented with seizures, loss of vision and disturbance of consciousness. magnetic resonance imaging (MRI) showed high signal intensity in the gray and white matter over the left parietal and both occipital lobes, compatible with MRI findings of cerebral vasculitis. The eye fundi revealed multiple branches of retinal artery occlusion. Intravenous pulse methylprednisolone (MTP) followed by oral steroid therapy was initially administered for HSP nephritis. Cerebral vasculitis developed 10 days post-MTP treatment, with progressive worsening of consciousness. Oral steroid was discontinued and plasmapheresis was performed alone. Her level of consciousness dramatically improved after plasmapheresis. The brain MRI and eye fundi findings were consistent with her clinical improvement. To the best of our knowledge, this is the first description of MRI abnormalities and multiple retinal artery branch occlusion of cerebral vasculitis in a patient with HSP that was successfully treated by plasmapheresis alone. In conclusion, we propose that plasmapheresis may be used as a first-line therapy or rescue therapy for cerebral vasculitis in HSP.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
3/12. hypertension in Henoch-Schonlein purpura with minimal urinary findings.Though hypertension is a common feature of Henoch: Schonlein purpura (HSP) acute nephritis, it is seen only rarely and transiently if renal function is normal and abnormal urinary findings are minimal. We report a 3.4 year old girl who had the typical arthritis and rash of HSP, but also had significant hypertension with only minimal urinary findings. Imaging investigations revealed one normal kidney, and one that was almost destroyed from previous reflux nephropathy; her hypertension resolved after unilateral nephrectomy. Other causes of hypertension should be excluded in children with HSP and minimal urinary findings.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
4/12. Cerebral vasculitis and intracerebral hemorrhage in Henoch-Schonlein purpura treated with plasmapheresis.Severe neurological complications in Henoch-Schonlein purpura (HSP) are rare. We report a 13-year-old girl with HSP receiving oral steroid therapy for HSP nephritis who presented with acute onset of headache, confusion, and loss of bilateral vision. However, immediate cranial computed tomography showed merely a small intracranial hematoma in the left occipital area, which was not consistent with her clinical manifestations. The subsequent magnetic resonance imaging of the brain demonstrated bilateral, multifocal abnormalities with features consistent with cerebral vasculitis. Therapeutic plasmapheresis was performed immediately and there was rapid clinical improvement. In conclusion, we propose that plasmapheresis is a reliable and effective therapy and may be considered as the first-line treatment in this critical condition to prevent massive intracerebral hemorrhagic complication of this immune complex-mediated cerebral vasculitis leading to irreversible neurological sequelae or mortality.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
5/12. Successful treatment of progressive Henoch-Schonlein purpura nephritis with tonsillectomy and steroid pulse therapy.Henoch-Schonlein purpura (HSP) is a systemic disorder characterized by a leukocytoclastic vasculitis involving small vessels with the deposition of IgA immune complexes. The renal involvement is the major cause of morbidity and mortality in patients with HSP. We report here an adult patient with HSP nephritis (HSPN) accompanied by persistent proteinuria and progressive renal dysfunction despite conventional therapy. The patient was successfully treated with tonsillectomy followed by intravenous pulse methylprednisolone and oral prednisone. The combination therapy resulted in a significant decrease in proteinuria, improvement of renal function and the disappearance of microhematuria. The patient finally reached a stage of clinical remission.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
6/12. Streptococcal origin of a case of Henoch-Schoenlein purpura nephritis.We report the case of a 25-year-old man with abdominal pain, purpura on the legs and proteinuria occurring 2 weeks after acute tonsillitis, and admitted to our hospital with suspected Henoch-Schonlein purpura nephritis (HSPN). He had increased anti-streptolysin O (ASO) titer and hypocomplementemia. A renal biopsy specimen showed endocapillary proliferative changes, which are typical of acute poststreptococcal glomerulonephritis (APSGN). However, immunofluorescence study revealed predominant IgA and C3 deposits in mesangial lesions, indicating a diagnosis of HSPN. Because of massive proteinuria initially, the treatment with a combination of prednisolone, cyclophosphamide, dipyridamole and warfarin was started along with 3 plasma exchanges. Angiotensin-converting enzyme inhibitor was also given. Response to the treatment was favorable. A follow-up biopsy was performed 8 months after the first biopsy. The renal biopsy specimen showed a figure of typical HSPN. To further investigate the cause of glomerular changes in our patient, an immunofluorescent study of nephritogenic nephritis-associated plasmin receptor (NAPlr) of group A, beta-hemolytic streptococci was performed. NAPlr was significantly positive in the glomeruli in the first biopsy specimen, but not in the second. His clinical course and pathological findings suggest that NAPlr may be related to the pathogenesis in a part of patients with HSPN, especially in patients with high ASO titer and hypocomplementemia.- - - - - - - - - - ranking = 1.2keywords = purpura (Clic here for more details about this article) |
7/12. Zonisamide eradicated paroxysmal headache with EEG abnormalities triggered by hypertensive encephalopathy due to purpura nephritic syndrome.Generally, prognosis of hypertensive encephalopathy in childhood is favorable. We reported a 5-year-old girl who presented with a headache attack and EEG abnormalities after hypertensive encephalopathy due to purpura nephritis. The patient had suffered from hypertensive encephalopathy due to purpura nephritis, which soon ameliorated. Five months later, she developed attacks of headache, vomiting and disturbed consciousness with left side-predominant EEG abnormalities. Although carbamazepine and sodium valproate failed to improve her condition, zonisamide eradicated both the symptoms and EEG abnormalities, and an attack has not reoccurred for 5 years since completion of her treatment. It is noteworthy that delayed-onset complications can occur in child hypertensive encephalopathy, cases of which should be followed up prudently. Zonisamide should be considered for treatment of attacks of headaches with an epileptic character.- - - - - - - - - - ranking = 1.2keywords = purpura (Clic here for more details about this article) |
8/12. Berger disease: Henoch-Schonlein syndrome without the rash.Identical 7-year-old twin boys each had a proved adenovirus infection at the same time. A few days later one developed florid Henoch-Schonlein purpura, severe alimentary tract symptoms, and transient joint symptoms. He had an acute nephritic syndrome, which progressed to nephrotic syndrome and renal insufficiency. biopsy showed severe proliferative glomerulonephritis with crescents and marked deposition of IgA, IgG, C3, and fibrin. The second twin had hematuria and abdominal pain but no rash. biopsy showed mesangial proliferative glomerulonephritis with mesangial deposits of IgA and, to a lesser extent, IgG and C3. The appearance was characteristic of Berger disease, and the subsequent clinical course has been that of symptomless microscopic hematuria and recurrent macroscopic hematuria with normal renal function. Immunologic studies have not revealed why these identical twins responded differently to the same provocation. Perhaps Berger disease may be considered a variant of Henoch-Schonlein nephritis.- - - - - - - - - - ranking = 0.2keywords = purpura (Clic here for more details about this article) |
9/12. Henoch-Schonlein anaphylactoid purpura nephropathy: electron microscopic lesions mimicking acute poststreptococcal nephritis.The clinical, laboratory, and histologic features of a patient with Henoch-Schonlein syndrome are presented. The skin biopsy examination showed "leucocytoclastic vasculitis." kidney tissue demonstrated deposits of IgG, C3, and fibrinogenfibrin by fluorescence microscopy in the mesangium and the peripheral glomerular basement membrane in a granular-nodular pattern. These correlated well with areas of mesangial hyperplasia and polymorphonuclear leucocyte infiltration seen by light and electron microscopy. Several well delineated, variably sized, subepithelial electron dense deposits flanked by polymorphonuclear leucotytes adherent to the glomerular basement membrane were seen in two of the four glomeruli examined by electron microscopy. The significance of these findings is discussed. A detailed ultrastructural evaluation of patients with Henoch-Schonlein nephropathy may yield information about the frequency of subepithelial deposits and perhaps may help to clarify the pathogenesis of this syndrome.- - - - - - - - - - ranking = 0.8keywords = purpura (Clic here for more details about this article) |
10/12. Thrombotic thrombocytopenic purpura syndrome in systemic lupus erythematosus: treatment with plasma infusion.Two patients with well documented systemic lupus erythematosus developed a syndrome resembling thrombotic thrombocytopenic purpura. Both had severe thrombocytopenia, microangiopathic hemolytic anemia, seizures, and renal dysfunction. prothrombin time, partial thromboplastin time, thrombin time, and fibrinogen levels were normal; fibrin degradation products were minimally elevated. Histologic evaluation of renal biopsies in both patients confirmed the impression of intravascular thrombosis. Therapy with corticosteroids, other immunosuppressive drugs and splenectomy (in one case) proved unsuccessful. The infusion of fresh frozen plasma, with or without plasmapheresis, reversed the syndrome. This report indicates that patients with systemic lupus may develop a thrombotic thrombocytopenic purpura like syndrome which responds to fresh plasma infusion.- - - - - - - - - - ranking = 1.2keywords = purpura (Clic here for more details about this article) |
| | Next -> |