Rapid progressive eosinophilic cardiomyopathy in a patient with Churg–Strauss syndrome (CSS)

Summary Churg–Strauss syndrome (CSS) is a rare necrotizing, systemic vasculitis that is almost invariably associated with bronchial asthma. Although overall prognosis is good and treatment with corticosteroids alone or in combination with other immunosuppressive agents is typically successful, there are reports of patients that do not show signs of clinical improvement under the usual pharmacotherapy. Small clinical studies suggested that cardiac or gastrointestinal involvement is associated with an adverse prognosis. We here report the case of a 38 year old male patient with a history of bronchial asthma who was admitted to our hospital for further evaluation of progressive dyspnea. Blood eosinophilia, infiltrates of both lungs, signs of necrosis and eosinophil deposits on myocardial biopsy combined with a history of bronchial asthma established the diagnosis of CSS with cardiac involvement. We initiated an immunosuppressive therapy with prednisone and methotrexate. Upon tapering of the dosage of prednisone, we noticed worsening of symptoms and further deterioration of cardiac function. Despite the addition of cyclophosphamide and adjustment of heart failure medication, we were not able to stabilize the cardiac situation. Due to rapid progressive eosinophilic cardiomyopathy associated with CSS refractory to medical therapy, our patient was placed on the urgent heart transplantation waiting list and, in the meantime, has undergone successful cardiac transplantation.     

Effective therapy of a child case of refractory nephrotic syndrome with tacrolimus

We report here the case of a 9-year-old Japanese boy with nephrotic syndrome caused by focal segmental glomerulosclerosis, which was refractory to treatment. Although aggressive immunosuppressive therapy consisting of methylprednisolone pulse therapy combined with cyclosporine A (CsA) and intermittent low density lipoprotein apheresis was effective in overcoming his steroid-resistant state, the child became persistently steroid-dependent, that is, more than 0.75 mg/kg per day of prednisolone combined with CsA was required to maintain a negative test for proteinuria. Since adverse effects of prednisolone, such as short stature, obesity, osteoporosis and cataract, were noted, CsA in his treatment regimen was replaced with tacrolimus at the dose of 0.1 mg/kg per day, with the trough blood level of the drug maintained at around 10 ng/ml. Within 4 months of the inclusion of tacrolimus in the treatment regimen, complete remission was achieved, with no recurrence of the proteinuria, while the prednisolone dose could be tapered to 0.3 mg/kg per day. No adverse effects of tacrolimus were observed. These clinical results suggest that tacrolimus may be the drug of choice in selected patients with refractory nephrotic syndrome, even if pediatric-onset cases, at least those in whom the steroid-sparing effects of CsA is unsatisfactory.     

变应性肉芽肿性血管炎11例临床分析

【目的】分析变应性肉茅肿性血管炎(CSS)的临床表现．以便提高临床诊断水平【方法】11例确诊CSS患者．分析其既往痛史、临床表现、实验室检查、活检病理及治疗情况【结果】11例患者中发生哮喘10例;10例出现皮肤损伤;分别有1例和2例出现周围神经和中枢神经病变：消化道症状者1例．包括腹痛、腹泻和脓血便：肾脏损害者4例;心脏受累者6例全部病例周围血嗜酸粒细胞(EOS)均增高．有1例活捡病理支持CSS诊断。所有患者均用糖皮质激素和免疫抑制制治疗。【结论】CSS的临床表现多种多样．对于有系统性表现．特别是血嗜酸细胞升高者应提高警惕。     

Prominent gallbladder enlargement: Kawasaki disease or other congenital or acquired gallbladder disease? A case report

Kawasaki disease (KD) is a common systemic vasculitis in childhood that can result in damage to multiple body systems. However, prominent gallbladder (GB) enlargement in the acute stage is especially rare. A 5-year-old boy was admitted to the hospital with an 8-day history of a cervical mass, 7-day history of fever, and 5-day history of abdominal pain and rash. The child was diagnosed with KD. After treatment with high-dose intravenous immunoglobulin therapy (2 g/kg), all clinical manifestations were relieved except the abdominal pain. Enhanced computed tomography showed distinct enlargement of the GB, and a congenital choledochal cyst was strongly suspected. After high-dose glucocorticoid treatment, his obviously enlarged GB returned to normal size in the subacute phase. No abnormality was found during 2 years of follow-up. Prominent GB enlargement may emerge in the acute stage of KD. The enlarged GB can return to normal size within the subacute stage by standard treatment for KD. Proper diagnosis, thorough differential diagnosis, and active anti-inflammatory treatment of KD are crucial to avoid surgery.     

Prednisolone at anti-inflammatory or immunosuppressive dosages in conjunction with doxycycline does not potentiate the severity of Rickettsia rickettsii infection in dogs.

Dogs were experimentally inoculated with Rickettsia rickettsii to determine if anti-inflammatory or immunosuppressive dosages of prednisolone, when administered in conjunction with an antirickettsial antibiotic (doxycycline), induced therapeutically relevant pathophysiological consequences that ultimately influence disease outcome. Although the duration of rickettsemia was prolonged in dogs receiving immunosuppressive, but not anti-inflammatory, corticosteroids, concurrent administration of doxycycline and corticosteroids conferred no other detected detrimental effects. Treatment with doxycycline or doxycycline in conjunction with prednisolone resulted in decreased R. rickettsii-specific antibody titers; however, examination of appropriately timed acute- and convalescent-phase serum samples would have facilitated an accurate diagnosis of Rocky Mountain spotted fever (RMSF) in all 16 dogs. We conclude that the concurrent use of anti-inflammatory or immunosuppressive doses of prednisolone in conjunction with doxycycline, early in the course of experimental RMSF, confers no clinically relevant detrimental effects and that additional studies might be indicated to detect possible beneficial effects in cases of severe or potentially fulminant RMSF. However, because the illness induced in these dogs was of mild to moderate severity, the results of this study should definitely not be construed as supporting the safety or efficacy of prednisolone for treatment of severe canine or human RMSF.     

表现为重症肺炎的变应性肉芽肿性血管炎的救治与思考

目的提高对变应性肉芽肿性血管炎(Churg-Strauss syndrome,CSS)的认识,减少误诊误治。方法对1例表现为重症肺炎的CSS患者的临床资料进行回顾性分析并复习相关文献。结果本例因咳嗽、气喘伴发热10 d,乏力、双下肢疼痛1 d入院。经查体、血常规及血气分析初步诊断为肺部感染、支气管哮喘、过敏性鼻炎等,予抗生素治疗效果不佳。后经查核周型抗中性粒细胞胞浆抗体(+),并结合右下肢斑疹处皮肤活检结果及糖皮质激素治疗有效,诊断为CSS。病情缓解后出院。随访近3年,糖皮质激素持续治疗,病情稳定。结论对以呼吸道感染为首发表现的多系统损伤性疾病,应考虑到CSS的可能,及时行相关检查,及早明确诊断并治疗。     

Successful Treatment of Opioid-Refractory Cancer Pain with Short-Course,Low-Dose Ketamine

Opioids remain the mainstay of treatment for severe cancer pain, but up to 20% of patients have persistent or refractory pain despite rapid and aggressive opioid titration, or develop refractory pain after long-term opioid use. In these scenarios, alternative agents and mechanisms for analgesia should be considered. This case report describes a 28-year-old man with metastatic pancreatic neuroendocrine cancer with severe, intractable pain despite high-dose opioids including methadone and a hydromorphone patient-controlled analgesia (PCA). After treatment with short-course, low-dose ketamine, his opioid requirements decreased by 99% and pain ratings by 50%, with the majority of this decrease occurring in the first 48 hours. As this patient's pain and opioid regimen escalated, he likely experienced some component of central sensitization and hyperalgesia. Administration of ketamine reduced opioid consumption by 99% and potentially “reset” neuronal hyperexcitability and reduced pain signaling, allowing for improved pain control.     

Asthma associated with worsening leg ulcer: a case of vasculitis in primary care

A 71-year-old black woman was admitted to the hospital with a 2-month history of a nonhealing leg ulcer. Her medical history included diabetes mellitus type 2, congestive heart failure, allergic rhinitis, and asthma. The patient's asthma was labile and steroid-dependent until 2 years before admission, at which time zafirlukast therapy was started. On further questioning, the patient revealed a 6-month history of malaise and a 40-lb weight loss. A physical examination showed a 2-cm Stage 3 ulcer on the medial aspect of the right ankle with diminished sensation in both feet and left footdrop. Electromyography revealed mononeuritis multiplex. The patient's white blood cell count was 11,100/mm3 with 60% eosinophils. A sural nerve biopsy showed vasculitis consistent with Churg-Strauss syndrome. One week after prednisone therapy was initiated, the patient's foot strength was nearly normal and her eosinophilia had resolved. Although Churg-Strauss syndrome is a rare disorder, in the setting of asthma and multiple disparate signs and symptoms, the broad diagnostic category of serious vasculitic illness should be considered.     

Extremely Painful Multifocal Acquired Predominant Axonal Sensorimotor Neuropathy of the Upper Limb

The differential diagnosis of upper extremity mononeuritis multiplex includes neuralgic amyotrophy, vasculitic neuropathy, and Lewis‐Sumner syndrome. We describe 3 patients initially suspected of neuralgic amyotrophy, who had an extremely painful, protracted, progressive disease course, not fitting one of these established diagnoses. Nerve ultrasonography showed focal caliber changes of the roots, plexus, and limb nerves. Electromyography showed predominant multifocal axonopathy. Ongoing autoimmune neuropathy was suspected. Steroid treatment provided temporary relief, and intravenous immunoglobulin A sustained pain decrease and functional improvement. These patients appear to have extremely painful axonal inflammatory neuropathy, with a good response to immune‐modulating treatment.     

Multiple mononeuritis with peripheral blood eosinophilia in a patient with Q fever: An unusual presentation: Case report and review of the literature

Coxiella burnetii is a gram-negative bacterium that typically lives and multiplies within monocytes and macrophages of the host, being the etiologic agent of the zoonosis Q fever. Q fever is usually divided into acute and chronic forms, with a significant percentage of patients being asymptomatic. In the wide spectrum of the disease, neurological involvement seems to be extremely rare and peripheral neuropathy presenting with mononeuritis multiplex is one of the possible presentations with low rates of occurrence. Hereby, we present an unusual case of a 55-year-old male with fever and multiple mononeuritis attributed to Q fever and we summarize a short review of C. burnetii infection.     

Anaphylaxis. Why it happens and what to do about it

Anaphylaxis is an often severe, potentially life-threatening symptom complex. Urticaria, airway edema, vascular collapse, asthma, abdominal pain, and diarrhea are common clinical signs. Recently recognized syndromes of anaphylaxis include reactions due to exercise, food preservatives, aspirin, steroids, dialysis, various serums, and human seminal fluid. Initial therapy is directed at maintaining an effective airway and circulatory system. Administration of aqueous epinephrine is always indicated. Other measures may include oxygen delivery by controlled flow, administration of an aerosolized beta agonist, slow infusion of aminophylline, and rapid infusion of intravenous fluid. Patients with severe acute reactions should be monitored in-hospital.     

Churg-Strauss syndrome (AGA) in Japan]

Churg-Strauss syndrome (CSS) is a rare disease belonging to the group of necrotizing vasculitis. With introduction of guideline therapy for bronchial asthma, many patients have enjoyed the benefit regardless their etiology of asthma. Recently, however, there have been multiple case reports of CSS with relation to leukotriene modifiers, leading to a concern of the potential risk association with the development of CSS and the treatment. We have analyzed recent 16 cases of CSS in our group. Twelve cases had been prescribed steroids (7 oral steroid and 5 inhaled steroid) for a treatment of asthma. Three cases had taken leukotriene modifiers. But two of them were under steroid reduction course. It remains unclear what the exact mechanism of this syndrome is, but physicians must be wary for the signs of CSS, particularly in patients with asthma in whom corticosteroids are tapered.     

Celiac plexus block following high-dose opiates for chronic noncancer pain in a four-year-old child

This is the first case report documenting the use of a neurolytic celiac plexus block for relieving chronic noncancer pain in a pediatric patient. The child was a 4-yr-old male with an unknown form of inflammatory bowel disease since 1 yr of age. Chronic abdominal pain became a problem at 3 yr of age, following multiple bowel resections; continuous intravenous narcotic administration was implemented for pain control. The patient's pain became refractory to high-dose morphine administration (maximum dose, 267 mg/kg/day, iv), and, for that reason, a CT-guided neurolytic celiac plexus block was performed. This procedure resulted in improved pain control along with a major reduction in narcotic use to 7 mg/kg/day of morphine.     

Complete occulusion of left renal artery in pediatric-onset Takayasu's arteritis

A-16-year-old male adolescent with a 4-year history of protean clinical manifestations such as fever, abdominal pain, back pain, erythema nodosum and uveitis developed complete occlusion of left renal artery. Although he had been suspicious of having an autoimmune disease and treated with prednisolone, a definite diagnosis was not made. Finally, an angiography disclosed stenosis of abdominal aorta just beneath the origin of the renal arteries as well as complete occlusion of left renal artery. It has been reported that pediatric-onset Takayasu's arteritis sometimes shows protean clinical manifestations as in ours. Takayasu's arteritis should be considered as one of the underlaying disease, when a child develops protean manifestations suggesting an autoimmune disease.     

Acute diverticulitis in patients 40 years of age and younger

Acute colonic diverticulitis typically occurs in patients older than 60 years of age but is uncommon in patients under the age of 40, which may lead to a delay in diagnosis. Because abdominal pain is a very common presenting symptom in emergency department patients, we retrospectively analyzed the cases of 21 patients 40 years of age and younger diagnosed with acute diverticulitis and characterized the presenting signs and symptoms, laboratory and radiographic findings, treatment, and outcome. There were 17 men and 4 women with a mean age of 34.1 +/-5.9 years. All patients had abdominal pain, with 14 (67%) patients noting pain in the left lower quadrant (LLQ) and 5 (24%) patients noting right lower quadrant (RLQ) pain. Nausea was present in 18 (86%) patients and fever in 15 (71%) patients. The mean pulse rate was 103 +/- 16 and the mean temperature was 100.7 +/- 1.4 F. Leukocytosis was present in 19 (90%) patients. Plain abdominal radiographs were obtained in 19 (91%) patients and were normal in 15 (79%) of these cases. Computed tomographic (CT) scans were obtained in 15 (71%) patients which revealed findings consistent with acute diverticulitis in 14 (93%) patients. The admitting diagnosis was diverticulitis in 10 of the 12 patients with LLQ tenderness and appendicitis in 4 of the 6 patients with RLQ tenderness. Overall, six patients were taken to surgery: three patients had cecal diverticulitis and three patients had perforated colonic diverticulitis. General treatment measures included bowel rest in 18 (86%) patients, and intravenous fluids and antibiotics in all patients. All patients survived. In conclusion, acute diverticulitis is uncommon in patients under 40 years of age; however, this condition may be confused with other conditions, usually acute appendicitis. As a result, clinicians should consider acute diverticulitis in young patients with acute abdominal pain, especially if they are male with nausea, fever, tachycardia, and leukocytosis, and consider obtaining a CT scan to aid in the diagnosis.     

Effectiveness of systemic immunoglobulins in the treatment of the systemic variants of juvenile rheumatoid arthritis]

Preparations of intravenous immunoglobulin (sandoglobulin, pentaglobin, intraglobin F, octagam) were given daily or each other day in a course dose 0.3-1 g/kg to 43 patients aged 4 to 15 years. Eight of them had allergosepsis, 22--allergoseptic variant of juvenile rheumatoid arthritis (JRA) and 13--systemic JRA and generalized joint syndrome. The treatment induced remission in patients with allergosepsis, prednisolone was discontinued. JRA patients coped with fever, eruption, polyserositis, infection symptoms. Leukocyte count normalized. Preparations of intravenous immunoglobulin in low doses can be therapy of choice in patients with rheumatic-like diseases. In JRA patients it can be effectively used against fever, eruption, polyserositis, carditis, leukocytosis, intercurrent infection before or in the course of immunosuppressive therapy.     

Rescue of a patient out of a grain container: the quicksand effect of grain

Grain storage containers not only present inherent dangers to the operators, but also to the rescuers if someone falls in. Here we report the rescue of a patient from a grain container using a novel technique involving a cylinder placed around the patient. This allowed the grain to be sucked out from around the patient and enabled his rescue uninjured. The rescue action was complicated by acute chest pain in the patient while he was submerged in the grain, and a severe asthma attack in the emergency physician. The rescue and the dilemmas encountered are described together with a review of the relevant literature.     

Severe hypotension and coma secondary to unrecognized chronic anterior hypophysitis

We report an endocrine emergency of a 52-year-old woman with chronic anterior-pituitary failure of autoimmune origin who developed hypopituitary crisis with coma and severe hypotension provoked by an intercurrent bronchopneumonia. At admission to the ICU hypopituitarism had not been diagnosed and only Hashimoto's thyroiditis with thyroid replacement therapy could be obtained from the patient's history. Although the patient presented with somatic signs suggestive of hypopituitarism, other causes of coma and hypotension had first to be excluded. In the absence of specific treatment the patient died 18 h later with refractory cardiac arrest. Diagnosis of acute decompensated chronic hypophyseal failure must be considered if hypothermia, refractory hypotension and signs of infection without fever are associated with a short stature and the loss of axillary and pubic hair. Waiting for laboratory confirmation of the diagnosis must not delay immediate life-saving specific glucocorticoid treatment.     

Spontaneous superior mesenteric artery (SMA) dissection: an unusual cause of abdominal pain

A 44-year-old woman presented to our Emergency Department with a 4-day history of severe, sharp left upper quadrant abdominal pain associated with nausea and vomiting. She had been seen 3 days prior at another Emergency Department, and had a negative work-up including a normal non-contrast computed tomography (CT) scan of the abdomen/pelvis for possible kidney stone. Vital signs were: temperature 36.3°C (97.3°F), pulse 100 beats/min, respiratory rate 18 breaths/min, and blood pressure 141/80 mm Hg. Physical examination was remarkable for marked tenderness in the left upper and middle quadrants and voluntary guarding. Bowel sounds were normal. Although laboratory studies were normal, a CT scan of the abdomen/pelvis with intravenous contrast suggested a superior mesenteric artery dissection. This was confirmed with arteriography. The clinical presentation, diagnostic evaluation, and management of superior mesenteric artery dissection are reviewed.     

Effective treatment with cyclosporine A of a child with systemic lupus erythematosus resistant to cyclophosphamide pulse therapy

Intermittent intravenous cyclophosphamide pulse therapy (IVCY) has been reported to be effective for the treatment of refractory systemic lupus erythematosus (SLE). However, there is a proportion of patients with SLE, who are IVCY-resistant and need a long-term therapy to sustain the remission. We report here a case of a 6-year-old Japanese girl with SLE refractory to IVCY. She suffered from persistent hypocomplementemia and recurrent flares despite receiving methylprednisolone pulse, mizoribine pulse and IVCY therapy. Administration of cyclosporine A (CsA) was, therefore, initiated. Within 2 months of the start of CsA administration, the serum levels of C3, C4 and complement hemolytic activity began to increase rapidly, and finally returned to the normal levels. The serum anti-dsDNA antibody titer was decreased significantly after the initiation of this treatment. The prednisolone dose could be successfully tapered without precipitation of any flares. No adverse effects of CsA were observed. Based on these clinical observations, we suggest that CsA might be an effective treatment option for selected cases of refractory SLE.