Showing papers in "Case Reports in 2018"
TL;DR: A young female vaper presented with insidious onset cough, progressive dyspnoea on exertion, fever, night sweats and was in respiratory failure when admitted to hospital and was suggestive of lipoid pneumonia.
Abstract: A young female vaper presented with insidious onset cough, progressive dyspnoea on exertion, fever, night sweats and was in respiratory failure when admitted to hospital. Clinical examination was unremarkable. Haematological tests revealed only thrombocytopenia, which was long standing, and her biochemical and inflammatory markers were normal. Chest radiograph and high-resolution CT showed diffuse ground-glass infiltrates with reticulation. She was initially treated with empirical steroids and there was improvement in her oxygenation, which facilitated further tests. Since the bronchoscopy and high-volume lavage was unyielding, a video-assisted thoracoscopicsurgical biopsy was done later and was suggestive of lipoid pneumonia. The only source of lipid was the vegetable glycerine found in e-cigarette (EC). Despite our advice to quit vaping, she continued to use EC with different flavours and there is not much improvement in her clinical and spirometric parameters.
116 citations
TL;DR: The effectiveness of therapeutic fasting to reverse their insulin resistance is demonstrated, resulting in cessation of insulin therapy while maintaining control of their blood sugars.
Abstract: This case series documents three patients referred to the Intensive Dietary Management clinic in Toronto, Canada, for insulin-dependent type 2 diabetes. It demonstrates the effectiveness of therapeutic fasting to reverse their insulin resistance, resulting in cessation of insulin therapy while maintaining control of their blood sugars. In addition, these patients were also able to lose significant amounts of body weight, reduce their waist circumference and also reduce their glycated haemoglobin level.
80 citations
TL;DR: A 15-year-old girl of Asian origin, hailing from a rural agrarian background, presented with history of multiple episodes of dizziness for 3 years, which revealed that the premature demise of the patient’s siblings which were attributed to snakebite and head trauma by the family could have been in reality sudden cardiac deaths.
Abstract: A 15-year-old girl of Asian origin, hailing from a rural agrarian background, presented with history of multiple episodes of dizziness for 3 years. The episodes were precipitated mostly by emotional and/or physical stress and relieved on lying down, with a few episodes culminating in transient loss of consciousness. As preliminary cardiac and neurological evaluation were normal, she was being treated by the primary physician as a case of probable psychogenic syncope, supported by the consistent association of the episodes with emotional stress. A detailed review of family history revealed that the premature demise of the patient’s siblings which were attributed to snakebite and head trauma by the family could have been in reality sudden cardiac deaths. Treadmill test revealed exercise-induced polymorphic ventricular tachycardia confirmatory for the diagnosis of catecholaminergic polymorphic ventricular tachycardia. She was initiated on beta-blocker therapy to which she showed remarkable response.
77 citations
TL;DR: In this article, a case of a patient with Crohn's disease and chronic iron-deficiency anaemia receiving multiple doses of FCM who developed severe hypophosphataemic osteomalacia with urinary phosphate loss and increased FGF23.
Abstract: Ferric carboxymaltose (FCM) is a novel iron formulation increasingly prescribed due to its effectiveness and fast infusion time. FCM administration can cause an asymptomatic hypophosphataemia secondary to fibroblast growth factor 23 (FGF23) dysregulation. In patients with chronic iron needs, however, a severe, long-lasting hypophosphataemia can lead to osteomalacia with associated bone pain. Lack of awareness of this complication results in delayed time to diagnosis and significant morbidity. We report a case of a patient with Crohn’s disease and chronic iron-deficiency anaemia receiving multiple doses of FCM who developed severe hypophosphataemic osteomalacia with urinary phosphate loss and increased FGF23. FGF23 excess and osteomalacia resolved only months after FCM discontinuation and aggressive phosphate repletion. Potential mechanisms of FGF23 dysregulation are discussed, with the aim of raising awareness of this significant side effect for prescribers of chronic intravenous iron supplementation, and to help guide future studies to determine the safety of FCM in all patient populations.
38 citations
TL;DR: The case of a 30-year-old man who presented with clinical features of G6PD deficiency-associated haemolysis and was found to have severe methaemoglobinaemia and was administered blood transfusions and intravenous ascorbic acid demonstrates the successful management of a patient with severe met Haemoglobin in the setting of G 6PD deficiency haenolysis.
Abstract: Methaemoglobin is a form of haemoglobin in which the ferrous (Fe2+) ion contained in the iron–porphyrin complex of haem is oxidised to its ferric (Fe3+) state. Methaemoglobinaemia, the presence of methaemoglobin in the blood, is most commonly treated with methylene blue. However, methylene blue cannot be used in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency as it is ineffective in such patients and it can worsen G6PD deficiency haemolysis. We report the case of a 30-year-old man who presented with clinical features of G6PD deficiency-associated haemolysis and was found to have severe methaemoglobinaemia (35%). He was administered blood transfusions and intravenous ascorbic acid. His methaemoglobinaemia resolved within 24 hours. This case demonstrates the successful management of a patient with severe methaemoglobinaemia in the setting of G6PD deficiency haemolysis. Emergency physicians should be aware of the possible co-occurrence of severe methaemoglobinaemia in a patient with G6PD deficiency haemolysis.
35 citations
TL;DR: A programme of weekly open (cold) water swimming led to an immediate improvement in mood following each swim and a sustained and gradual reduction in symptoms of depression, and consequently a reduction in, and then cessation of, medication.
Abstract: A 24-year-old woman with symptoms of major depressive disorder and anxiety had been treated for the condition since the age of 17. Symptoms were resistant to fluoxetine and then citalopram. Following the birth of her daughter, she wanted to be medication-free and symptom-free. A programme of weekly open (cold) water swimming was trialled. This led to an immediate improvement in mood following each swim and a sustained and gradual reduction in symptoms of depression, and consequently a reduction in, and then cessation of, medication. On follow-up a year later, she remains medication-free.
33 citations
TL;DR: It is reported here on a case of autoimmune hepatitis, occurring in a 71-year-old woman taking turmeric dietary supplements for the maintenance of cardiovascular health, which resolved rapidly following discontinuation of the turmeric supplements.
Abstract: Turmeric dietary supplement sales, which accounted for US$69 million in spending in 2016, have been increasing exponentially in the USA, making this one of the most popular botanical supplements sold in the USA. Herbal supplement use, which is generally regarded as safe by consumers, is not usually reported to healthcare providers. We reported here on a case of autoimmune hepatitis, occurring in a 71-year-old woman taking turmeric dietary supplements for the maintenance of cardiovascular health, which resolved rapidly following discontinuation of the turmeric supplements. Of particular note, turmeric use was not documented in the patient’s medical records and the potential causative role of the turmeric supplementation was ultimately identified by the patient rather than the healthcare providers. To our knowledge, this is the first documented report of turmeric supplement-induced autoimmune hepatitis.
31 citations
TL;DR: A patient with severe postural orthostatic tachycardia syndrome (POTS) and mast cell activation syndrome (MCAS) received immunotherapy with low-dose naltrexone (LDN) and intravenous immunoglobulin (IVIg) and antibiotic therapy for small intestinal bacterial overgrowth (SIBO).
Abstract: A patient with severe postural orthostatic tachycardia syndrome (POTS) and mast cell activation syndrome (MCAS) received immunotherapy with low-dose naltrexone (LDN) and intravenous immunoglobulin (IVIg) and antibiotic therapy for small intestinal bacterial overgrowth (SIBO). A dramatic and sustained response was documented. The utility of IVIg in autoimmune neuromuscular diseases has been published, but clinical experience with POTS is relatively unknown and has not been reported in MCAS. As a short-acting mu-opioid antagonist, LDN paradoxically increases endorphins which then bind to regulatory T cells which regulate T-lymphocyte and B-lymphocyte production and this reduces cytokine and antibody production. IVIg is emerging as a promising therapy for POTS. Diagnosis and treatment of SIBO in POTS is a new concept and appears to play an important role.
28 citations
TL;DR: The case of a patient with metastatic non-small-cell lung cancer treated with pembrolizumab, who demonstrated a marked clinical response with resolution of congestive heart failure symptoms, highlights that programmed death receptor-1 inhibition can cause a spectrum of autoimmune adverse events requiring clinical monitoring and periodic screenings.
Abstract: Pembrolizumab is an immune checkpoint inhibitor that significantly improves clinical outcomes in numerous solid organ malignancies. Despite successful therapeutic responses, this new drug comes with a constellation of adverse reactions. Herein, we chronicle the case of a patient with metastatic non-small-cell lung cancer treated with pembrolizumab. After two cycles, he developed new-onset dyspnoea on exertion. Electrocardiogram showed idioventricular rhythm with diffuse ST-segment elevations. Echocardiography revealed severe biventricular cardiac dysfunction. Based on diagnostic workup and exclusion of probable aetiologies, the patient was diagnosed with pembrolizumab-induced myocarditis. The treatment was initiated with corticosteroids and guideline-conform heart failure therapy. He demonstrated a marked clinical response with resolution of congestive heart failure symptoms. This article summarises the clinical evidence regarding the epidemiology, pathophysiology, clinical features, diagnostic modalities and management of patients with pembrolizumab-associated myocarditis. In addition, it highlights that programmed death receptor-1 inhibition can cause a spectrum of autoimmune adverse events requiring clinical monitoring and periodic screenings.
25 citations
TL;DR: Infiltration using fluid resin proved to be a valid micro-invasive alternative compared with traditional conservative therapy and allowed treatment without any trauma of numerous lesions in only one session with stable clinical and aesthetic results over the time.
Abstract: White spot lesions are defined as enamel surface and sub surface demineralisation, although these lesions can be reversed and do not form cavities. Infiltration using fluid resin proved to be a valid micro-invasive alternative compared with traditional conservative therapy. This has allowed treatment without any trauma (no use of local anaesthesia and cavity preparation) of numerous lesions in only one session with stable clinical (mechanical stability, enamel hardness, conservation of sound tissue) and aesthetic results (disappearance of lesions) over the time. The present report evaluates the effectiveness of Icon infiltration resin on postorthodontic white spots at 6 months, 1 and 4 years.
22 citations
TL;DR: A case of a 36-year-old active duty service member presenting with progressive dyspnoea, substernal chest pain and lower extremity swelling 5 weeks after receiving the vaccinia vaccination was reported, and the aetiology of his acute decompensated heart failure was determined to be from myocarditis.
Abstract: The development of vaccines ushered in the most profound advancement in 20th century medicine, and have widely been regarded as the one of the most important scientific discovery in the history of mankind. However, vaccines are not without risk; reactions can range from injection site reactions to life-threatening anaphylaxis. Among the more serious vaccine-related sequela is myocarditis. Although myocarditis has been reported following many different vaccines, the smallpox vaccine has the strongest association. We report a case of a 36-year-old active duty service member presenting with progressive dyspnoea, substernal chest pain and lower extremity swelling 5 weeks after receiving the vaccinia vaccination. The aetiology of his acute decompensated heart failure was determined to be from myocarditis. Although the majority of cases of myocarditis resolve completely, some patients develop chronic heart failure and even death. Vaccine-associated myocarditis should always be on the differential for patients that exhibit cardiopulmonary symptoms after recent vaccinations.
TL;DR: A case of a life-threatening diabetic emergency 25 days after initiation of nivolumab for stage 4 lung adenocarcinoma, with hyperglycaemia-related signs and symptoms, which assumed the diagnosis of new onset immune-mediated type 1 diabetes mellitus.
Abstract: The authors describe a case of a life-threatening diabetic emergency 25 days after initiation of nivolumab (3 mg/kg) for stage 4 lung adenocarcinoma. She was admitted to the emergency department, with hyperglycaemia-related signs and symptoms, such as polyuria, polydipsia, weight loss, confusion, asthenia, dehydration, hypotension and Kussmaul respiratory pattern. Her body mass index was 21.9 kg/m2 and she did not show acanthosis nigricans. Arterial blood gas determination revealed high anion gap metabolic acidaemia and blood tests showed hyperglycaemia (1060 mg/dL), hyperketonaemia (beta-hydroxybutyrate: 6.6 mmol/dL), elevated total serum osmolality (389 mOsm/kg), low serum and urinary C-peptide and positive antiglutamic acid decarboxylase antibodies. Since nivolumab was initiated a few days before, and due to its known immune-mediated endocrine adverse events, we assumed the diagnosis of new onset immune-mediated type 1 diabetes mellitus. After prompt and adequate treatment of diabetic ketoacidosis/hyperosmolar hyperglycaemic state, she was discharged improved on multiple daily injections of insulin.
TL;DR: A case of hydralazine-associated rapidly progressive glomerulonephritis and pulmonary haemorrhage is described, to review the current literature and highlight the importance of a high degree of clinical acumen, early diagnosis and prompt treatment for better clinical outcomes.
Abstract: Hydralazine, a vasodilator, is commonly used as an adjunctive treatment for moderate to severe hypertension, heart failure and hypertensive emergencies in pregnancy. Hydralazine-induced lupus was first described in 1953. Clinical presentation ranges from arthralgia, myalgia, petechiae, or rash to single or multiorgan involvement. An occurrence of systemic vasculitis is a rare complication. When presented as the pulmonary–renal syndrome, it could have a rapidly progressive course which can be fatal. Here, we describe a case of hydralazine-associated rapidly progressive glomerulonephritis and pulmonary haemorrhage. We use this case to review the current literature and discuss and highlight the importance of a high degree of clinical acumen, early diagnosis and prompt treatment for better clinical outcomes.
TL;DR: In vitro studies indicate Bruton tyrosine kinase inhibition impairs phagocyte function and offer a mechanism for the apparent association between ibrutinib and invasive fungal infections.
Abstract: We report a case of Cryptococcus neoformans pulmonary infection complicated by empyema in a 79-year-old man with diffuse large B-cell lymphoma treated with R-CHOP and ibrutinib. A literature review identified 25 cases of cryptococcal pleural disease published since 1980. Most cases were caused by the C. neoformans species in immunocompromised hosts with an exudative pleural effusion and lymphocyte-predominant infiltrate. The cryptococcal antigen test was often positive when pleural fluid and serum were tested. The outcome was favourable in most cases with antifungal therapy and either thoracocentesis or surgical resection. We also identified 40 cases of opportunistic infections, most commonly aspergillosis, cryptococcosis and Pneumocystis jirovecii pneumonia, in patients treated with ibrutinib. In vitro studies indicate Bruton tyrosine kinase inhibition impairs phagocyte function and offer a mechanism for the apparent association between ibrutinib and invasive fungal infections.
TL;DR: In the literature review, five patients with DIHS/DRESS were found to develop autoimmune sequelae consistent with PASIII and four out of the five patients were treated with intravenous immunoglobulin or pulsed prednisolone in the acute stage, although effective in short-term outcomes.
Abstract: We experienced a 6-year-old case of drug-induced hypersensitivity syndrome (DiHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) with subsequent development autoimmune thyroiditis (Hashimoto’s thyroiditis), type 1 diabetes with antithyroglobulin, thyroid peroxidase, insulinoma-associated antigen and anti-insulin antibodies at 4 months, alopecia at 7 months, vitiligo, uveitis due to Vogt-Koyanagi-Harada disease at 8 months after clinical resolution of the DiHS/DRESS. He was diagnosed as type III polyglandular autoimmune syndrome (PASIII) after DiHS/DRESS. Prompted by this case, we sought to determine which triggering factors were responsible for later development of PASIII in previously published cases with autoimmune sequelae. In the literature review, five patients with DIHS/DRESS were found to develop autoimmune sequelae consistent with PASIII. All cases with PASIII were much younger than those without them. Four out of the five patients were treated with intravenous immunoglobulin or pulsed prednisolone in the acute stage, although effective in short-term outcomes.
TL;DR: An unusual case of severe rhabdomyolysis with acute renal failure due to influenza A infection that improved with high-dose oseltamivir and intravenous fluids and noticed a temporal relation between fever spikes and subsequent increase in serum creatine kinase.
Abstract: Rhabdomyolysis is characterised by muscle breakdown with release of damaging proteins that can have devastating consequences. Acute influenza infection is being increasingly recognised as an underlying aetiology. We report an unusual case of severe rhabdomyolysis with acute renal failure due to influenza A infection that improved with high-dose oseltamivir and intravenous fluids. In our case, we also noticed a temporal relation between fever spikes and subsequent increase in serum creatine kinase. The precise mechanism between the rise in temperature and creatine kinase is unclear but it could be due to direct viral invasion of myocytes or due to release of new viral progeny following replication in the myocyte.
TL;DR: Two patients with NSCLC who developed severe pneumonitis following therapy with nivolumab and pembrolizumab are presented and it is illustrated that severe cases can often present refractory to steroid therapy.
Abstract: Anti-programmed death 1 (PD-1) immune checkpoint inhibitors enhance the antitumour activity of the immune system and have produced durable tumour responses in several solid tumours including non-small cell lung cancer (NSCLC). However, PD-1 inhibitors can lead to immune-related adverse events , including pneumonitis, which is typically mild, but can be severe and potentially fatal. Pneumonitis often resolves with steroids, but some cases are steroid refractory, leading to a relapsing and remitting course in milder cases or the need for salvage therapies in more severe cases. Here, we present two patients with NSCLC who developed severe pneumonitis following therapy with nivolumab and pembrolizumab. While one patient improved with steroids and infliximab, the other patient failed to respond to steroids and subsequently died. These cases demonstrate the highly variable presentation and therapeutic responses seen in patients with pneumonitis following anti-PD-1 therapy and illustrate that severe cases can often present refractory to steroid therapy.
TL;DR: A 75-year-old Hispanic man treated for septic shock after undergoing surgery for impacted renal stones was given vasopressors and later developed symmetrical peripheral gangrene (SPG), a serious and rare condition presenting clinically as an acute onset of ischaemia with no vessel occlusion.
Abstract: We report a case of a 75-year-old Hispanic man treated for septic shock after undergoing surgery for impacted renal stones. He was given vasopressors and later developed symmetrical peripheral gangrene (SPG) on both his feet and left hand. SPG is a serious and rare condition presenting clinically as an acute onset of ischaemia with no vessel occlusion. Vasopressors are identified as a contributing factor in SPG development. The patient ultimately underwent transmetatarsal amputations of both feet and amputation of three digits on his left hand. Early monitoring and swift management of peripheral ischaemia are essential when using vasopressors for the treatment of septic shock.
TL;DR: The successful treatment of fulminant neonatal enteroviral myocarditis in a pair of monochorionic diamniotic twins with cardiopulmonary support, intravenous immunoglobulin and pocapavir, anEnteroviral capsid inhibitor is presented.
Abstract: Neonatal cardiogenic shock most commonly occurs due to critical congenital heart disease, sepsis, metabolic disorder or arrhythmias. In particular, enterovirus infections are common in the neonatal period, and patients can present with fulminant myocarditis. Early recognition is imperative due to its high morbidity and mortality without prompt and aggressive treatment. We present the successful treatment of fulminant neonatal enteroviral myocarditis in a pair of monochorionic diamniotic twins with cardiopulmonary support, intravenous immunoglobulin and pocapavir, an enteroviral capsid inhibitor. The twins took an almost exact parallel hospital course, including day of extracorporeal membrane oxygenation (ECMO) cannulation, day of ECMO decannulation, improvement of cardiac function, discharge and status at follow-up. While it was difficult to assess the relative contribution of each intervention, our case shows promise in the use of pocapavir for treatment of severe enteroviral infections. Remarkably, both twins demonstrated remarkable recovery within 2 weeks, underscoring that early aggressive cardiopulmonary support, and potentially pocapavir, contributed to their recovery.
TL;DR: A healthy 32-year-old woman who went to the local emergency department 30 min after ingesting, accidentally, 5000 mg of anhydrous caffeine for a preworkout supplement was discharged asymptomatic with internal medicine follow-up.
Abstract: Caffeine (1,3,7-trimethylxanthine) is a natural product commonly presented in food’s composition, beverages and medicinal products. Generally, it is thought to be safe under normal dosage, yet it can be fatal in case of severe intoxication. We report a case of a healthy 32-year-old woman who went to the local emergency department (ED) 30 min after ingesting, accidentally, 5000 mg of anhydrous caffeine for a preworkout supplement. At the ED, she presented an episode of presyncope followed by agitation. ECG showed polymorphic broad complex QRS tachycardia and arterial blood gas revealed metabolic acidaemia with severe hypokalemia. The dysrhythmia was successfully treated with intravenous propranolol. Acid–base and hydroelectrolytic disorders were also corrected. A persistent sinus tachycardia was observed in the first 2 days in the ward and 5 days later she was discharged asymptomatic with internal medicine follow-up.
TL;DR: An investigation of cannabis samples from the patient’s preferred dispensary demonstrated contamination with several varieties of Cryptococcus, including C. neoformans, and other opportunistic fungi, raising concern regarding the safety of dispensary-grade cannabis, even in immunocompetent users.
Abstract: Cryptococcal meningitis is a life-threatening condition most commonly observed in immunocompromised individuals We describe a daily cannabis smoker without evidence of immunodeficiency presenting with confirmed Cryptococcus neoformans meningitis An investigation of cannabis samples from the patient's preferred dispensary demonstrated contamination with several varieties of Cryptococcus, including C neoformans, and other opportunistic fungi These findings raise concern regarding the safety of dispensary-grade cannabis, even in immunocompetent users
TL;DR: The current understanding of the epidemiology of and risk factors for Dieulafoy’s lesion of the duodenum, the pathophysiology of this disorder, and currently available approaches to diagnosis and management are outlined.
Abstract: Dieulafoy's lesion is an abnormally large, tortuous, submucosal vessel that erodes the overlying epithelium without primary ulceration or erosion. The lesion predominantly occurs in the proximal stomach but it is also reported in extragastric sites. The pathogenesis and precipitating factors are poorly understood. Patients frequently present with gastrointestinal haemorrhage that can range from being self-limited to massive life threatening. Although there are no standard guidelines, endoscopy has significantly impacted the diagnosis and management. This review outlines our current understanding of the epidemiology of and risk factors for Dieulafoy's lesion of the duodenum, the pathophysiology of this disorder, and currently available approaches to diagnosis and management.
TL;DR: A case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment syndrome, and the severe episode of capillary leak was potentially triggered by influenza virus infection.
Abstract: Systemic capillary leak syndrome (SCLS), also known as Clarkson syndrome, is a rare disease with potential fatal outcome. The clinical picture involves leakage of fluid and protein from the bloodstream into peripheral tissues, resulting in hypoalbuminaemia, elevated haematocrit, oedema and hypotension. The spectrum of the symptoms ranges from discrete swelling/oedema of extremities to fulminant cardiogenic shock. We present a case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment syndrome. The severe episode of capillary leak was potentially triggered by influenza virus infection. With the benefit of hindsight, he presented with symptoms of SCLS 2 years prior the major acute episode. Here we describe this case and review some aspects of the literature on SCLS, with particular focus on the pathogenesis, treatment/prophylaxis and long-term physical and psychological complications.
Posted Content•
TL;DR: The Report contains an econometric analysis of VAT Gap determinants, which is a novelty introduced from this year’s Study.
Abstract: In this Report, the Authors present the new Value Added Tax (VAT) Gap estimates for 2016, as well as updated estimates for 2012-2016. In addition to the analysis of the Compliance Gap, this Report examines the Policy Gap in 2016 as well as the contribution that reduced rates and exemptions made to the theoretical VAT revenue losses. Moreover, the Report contains an econometric analysis of VAT Gap determinants, which is a novelty introduced from this year’s Study.
TL;DR: A switch to another BRAF inhibitor in a progressively ascending pattern was successfully performed in a middle-age man affected by a metastatic, BRAF mutant melanoma with a good response of the metastatic melanoma.
Abstract: We present a case report of an early-onset drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) induced by vemurafenib (BRAF inhibitor) in a middle-age man affected by a metastatic, BRAF mutant melanoma who was started on first-line metastatic treatment with vemurafenib and cobimetinib.After initiating the treatment, the patient presented an extensive cutaneous rash with eosinophilia and renal impairment. Due the constellation of signs and symptoms, a diagnosis of DRESS syndrome was made which strongly contraindicated the reintroduction of vemurafenib due to its hypersensibility reaction. Thus, vemurafenib was stopped immediately, and we started corticoid treatment with clinical improvement.Due to the contraindication to start vemurafenib again, after multidisciplinary view of the case and having balanced the risks and benefits, we successfully performed a switch to another BRAF inhibitor in a progressively ascending pattern, without any skin toxicity and with a good response of the metastatic melanoma.
TL;DR: A 25-year-old woman with l upus nephritis on peritoneal dialysis whose lupus was quite silent for almost three years is presented, and secondary to HCQ she developed severe hypoglycaemic episodes, which were completely resolved after stopping HCQ.
Abstract: Hydroxychloroquine (HCQ) is a commonly used drug for the treatment of systemic lupus erythematosus (SLE). Renal involvement is common in SLE. We present a 25-year-old woman with lupus nephritis on peritoneal dialysis whose lupus was quite silent for almost three years, and secondary to HCQ she developed severe hypoglycaemic episodes, which were completely resolved after stopping HCQ.
TL;DR: A 43-year-old woman with a history of recently diagnosed metastatic melanoma was commenced on systemic therapy with nivolumab, an anti-programmed cell death-1 monoclonal antibody and one of an increasing group of the so-called ‘immune checkpoint inhibitors’, and developed incident autoimmune hypothyroidism and progressive fatigue, proximal weakness, myalgia and dysphagia.
Abstract: A 43-year-old woman with a history of recently diagnosed metastatic melanoma was commenced on systemic therapy with nivolumab, an anti-programmed cell death-1 monoclonal antibody and one of an increasing group of the so-called ‘immune checkpoint inhibitors’. She experienced a dramatic complete response within 6 months of initiation. However, in addition to developing incident autoimmune hypothyroidism, she also developed progressive fatigue, proximal weakness, myalgia and dysphagia. Initial investigations with blood tests, electrophysiology and a muscle biopsy were non-specific or normal. Subsequent examination revealed ‘woody’ thickening of the subcutaneous tissues of the forearms, thighs and calves consistent with fasciitis. MRI and a full-thickness skin–muscle biopsy were ultimately diagnostic of a likely iatrogenic autoimmune myofasciitis. The clinical manifestations only responded partly to prednisolone 30 mg orally and treatment was escalated to include intravenous immunoglobulin. At 3 months, this has only resulted in a modest incremental improvement.
TL;DR: A 73-year-old man presented with three episodes of dysphasia and disinhibited behaviour, a single seizure and transient ischaemic attack-like events characterised by right arm and/or leg weakness, leading to clinical stabilisation and diagnosis of rheumatoid meningoencephalitis.
Abstract: A 73-year-old man presented with three episodes of dysphasia and disinhibited behaviour, a single seizure and transient ischaemic attack-like events characterised by right arm and/or leg weakness. These episodes were separated by month-long asymptomatic intervals. Medical history included rheumatoid arthritis, which was clinically quiescent on leflunomide. Repeated cerebrospinal fluid examination showed a persistent lymphocytosis with mildly reduced glucose and elevated protein; oligoclonal bands and viral PCR were negative. MRI of the brain was initially normal, but 7 months after initial presentation revealed meningeal enhancement with bifrontal cortical hyperintensities on T2/fluid-attenuated inversion recovery. Brain biopsy demonstrated necrotising granulomatous meningitis with mixed T cell and B cell infiltrates and without evidence of vasculitis or infection. Serum anticyclic citrullinated peptide antibodies were strongly positive. The diagnosis of rheumatoid meningoencephalitis was made on the basis of brain biopsy findings and serological evidence of active rheumatoid disease. Steroids and rituximab therapy were started leading to clinical stabilisation.
TL;DR: A patient with extremely high calcium level was admitted to the authors' department unconscious with pathological ECG recording, demonstrating shortening of QT interval, and hyperparathyroidism and a large parathyroid adenoma was then surgically removed.
Abstract: A patient with extremely high calcium level of 23.9 mg/dL (5.97 mmol/L) was admitted to our department unconscious with pathological ECG recording, demonstrating shortening of QT interval. The patient was treated by fluid resuscitation, bisphosphonates, salmon calcitonin and steroids. Haemodialysis with low calcium bath had been promptly provided with improvement of consciousness and calcium level. ECG changes disappeared. Subsequent investigations revealed hyperparathyroidism and a large parathyroid adenoma was then surgically removed. Extreme and rapid calcium elevation (parathyroid crisis) is rarely seen in primary hyperparathyroidism and usually is distinctive for malignancy. In the context of acute kidney injury and refractory hypercalcaemia with life-threatening complications (coma, ECG changes with impending danger of arrhythmia), haemodialysis may effectively decrease calcium levels. It should be pointed out that dialysis is an efficient method of treatment of refractory hypercalcaemia, parathyroid crisis, but it is rarely used due to its invasive nature.
TL;DR: This case illustrates a 36-year-old man who presented with a factor VIII (FVIII) inhibitor (acquired haemophilia A) with cutaneous bleeding and a significant thigh haematoma, adding further evidence to the current body of literature suggesting alemtuzumab as a causative agent for the development of an FVIII inhibitor.
Abstract: This case illustrates a 36-year-old man who presented with a factor VIII (FVIII) inhibitor (acquired haemophilia A) with cutaneous bleeding and a significant thigh haematoma. No traditional risk factors for the development of a FVIII inhibitor were identified. However, previous treatment with alemtuzumab for multiple sclerosis was noted in the patient's history. Alemtuzumab is an anti-CD52 monoclonal antibody and is known to be associated with the development of a number of autoimmune conditions, with a delay in onset of these conditions as long as 5 years after the cessation of treatment. To our knowledge, there have only been three previously documented cases of a FVIII inhibitor in the setting of alemtuzumab therapy. This case adds further evidence to the current body of literature suggesting alemtuzumab as a causative agent for the development of an FVIII inhibitor.