Rapid Responses to:
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Rapid Responses: Rapid Responses published:
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Malignant hypertension
- Mohammad A. Al-Jubouri (6 July 2007)
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Why resist antibiotics?
- Robert M Bernstein (8 July 2007)
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Reversible posterior leucoencephalopathy syndrome / Accelerated hypertension
- Marcela P. Vizcaychipi, Eduardo Svoren (8 July 2007)
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Prompt therapy is vital
- Prasanta Padhan (8 July 2007)
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PRES with accelerated hypertension
- Adam P Morton (9 July 2007)
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Key is regulated BP reduction
- Faisal Mohammad (9 July 2007)
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Progressive multifocal leucoencephalopathy possibly due to her underlying SLE!
- Kathir Yoganathan, Susannah Danino (10 July 2007)
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PREP?! Query cause
- DAVID GWYNFOR SAMUEL (11 July 2007)
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Hypertensive Encephalopathy with Background Collagen Disorder
- Chandra M. Jha, Oman (14 July 2007)
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Malignant hypertension with underlying disease
- Dr. Cristina Verdaguer (16 July 2007)
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Mohammad A. Al-Jubouri, Consultant Chemical Pathologist St. Helens & Knowsley Hospitals NHS Trust, Prescot, Merseyside L35 5DR
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1. Posterior leucoencephalopathy is an increasingly recognised complication of hypertensive encephalopathy due to malignant hypertension and eclampsia. 2. This case presented with a hypertensive emergency, therefore blood pressure should have been reduced immediately i.e within minutes-hours. 3. Investigation of a case of malignant hypertension, microangiopathic haemolysis, renal failure and encephalopathy should include: Autoantibody screen for collagen disease including ANCAs & anti-GBM antibodies, urinary free catecholamines, plasma renin/aldosterone, renal arteriography/isotopic renogram and renal biopsy. With normal prothrombin time and activated partial thromboplastin time, antiphospholipid syndrome and DIC are less likely. Malignant hypertension in this case could have been due to eclampsia (HELLP), collagen disease such as SLE, Goodpasture syndrome, renal artery or renal parenchymal pathology. Competing interests: None declared |
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Robert M Bernstein, Rheumatologist MRI,Manchester M13 9WL
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We shouldn't forget bacterial endocarditis amongst the causes of secondary TTP: echocardiogram/TOE, blood cultures --and antibiotics while we have them. Competing interests: None declared |
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Marcela P. Vizcaychipi, Senior Registrar in Anaesthesia Charing Cross Hospital, London. W6 8RF, Eduardo Svoren
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Our first impression after reading this fascinating case was accelerated hypertension leading to acute vascular damage resulting in end organ dysfuction ( retina, brain, kidney, liver). The cause of malignant hypertension may be Thrombocytopenic Thrombocytopenic Purpura (TTP) charachterised by thrombocytopenia, microangiopathic haemolytic anaemia, acute renal failure and reversible posterior leukoencephalopathy syndrome (RPLS). TTP may be accompanying vascular collagen diseases such as Systemic Lupus Erythematosus (SLE). Regarding the first question: what does leucoencephalopathy found on the patient's MRI indicate? These clear symmetric areas of hyperintensity involving the occipital lobes may be part of the reversible posterior leucoencephalopathy syndrome that has been reported in association with malignant hypertension, toxemia of pregnancy or immunosupresant drugs. The mechanisms of injury or pathophysiology is not well understood. There are few theories. One of them postulated that the brain autoregulation is overcomed by the sudden increased in blood pressure leading to arteriolar dilatation, capillary leakage and consequently extracellular oedema secondary to extrasudation of plasma. Another mechanisms postulated is ischaemia secondary to severe vasoconstriction that seems to be supported by angiography. Other authors postulate hypoalbuminemia and steroids as a couse of leucoencephalopathy. Regarding the second question: How quickly should you lower the patient's blood presure? This is a difficult one as we have two organs of interest to preserve function and the mechanism and urgency of control the blood pressure may differe between them. For instance to avoid further neurological damage the blood pressure should be controlled in hours, however, this may afect the perfusion pressure of the kidney aggravating the renal function. In consequence our goal would be to reduce the mean arterial pressure by approximately 25-30% over the first 24-48 hours by using Na Nitroprusiate infusion. The tittration of the infusion per hour will be according to invasive blood pressure monitoring, clinical signs and symptoms. Sodium and volume depletion may be severe, and volume expansion with isotonic solution may be considered. Finally, regarding the last question, What diagnostic tests would you do next? Laboratory tests: 1- 24-hour urine for proteins and creatinine 2- Eritrosedimentation rate (ESR) 3- CRP 4- Antinuclear antibody 5-Antidouble stranded DNA antibody 6-Anti-Sm (and anti RNP) antibody 7-Anticardiolipine antibody (IgM, IgG, IgA) 8- Antibeta2 glycoprotein 1 antibody 9-Lupus anticoagulant 10-DDimer 11-Dilute Russel Viper Venom Time (or lupus anticoagulant test) 12-C3-4 and CH50 13-Thyroid antibodies 14-Testing lipid basal 15-Hemocysteine 16- Fibrinogen 17- Coombs' test and VDRL 18- Renin level in plasma 19-cathecolamines in urine 20- Serial blood cultures Others tests: 1- Echocardiogram 2-Renal ultrasound and doppler of renal arteries if caucasian 3-Bone marrow biopsy 4- Renal biopsy (when the patient is stable and depending on the above results) Competing interests: None declared |
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Prasanta Padhan, Post Doctoral Fellow,Clinical Immunology and Rheumatology CMC,Vellore,632004,TN,India
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This patient has posterior leucoencephalopathy which is related to malignant hypertension and renal failure. It is reversible if treated promptly. Blood pressure should be reduced to normal as early as possible. Such a situation is likely in patient with collagen vascular disease like SLE. The possibility of lupus with catastrophic antiphospholipid syndrome or HUS is the likely etiology in this case. Competing interests: None declared |
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Adam P Morton, Physician Mater Hospital, South Brisbane
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The MRI reveals the typical pattern of changes seen with posterior reversible encephalopathy syndrome (PRES). This syndrome is characterised by visual changes, headache, seizures and altered sensorium. The MRI changes are thought to represent vasogenic oedema. PRES occurs with a diverse clinical conditions including preeclampsia, glomerulonephritis, systemic lupus erythematosus and antiphospholipid syndrome, thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome, as well as drug toxicity from agents such as cyclosporine, tacrolimus, cisplatin, and erythropoietin. Management should be directed towards control of blood pressure aiming for a mean arterial pressure of 105-125 mm Hg, control of seizures and discontinuation of provocative drugs. A case report suggested intravenous nitroglycerin may aggravate PRES, and intravenous nimodipine may be a useful agent on the basis of its effects in preventing vasospasm as well as neuroprotection. The patient's hypertension is chronic, and I suspect her renal dysfunction is also chronic given her hypocalcemia, hyperphosphatemia, severe compensated metabolic acidosis and anaemia (the degree of haemolysis on presentation on presentation seems mild based on the minimally elevated bilirubin). The features of microangiopathic haemolytic anaemia, thrombocytopenia, renal failure, hypertension and pulmonary oedema in this patient can be seen with any cause of accelerated hypertension. As the renal failure and thromocytopenia were severe at presentation and rapidly progressive haemolytic-uraemic syndrome (HUS) seems the most likely diagnosis, scleroderma renal crisis next most likely in view of the history of Raynauds and chronic hypertension, although an identical picture could be seen with catastrophic antiphospholipid syndrome, acute glomerulonephritis or acute hypertension due to renal artery stenosis, primary aldosteronism or phaeochromocytoma. The latter needs to be considered in view of pulmonary oedema which could be due to catecholamine cardiomyopathy. Investigations I would perform would include : 1. Urinalysis on a catheter specimen looking for casts, dysmorhic red blood cells which might suggest acute glomerulonephritis. 2. I would chase previous results for renal function. 3. Echocardiography to assess cardiac function - globally poor left ventricular systolic function should raise suspicion of phaeochromocytoma, left ventricular hypertrophy would confirm longstanding hypertension. 4. Serum haptoglobin should be undetectable in the setting of haemolysis, a Coombs test should be negative in non-immune mediated haemolysis. 5. Ultrasound of kidneys (small if chronic disease) with renal artery dopplers. 6. Autoantibody screen including anti-centromere and SCL-70 antibodies, anticardiolipin antibodies and lupus anticoagulant, and anti- neutrophil cytoplasmic antibodies. 7. Plasma metanephrines to exclude phaeochromocytoma. 8. ADAMTS13 levels with respect to HUS / TTP. 9. If the above were unhelpful renal biopsy may be illuminating. With respect to management : 1. This lady would be best managed in intensive care with an arterial line and Swan-Ganz monitoring. 2. Management of blood pressure must include an angiotensin- converting enzyme inhibitor given the possibility of scleroderma renal crisis . In addition I would use intravenous nimodipine, and avoid nitrates in view of the presence of PRES on MRI. I would lower the patients blood pressure gradually aiming for a mean arterial pressure of 105-125mm Hg. 3. The possibilities of HUS and catastrophic antiphospholipid syndrome mandate plasma exchange until these diagnoses are excluded or other diagnoses are established. 4. If phaeochromocytoma is a possibility I would use intravenous magnesium because of its specific benefits in phaeochromocytoma crisis / catecholamine cardiomyopathy. Magnesium levels would need to be monitored at least every 4 hours because of the potential for toxicity in the setting of renal failure. The earliest gestation at which preeclampsia has been reported is at 15 weeks. Significant renal dysfunction is a rare complication of severe preeclampsia. In 12 years as an obstetric physician at a tertiary referral obstetric hospital with approximately 3000 deliveries per year, I have seen only a handful of cases of renal failure with severe preeclampsia. I doubt that the patient had been pregnant recently. Her alkaline phosphatase was normal (usually elevated in pregnancy due to placental isoenzyme), and her serum beta-hCG would have been markedly elevated for a prolonged period given her renal dysfunction had she been pregnant recently. Competing interests: None declared |
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Faisal Mohammad, SHO - Medical Rotation Arrowe Park Hospital Wirral NHS Foundation Trust, CH49 5PE
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1) Posterior leukoencephalopathy syndrome affects the cerebral white matter. Oedematous lesions particularly involve the posterior parietal and occipital lobes. This condition is clinically characterised by headache,vomiting, seizures, visual disturbances and altered sensorium. Posterior leukoencephalopathy syndrome is often associated with an abrupt increase in blood pressure and is usually seen in patients with eclampsia, renal disease, and hypertensive encephalopathy. It is also seen in the patients treated with cytotoxic and immunosuppressive drugs such as cyclosporin, tacrolimus, and interferon alfa. 2) In this Hypertensive emergency with a BP of 240/127 and evidence of end organ damage i.e papilloedema,pulmonary oedema and encephalopathy immediate reduction of MAP by 20-25% in first 2 hours is necessary to prevent or minizine end organ damage (i.e hypertensive encephalopathy,Intracranial Haemorrhage,Unstable angina,Acute MI,Acute left ventricular failure with pulmonary Odema,Dissection of aorta or renal failure). Then SBP can be reduced to 160 and diastolic to 100 over next 4- 6 hours.Quick reduction of BP to normal can be catastrophic. 3) Investigate for underlying cause by requesting Autoantibody screen and especially looking for ANA,Anti dsDNA and Anti Sm for SLE, ANCA, Anti SCL-70 and Anti Centromere, Anti cardilolipin and Lupus anti coagulant, Anti GBM, ADAMST 13 for TTP Ref: R K Garg Posterior leukoencephalopathy syndrome ,Postgrad Med J 2001;77:24 ( January ) Competing interests: None declared |
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Kathir Yoganathan, Consultant Physician Swansea NHS Trust,Singleton Hospital,Swansea,SA2 8QA, Susannah Danino
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MRI scans shows multiple white matter lesions without any evidence of surrounding oedema or mass effect i.e. no midline shift. These appearances are typical of a progressive multifocal leucoencephalopathy (PML), commonly seen in patients with advanced HIV infection. However PML has been reported in other non - HIV conditions such as patients with SLE, lympho- and myeloproliferative diseases, sarcoidosis, chronic granulomatosis, transplant patients and Whipple’s disease. In her case, history of suspected miscarriage, renal involvement, seizures and possible pulmonary haemorrhages are all suggestive of SLE. Diagnostic tests include HIV test and CSF PCR for JC virus. A negative JC virus in the CSF will not exclude PML as its sheds intermittently in to the CSF, another reason for negative JC PCR is deep seated lesions of PML. So repeated CSF examination is important. A stereotactic brain biopsy will provide a definitive diagnosis. Histopathological hallmarks of PML are triad of multifocal demyelination, enlarged oligodendroglial nuclei and enlarged bizarre astrocytes. However brain biopsy carries a significant morbidity and mortality. Therefore typical radiological appearances and a positive JC virus suffice to make a diagnosis of PML in these conditions. Competing interests: None declared |
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DAVID GWYNFOR SAMUEL, 4TH YEAR MEDICAL STUDENT CF48 2AS
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1. Posterior leucoencephalopathy on scan indicates oedema casued by rasied inta-cranial pressure. The probable diagnosis is POSTERIOR REVERSIBLE CEREBRAL OEDEMA SYNDROME (PREP) which is recognsied to be caused by raised BP. It is also associated with drug therapies including Hypertensive drugs, Immunsopression drugs and Chemotherapy. Cocaine has also been linked to several cases and there are reports in the literature of cases being linked to meningitis. ECLAMPSIA AND PRE-ECLAMPSIA are recongnised underlying conditions as is HUS. Presenting compaints fit with the history and include: - Headache; altered mental state; Coma and convulsions; Papilloedema; Elevated BP 2. Lowering blodd pressure: AIM to reduce blood pressure to ~110mmHg over 4 hrs. Use an intra-arterial line to monitor the pressure changes. A 25% reduction in MAP over 24 hrs is also recognised as appropriate to limit CVA risks. The use of i.v Frusemide with either i.v.Sodium Nitroprusside or i.v Labetalol should be used in the first instance. GRADUAL decrease is crucial as cerebral autoregulation is lost 3. Diagnostic test carried out should include a sepsis screen to rule out meningitis. Lumbar puncture would be contra-indicated if sigans of rasied ICP still present so reducing the BP must be number 1 priority. An antibody screen including ANA; pANCA would be jusitified as scleroderma, Glomerulonephritis and SLE have been shown as casues of malignant hypertensive crises. A renal biopsy would be extremelly helpful in recognising any glomerulnephritis condition. Would a screen for a PHAEOCHROMOCYTOMA be warranted in a young hypertensive??? Cathecholamines could be measured in a 24hr urine collection and an USS abdomen/ CT scan perfomed. Competing interests: None declared |
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Chandra M. Jha, Incharge Nephrologist Rusatq Regional Hospital, Rustaq., Oman
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1. Posterior leucoenchalopathy in this lady most likely indicates hypertensive encephalopathy1,2. Observed MAHA could be due to SLE, HUS, TTP or scleroderma. An associated immunocompromised state should be searched for1. Noted hypertension, history suggestive of Raynaud's syndrome and suspected miscarriage favours diagnosis of SLE, probably with anticardiolipin or antiphospholipid antibody positivity. One may argue against it because of successful first three pregnancies. SLE and "multiparity" are common in "Middle-East", and, we have encountered cases of SLE diagnosed in third to fifth pregnancies with uneventful or successful initial pregnancies. One may also speculate development of scleroderma due to microchimerism after initial three pregnancies3. 2. A 20 to 25% reduction in MAP should be targeted over a few hours. Many authorities advise it over 2 hrs. Lowe target should be achieved gradually over days with a watch over target-organ function. Among the target organs, our priority should be for central nervous system than for kidneys. 3. Auto-antibody profile targeted for ANAs, dsDNA, ENA, ScL-70, anticardiolipin antibody titer and dilute Russel's viper venom test (DRVVT). A kidney biopsy is warranted but contraindicated because of thrombocytopenia. References: 1. Judy Hinchey, Claudia Chaves, Barbara Appignani et al. A Reversible Posterior Leucoenchalopathy Syndrome. N Engl J Med 1996;334:494 -500. 2. Carl J Vaughan, Norman Delanty. Hypertensive emergencies. Lancet 2000; 356: 411–17. 3. J Lee Nelson, Daniel E Furst, Sean Maloney et al. Microchimerism and HLA-compatible relationships of pregnancy in scleroderma. The Lancet 1998; 351:559-562. Competing interests: None declared |
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Dr. Cristina Verdaguer, Nephroligist Private Hospital Uruguay
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1 What does posterior leucoencephalopathy found on the patient's
magnetic resonance imaging scan indicate?
2 How quickly should you lower the patient's blood pressure?
3 What diagnostic tests would you do next?
We should do test to rule out, lupus, antiphospholipidic syndrome,
vascultis.
We should do an echocardiogram to test cardiac function and if there is
repercusion of her chronic hypertension
Competing interests: None declared |
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