Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report

BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.CASE SUMMARY A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage.However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids;further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.CONCLUSION For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death.     

Biliary causes of postcholecystectomy syndrome

Background: The postcholecystectomy syndrome refers to the persistence of gastrointestinal symptoms after cholecystectomy; patients with this syndrome commonly seek treatment in the Emergency Department (ED). There are a multitude of biliary and non-biliary causes of postcholecystectomy syndrome. Objectives: To review the biliary causes of postcholecystectomy syndrome and present a suggested diagnostic workup. Discussion: A number of biliary causes are discussed, including choledocholithiasis, bile duct injury and biliary leaks, cystic duct and gallbladder remnants, sphincter of Oddi dysfunction, and biliary ascariasis. The ED workup should focus on differentiating biliary from non-biliary causes of the patient's symptoms. Conclusion: A right upper quadrant abdominal ultrasound, liver transaminases, alkaline phosphatase, and serum bilirubin are the tests most useful in the ED for making this distinction and determining the most appropriate treatment and disposition of the patient. Computed tomography may be of use in the initial post-operative period.     

Association of in vitro oxidative stress, serum ferritin concentration and C-reactive protein in febrile emergency room patients

BACKGROUND: Both C-reactive protein (CRP) and ferritin have been reported to reflect the extent of oxidative stress and inflammation in individual patients and may be useful markers of disease activity and mortality risk. Exposure to oxidative stress has been reported to increase ferritin synthesis. We investigated the relationship between oxidative stress with CRP and ferritin concentrations in febrile emergency room patients to test the hypothesis whether the intensity of oxidative stress correlated with serum ferritin concentration. METHODS: Six normal healthy volunteers and 59 emergency room, febrile patients with body temperature >38.3 we enrolled before receiving medical treatment. Baseline measurements included complete blood count, blood biochemistry, CRP and serum ferritin concentrations, and transferring saturation (TSAT). The intensity of lucigenin-enhanced chemiluminescence (LucCL), corresponding to the level of superoxide, was detected by luminometer. RESULTS: In febrile patients, plasma LucCL intensity was higher than in normal healthy volunteers (P<0.05). The group with bacterial infection had higher serum ferritin (319.4+/-53.7 vs 102.0+/-21.2 ng/dL, P<0.05) and CRP concentrations (7.2+/-1.2 vs 2.2+/-0.6 mg/dL; P<0.05) than the group without bacterial infection. There were no differences in leukocytes (9790+/-606 vs 9577+/-656 /mm3) or plasma LucCL intensity (423.7+/-10.8 vs 409.5+/-6.9 relative light unit?RLU?;) between the two groups. LucCL intensity showed no correlation with serum ferritin concentration (r= -0.0599, P>0.05), TSAT(r= -0.0592, P>0.05), CRP(r= 0.1027, P>0.05) and absolute neutrophil counts (r= 0.1059; P >0.05). CONCLUSION: In this sample of emergency room febrile patients, plasma LucCL intensity was higher than in normal healthy control volunteers. A single point measurement of oxidative stress, particularly plasma LucCL intensity, may not be sufficient to differentiate the origin of fever in febrile patients. These data demonstrate that patients with bacterial infection had increased levels of CRP and ferritin, but this was not associated with LucCL intensity.     

Adult-onset Still's disease

Background: Adult‐onset Still's disease (AOSD) is a febrile disorder of unknown aetiology characterised by typical spiking fever, evanescent rash, arthralgia and leucocytosis. Methods: According to the diagnostic criteria of AOSD, we identified 84 patients between 1990 and 2003. The aim of this study was to analyse the characteristics of AOSD in Turkish patients who were followed‐up in a tertiary referral centre. Results: Of 84 patients of AOSD, 59 (70.2%) were female, 25 (29.8%) were male. Arthralgia (96.4%), fever (95.2%), arthritis (69%), sore throat (65.5%) and typical rheumatoid rash (59.5%) were the most common findings. The mean value of laboratory findings were as follows; C‐reactive protein level of 11.59 ± 6.81 mg/dl, erythrocyte sedimentation rate (ESR) of 89.05 ± 31 mm/h, leukocyte count of 16,234.51 ± 7785.2/μl. Leucocytosis was present in 69 patients (84.15%). Forty‐eight patients had a WBC count ≥ 15,000/μl. Hypoalbuminaemia was present in 35 patients. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 30 patients, whereas abnormal levels of alkaline phosphatase in 16 patients. Thirty‐seven patients had an ESR value of more than 100 mm/h. Thirty‐two patients had a ferritin value of more than 1000 ng/dl. Conclusion: High fever, sore throat, rheumatoid rash, polyarthritis, hyperferritinaemia (≥ 1000 ng/ml), leucocytosis with a neutrophilic predominance, anaemia and hypoalbuminaemia were remarkable observations in the initial examination.     

Low-dose amphotericin B lipid complex for the treatment of persistent fever of unknown origin in patients with hematologic malignancies and prolonged neutropenia

We studied the safety of low-dose amphotericin B lipid complex (ABLC, at 1 mg/kg/day) in 30 persistently febrile (>38 degrees C for at least 5 days or with recurrent fever after 3 days of apyrexia) and neutropenic (<0.5 x 10(9)/l) adult patients with hematologic malignancies. The median age was 45 years (range 18-67), most (60%) had an acute leukemia and all had fever of unknown origin (FUO). The total duration of neutropenia was a median of 17 days (range 9-33), and the total number of days with fever 10 days (range 6-39). Seven patients experienced mild-to- moderate infusion-related adverse events (IRAE). The serum creatinine and urea increased from baseline to end of therapy in 76 and 63% of cases, but the maximum levels reached were <130 micromol/l and <11 mmol/l, respectively, in all cases. Liver enzymes showed modest but significant increases in most patients during therapy, while bilirubin decreased in 74% of cases. Response to treatment (defervescence within 6 days without developing a fungal or nonfungal infection) was seen in 22 cases (73%, 95% CI 58-89%), while 8 episodes were considered treatment failures: 2 due to persistent FUO, 1 withdrew due to IRAE, 2 developed nonfungal infections and 3 developed a presumed or definite invasive mycosis. We conclude that low-dose ABLC is very safe and well tolerated and seems as effective as c-AmB for this indication. Thus, randomized trials at this dose level appear justified to demonstrate any real benefit over c-AmB or other lipid formulations for the treatment of FUO in neutropenic patients.     

Iron,zinc and aluminium ferritin content of hemodialysis hyperferritinemic patients: Comparison with other hyperferritinemic clinical conditions and normoferritinemic blood donors

The present study describes the specific content of ferritin iron, zinc and aluminium in four different groups: 1) hemodialysis hyperferritinemic patients; 2) septic patients; 3) iron overloaded patients with hematologic diseases; and 4) blood donors.In all four groups high levels of aluminium and zinc were found in addition to those of iron. However, the sum of the ferritin ions of the control group is significantly higher than that of the other three groups. Furthermore, while ferritin of hemodialysis patients has the same molecular ratio of metal ions as control group (high Al content vs. Fe and Zn), a lower Al/Fe ratio is found both in septic and hematological patients.The results of the present paper might help to explain the high percentage of hyperferritinemia found in hemodialysis patients also in presence of low transferrin saturation and in absence of inflammatory markers. Moreover, the high content of ions other than iron in the ferritin core leads us to believe that ferritin is not only an iron storage protein but rather a regulator of redox active ions.     

Adult-onset Still's disease—pathogenesis,clinical manifestations,and new treatment options

Abstract

Adult-onset Still's disease (AOSD), a systemic inflammatory disorder, is often considered a part of the spectrum of the better-known systemic-onset juvenile idiopathic arthritis, with later age onset. The diagnosis is primarily clinical and necessitates the exclusion of a wide range of mimicking disorders. AOSD is a heterogeneous entity, usually presenting with high fever, arthralgia, skin rash, lymphadenopathy, and hepatosplenomegaly accompanied by systemic manifestations. The diagnosis is clinical and empirical, where patients are required to meet inclusion and exclusion criteria with negative immunoserological results. There are no clear-cut diagnostic radiological or laboratory signs. Complications of AOSD include transient pulmonary hypertension, macrophage activation syndrome, diffuse alveolar hemorrhage, thrombotic thrombocytopenic purpura and amyloidosis. Common laboratory abnormalities include neutrophilic leukocytosis, abnormal liver function tests, and elevated acute-phase reactants (ESR, CRP, ferritin). Treatment consists of anti-inflammatory medications. Non-steroidal anti-inflammatory drugs have limited efficacy, and corticosteroid therapy and disease-modifying anti-rheumatic drugs are usually required.

Recent advances have revealed a pivotal role of proinflammatory cytokines such as tumor necrosis factor-α (TNF-α), interleukin (IL)-1, IL-6, IL-8, and IL-18 in disease pathogenesis, giving rise to the development of novel targeted therapies aiming at optimal disease control.

The review aims to summarize recent advances in pathophysiology and potential therapeutic strategies in AOSD.     

The Frequency of Unsuspected Minor Illness or Injury in Intoxicated Patients

Objective: To determine the frequency of unsuspected minor illness or injury in a group of patients frequently seen in the ED for acute intoxication.
Methods: The medical records of the 20 patients seen most frequently in the ED for acute intoxication in 1993 were reviewed for the number of ED visits for intoxication, the number of associated documented episodes of minor trauma or illness, the extent of ED workup of discovered illness or injury, and patient disposition from the ED.
Results: The 20 study patients were evaluated in the ED 1,858 times in 1993 for acute intoxication, a mean of 92.5 visits/patient (±26.6). The most frequent injury was minor trauma above the neck, occurring a mean of 9 times (±3.6) in each of the study patients during 1993. Evaluation included repeated neurologic examinations and frequent radiography of the cervical spine ( n = 80), skull ( n = 5), facial bones ( n = 6), and mandible ( n = 5). A limited number of head CT scans also were done ( n = 8). The most frequent minor illnesses were gastritis ( n = 7), managed with hydration, and mild hypothermia ( n = 6), managed with passive rewarming.
Conclusions: The incidence of unsuspected minor illness or injury in this patient group was substantial. While most unsuspected medical problems had little clinical significance, some were potentially dangerous, and some necessitated hospitalization (e.g., hypothermia, hematemesis, and respiratory depression).     

Impact of a Negative Evaluation for Underlying Coronary Artery Disease on One-year Resource Utilization for Patients Admitted with Potential Acute Coronary Syndromes

Reduction in emergency department (ED) overcrowding is a major Joint Commission on the Accreditation of Healthcare Organizations (JCAHO) initiative. One major source of ED overcrowding is patients waiting for telemetry beds. OBJECTIVE: To determine whether, in patients admitted with a potential acute coronary syndrome, a negative evaluation for underlying coronary artery disease would reduce ED and hospital revisits over the subsequent year compared with patients who did not receive an evaluation for underlying coronary artery disease. METHODS: Nine hundred ninety-nine consecutive patients admitted for potential acute coronary syndromes through the ED during a one-year period were screened for inclusion. Patients who had a negative evaluation for underlying coronary disease were compared with patients who were not evaluated for underlying coronary artery disease for subsequent ED visits, hospital admissions, and cardiac resource utilization over the year following the index visit via a health system-wide computerized record review. Patients with positive tests or biomarkers at the index visit were excluded. Each repeat visit was rated as "potentially cardiac" or "noncardiac." Results of echocardiograms, stress tests, and catheterizations and information about in-hospital deaths were obtained. RESULTS: Six hundred ninety-two patients met the inclusion criteria: 556 patients received no evaluation for underlying coronary artery disease, 116 had a negative stress test, and 20 had a negative cardiac catheterization during the index visit. Patients with no evaluation for underlying coronary artery disease and patients with a negative evaluation had similar likelihoods of a repeat ED visit (negative test 39.0% vs. no test 40.3%; p = 0.85) and repeat hospital admission (28.7% vs. 31.5%; p = 0.61). The rates of a potentially cardiac-related ED visit (21.3 vs. 23.4%; p = 0.65) and hospital admission (17.7% vs. 20.7%; p = 0.48) were not significantly different. The two populations had similar utilization rates of echocardiograms, stress tests, and catheterizations (p > 0.70 for all). CONCLUSIONS: For patients admitted to the authors' institution with a potential acute coronary syndrome, there was no association between a negative evaluation for underlying coronary artery disease and overall or potentially cardiac ED visits, admissions, or cardiac resource test utilization over the year following the index visit.     

A case presentation of Sweet's syndrome and discussion of life-threatening dermatoses

Sweet first described acute febrile neutrophilic dermatosis in 1964. Since then, more than 425 cases of this typically benign, steroid-responsive disease have been recorded. Although often associated with myelodysplastic syndromes or hematologic malignancies, Sweet's syndrome has also been related to pregnancy, autoimmune disorders, and many drug therapies. Although it is not typically an acutely life-threatening illness, there is a potential for significant pulmonary involvement and respiratory compromise. Additionally, emergency physicians should be aware of this unusual disease and its frequent association with systemic illnesses. We report the first case of Sweet's syndrome in the emergency medicine literature and present a review and discussion of several common life-threatening dermatoses.     

铁蛋白和血红蛋白与原发性不宁腿综合征的关系

目的 探讨血红蛋白和铁蛋白水平与原发性不宁腿综合征(RLS)发病之间的关系.方法 对35例原发性不宁腿综合征患者(RLS组)及对照组20例失眠患者的血红蛋白及铁蛋白水平进行检测并行统计学比较.结果 RLS组患者血清铁蛋白水平为(89.77±48.52)μg/L,对照组为(123.36±35.06)μg/L,RLS组较对照组血清铁蛋白水平低,差异有统计学意义(t=-2.713,P<0.01),而血红蛋白水平RLS组为(142.77±11.79)g/L,对照组为(139.05±12.33)g/L,2组相比差异无统计学意义(t=1.108,P>0.05).结论 血清铁蛋白的降低可能是原发性不宁腿综合征的危险因素之一,而血红蛋白水平可能与原发性不宁腿综合征无关.     

成人斯蒂尔病112例临床分析

目的:探讨成人斯蒂尔病(adult-onset Still's disease,AOSD)的临床特征、诊断、治疗与转归,以提高对该病的认识.方法:对112例AOSD的临床表现、实验室检查、治疗和预后等临床资料进行数理分析.结果:发热、关节痛、咽痛、皮疹和肌肉疼痛是AOSD患者最常见的5个症状.呼吸系统受累多见(33%),其中间质性肺疾病5例,其次为循环系统 (17%),消化系统 (7%),神经系统受累少见(4%).贫血72例(64%),白细胞增多92例(82%),中性粒细胞增多71例(63%),血小板增多53例(47%),ESR增快105例(94%),CRP升高92例(82%),ALT升高73例(65%),乳酸脱氢酶升高64例(57%),血清铁蛋白升高84%(59/70).仅7%的患者用非甾体抗炎药可控制病情,其余均需要加用肾上腺皮质激素(激素),需再加用1种免疫抑制药占44%,需要加用2种免疫抑制药占13%.随访62例患者,获长期缓解56%,病情呈慢性持续性16%,反复发作23%,死亡3例.结论:AOSD是一种以发热、皮疹、关节痛、白细胞增多为主要特征的自身免疫性疾病,预后良好,多数患者用激素治疗有效,但对以关节炎表现为主以及合并内脏损害的患者应尽早使用免疫抑制药,以控制病情.     

The impact of COVID-19 lockdown on infectious diseases epidemiology: The experience of a tertiary Italian Pediatric Emergency Department

IntroductionThe aim of this study was to describe the rate and types of community-acquired respiratory infections observed in a pediatric ED during the SARS-CoV-2 related lockdown in Italy and to compare data with the same period of previous year.MethodsA retrospective analysis of medical charts of patients arrived at the ED of Gaslini Children's Hospital from 10th March 2020 to 30th April 2019 and the same frame of 2020 were performed. We compared two groups by demographics, duration of fever before ED admission, triage code, number of patients hospitalized after ED evaluation. We calculated proportion and incidence rate for airborne infections, fever, and urinary tract infections (UTI), appendicitis, and gastroenteritis for control.Results1362 children arrived at the ED during the lockdown compared to 5628 in the same period of 2019 (−75,8%). No difference was noticed (27.7% vs 28.4%) in the total amount of infectious episodes. A significant reduction in rate of incidence and proportion were observed for upper respiratory tract infections (21,4% vs 28%), otitis (2,6% vs 16,2%), streptococcal infections (0,5% vs 5,2%) and bronchiolitis (2,1% vs 5,7%). Conversely, FUO (27,8 vs 11,1%), infectious mononucleosis (2,6% vs 0,4%), UTI (7,4% vs 2,9%) and appendicitis (6,8% vs 1,1%) significantly increased. Median time from the onset of fever and arrival in ED was significantly lower in 2020 group.ConclusionOur results demonstrated a reduction in community-acquired respiratory infections during the lockdown for COVID-19. The increase in rate of FUO and febrile conditions, together with the short time from fever onset and ED visit could be related to the fear for a SARS-CoV-2 infection.     

Is my baby normal? A review of seemingly worrisome but normal newborn signs,symptoms and behaviors

Infant patients are a unique challenge to emergency department (ED) physicians as the spectrum of normal infant signs, symptoms and behaviors are often difficult to differentiate from abnormal and potentially life-threatening conditions. In this article, we address some common chief complaints of neonates and young infants presenting to the ED, and contrast reassuring neonatal and young infant signs and symptoms against those that need further workup and intervention.     

Toxic shock syndrome

Toxic shock syndrome (TSS) is an exotoxin-mediated illness that occurs primarily in young menstruating women who use tampons. The syndrome ranges from a potentially fatal disease characterized by hypotension and failure in multiple organ systems to a less severe condition commonly misdiagnosed as a nonspecific viral illness or gastroenteritis. Physicians should recognize that an exanthematous, febrile illness that recurs during menstruation or that occurs primarily in the postoperative or postpartum period and in association with staphylococcal infections may be TSS even in the absence of requisite diagnostic criteria. Unless TSS can be excluded with reasonable certainty, appropriate cultures should be obtained, with treatment initiated presumptively. In all menstrual cases, women should be advised to avoid tampon use indefinitely.     

Outcomes in patients with an emergency department diagnosis of fever of unknown origin

Objective: To describe the outcomes in patients given an ED diagnosis of fever of unknown origin (FUO). Methods: A retrospective analysis of ED records linked to hospital morbidity, mortality and microbiology records of patients presenting to Western Australia’s teaching hospitals from July 2000 to July 2003. Results: There were 3218 presentations diagnosed with FUO, 2049 (63.7%) children (median age 1.8 years) and 1169 (36.3%) adults (median age 56.0 years). FUO accounted for 0.3% of adult and 1.5% of paediatric ED presentations. Overall, 1997 (62.1%, 95% confidence interval 60.4–63.8%) were admitted (82% adults vs 50.7% children; P < 0.001). Adults had a longer median length of stay than children (4 days vs 2 days; P < 0.001) and a higher proportion of positive blood cultures (admissions 15.1%vs 4.9%; P < 0.001) commonly with Escherichia coli. Streptococcus pneumoniae was the most common organism isolated from children. Of 3053 FUO index presentations, 338 (11.1%, 95% confidence interval 10.0–12.2%) re‐presented. Children were more likely to re‐present than adults (13.5% of 1959 vs 6.8% of 1094; P < 0.001). Conclusions: Fever of unknown origin is diagnosed less frequently in adults than in children. Adult patients are more likely to be admitted, have longer lengths of stay and have positive blood cultures. Although FUO is diagnosed infrequently in the ED, blood cultures remain useful in the evaluation of unexplained fever, particularly in adults as age increases.     

"Cotton fever": a benign febrile syndrome in intravenous drug abusers

Cotton fever is a benign, self-limited syndrome that may mimic sepsis in intravenous drug addicts. We present an illustrative case and a review of the literature. Serious illness such as pneumonia and infectious endocarditis must always be considered in febrile addicts. However, trivial illness accounts for 16% to 26% of such fevers. Recent evidence suggests that emergency physicians are able to diagnose trivial illness with 93% specificity in febrile adult drug addicts. Short-term observation units may be an alternative to hospital admission for febrile drug users with a presumptive diagnosis of trivial illness and in those in whom the diagnosis of cotton fever is entertained.     

Atypical adult-onset Still’s disease with an initial and sole manifestation of liver injury: A case report and review of literature

BACKGROUNDAdult-onset Still''s disease (AOSD) typically presents with a high spiking fever, polyarthritis, transient maculopapular rash, neutrophilic leukocytosis, and hepatosplenomegaly. It has a wide spectrum of clinical symptoms ranging from mild to severe, with extensive involvement of almost every organ. Although liver involvement in the form of increased hepatic enzymes and bilirubin is common, no AOSD case with liver involvement as the initial manifestation of AOSD has been reported. CASE SUMMARYA 35-year-old woman presented to the hepatology department with progressively worsening jaundice for one week. Liver chemistry tests revealed a significantly increased liver enzymes and bilirubin level. Given that the clinical examination was unremarkable, liver biopsy was considered because the patient had a history of AOSD 6 years ago. Liver histopathology revealed that most hepatic lobules were still recognizable. Fusional necrosis was observed around most central veins. A few bridging necrotic zones were also found. Infiltration of multiple plasma cells were observed in the necrotic zone, and the reticular scaffold was still expanded. Additionally, no obvious fibrosis was observed in the portal area. Mild mixed inflammatory cell infiltration was noted in the interstitium of the portal area. Further examination was unremarkable except for a remarkably high level of ferritin. Collectively, a presumptive diagnosis of liver injury secondary to AOSD was made. The hepatic involvement responded well to glucocorticoid treatment.CONCLUSIONThis case highlights that hepatic involvement as an initial and sole manifestation could be a pattern of relapsed AOSD. The diagnosis of AOSD should be considered in the case of nonresolving liver injury after the exclusion of common etiologies for liver diseases. A liver biopsy can be useful for the differential diagnosis of liver injury associated with AOSD.     

Interventional antimicrobial therapy in febrile neutropenic patients.

In febrile neutropenic patients, prompt empiric antimicrobial intervention is mandatory. Numerous studies have demonstrated the benefit of broad-spectrum beta-lactams active against Gram-negative aerobes as well as against streptococci and Staphylococcus aureus in this setting. With this interventional strategy, a reduction of infection-related mortality to < or = 10% of patients undergoing intensive remission induction or consolidation chemotherapy could be obtained. Thereby, subgroups of patients have been identified who require an empiric modification of antimicrobial treatment, e.g., patients with catheter-related infections, with pulmonary infiltrates, or with unexplained fever (FUO) not responding to first-line antibacterials. In two consecutive, prospectively randomized trials conducted by the German Paul Ehrlich Society it could be shown that empiric antifungal therapy is beneficial for second-line treatment in patients with persistent FUO and improves first-line treatment results in patients with lung infiltrates. The addition of glycopeptides, however, should be restricted to patients with catheter-related infections due to coagulase-negative staphylococci or with infections due to multiresistant Gram-positive pathogens.     

Chronic facial pain from psychiatric point of view - differential diagnosis and therapeutic strategies

Chronic facial pain may cause diagnostic and therapeutic difficulties, thus demanding a complete interdisciplinary consultation. As in 20 to 25% of patients with a nociceptive or primary neuropathic pain a psychiatric comorbidity is expected, it is necessary to include psychiatric and psychosomatic examinations. Every 8th male and every 4th female pain patient has the statistical risk of having a depression at the same time. Depression may develop as a consequence of pain, but may also be the primary basis for a pain syndrome. An independent coincidence is possible as well.Besides affective disorders, persistent somatoform pain syndromes, syndromes of conversion, hypochondriac or artificial disorders and pain in psychosis have to be excluded in patients presenting with chronic facial pain.In depression, persistent somatoform pain syndromes, atypical facial pain but also when dysfunctional illness beliefs and coping mechanisms are present, cognitive-behavioral therapy should be offered to the patient. Motivation to psychotherapy may be difficult, especially in patients relying exclusively on physical illness beliefs. The physician should develop and extend the physical illness beliefs together with the patient to a model that includes biological as well as psychosocial factors.Tricyclic antidepressants may be indicated not only in depression, but also in chronic pain due to the analgetic effects of these drugs. The choice of drug therapy should conform to the main complaints of depression and accompanying illnesses.Indication of antidepressants or neuroleptic drugs in somatoform pain syndromes is still unclear. Their potential slight effect may simply be due to the high comorbidity between somatoform pain syndromes and depression.