Pain In Systemic Lupus Erythematosus Research Articles

Clinical features, imaging findings, and outcomes of acute abdominal pain in systemic lupus erythematosus: comparing mesenteric vasculitis, non-mesenteric vasculitis, and surgical conditions.

To determine the spectrum, clinical features and outcomes in systemic lupus erythematosus (SLE) patients with acute abdominal pain (AAP). Medical records of SLE patients in a lupus cohort from January 1987 to June 2023 were reviewed. Patients with AAP requiring hospitalization were identified and categorized into 3 groups: lupus mesenteric vasculitis (LMV), non-LMV, and surgical AAP. Each AAP episode represented one patient. Of 1,538 patients in the cohort, 62 (4.03%) had 93 episodes of AAP. After exclusion, 31 patients had 39 LMV episodes, and 30 had 40 non-LMV episodes (19 due to surgical AAP). Seventy-six of the 79 AAP episodes (96.20%) were in females, with a mean ± SD age and median (IQR) disease duration of 36.76 ± 13.60 years and 6 (2, 9) years, respectively. Patients in the LMV group had more fever, nausea and vomiting, and diarrhea than those in the non-LMV group. They also had more small bowel involvement, bowel wall thickening, target water enhancement signs, mesenteric vessels engorgement and mesenteric fat cloudiness, and higher SLE disease activity. These differences were more pronounced when compared to the surgical AAP group. Treatment with corticosteroids and immunosuppressive drugs gave favorable outcomes in the LMV group. Two of 40 (5.00%) non-LMV AAP patients died, of which 1 (5.26%) was in the surgical AAP group. LMV was common among SLE patients admitted for AAP. LMV usually presented with fever, gastrointestinal dysmotility symptoms, diffused abdominal pain, together with evidence of active disease. Localized abdominal pain with peritoneal signs favored surgical AAP. Key Points • Lupus mesenteric vasculitis is common among SLE patients presenting with acute abdominal pain. Its presence often associates with gastrointestinal symptoms together with other clinical manifestations of SLE • The signs in abdominal computed tomography findings are not specific and could be observed in other causes of abdominal pain in SLE. Interpretation of these signs should be cautionary and accompanied by history taking and physical abdominal findings • Treatment of lupus mesenteric vasculitis with corticosteroids alone, or in combination with immunosuppressive drugs, usually results in good outcomes.

The experience and implications of pain in systemic lupus erythematosus: A qualitative interview study focusing on the patient's perspective.

Pain is one of the most frequently reported symptoms and often one of the first subjective symptoms in patients with systemic lupus erythematosus (SLE). A previous study indicated that most patients with SLE reported low levels of SLE-related pain. However, a subgroup of patients reported high levels of pain ≥40mm (0-100mm) and had a substantial symptom burden in terms of fatigue, anxiety, depression, and reduced health-related quality of life. Thus, there is a need to elucidate the implications of high levels of pain in everyday life. This study explored the patient's experiences and implications of SLE-related pain in daily life and the support requested from healthcare providers. A total of 20 patients, previously reported high levels of SLE-related pain intensity measuring ≥40mm (0-100mm) in a research context at one or two occasions participated in individual semi-structured interviews, which were transcribed and analysed with content analysis. The interviews revealed four main categories and 13 generic categories. SLE-associated pain was described by its multifaceted nature, exhibiting longstanding, unpredictable, migrating, and various physical sensations. The pain entailed multidimensional consequences, restricting everyday life by interfering with roles and relationships and causing various emotions, including existential thoughts. The informants used comprehensive strategies to deal with the pain, including their inner resources, support from family and significant others, and pharmaceuticals and relieving treatments. They expressed the need for security and acknowledgement, which involved individualized support and accessibility of healthcare. This study provides comprehensive insights into the nature and multifaceted impact of SLE-related pain in different dimensions of the informants' daily lives. Except for medications the informants used several strategies, including their inner resources and support from family and others, to manage the pain. Support requested from healthcare providers by the informants included understanding, compassion, individualized care and accessibility.

The Utility of Ultrasound in Evaluating Joint Pain in Systemic Lupus Erythematosus: Looking beyond Fibromyalgia.

Systemic lupus erythematosus (SLE) is a complex autoimmune condition with varied clinical presentations, and musculoskeletal pain is one of the most commonly associated symptoms. However, fibromyalgia (FM) is a prevalent co-existing condition in SLE patients that can also cause widespread pain, and in patients with both conditions, it is often difficult to distinguish the underlying cause of musculoskeletal pain and provide optimal therapy. A retrospective cohort study was conducted including all adult SLE patients who received musculoskeletal ultrasound (US) examinations for joint pain at the Ohio State University Wexner Medical Center between 1 July 2012, and 30 June 2022. Binary and multiple logistic regression analyses were performed to determine predictors of US-detected inflammatory arthritis as well as improved musculoskeletal pain. A total of 31 of 72 SLE patients (43.1%) had a co-existing diagnosis of FM. In binary logistic regression, a co-existing diagnosis of FM was not significantly associated with US-detected inflammatory arthritis. In multiple logistic regression analysis, clinically detected synovitis was significantly associated with US-detected inflammatory arthritis (aOR, 142.35, p < 0.01), and there was also a weak association with erythrocyte sedimentation rate (ESR) (aOR 1.04, p = 0.05). In separate multiple logistic regression analysis, US-guided intra-articular steroid injection was the only predictor of improved joint pain at follow-up visit (aOR 18.43, p < 0.001). Musculoskeletal US can be an effective modality to detect inflammatory arthritis as well as to guide targeted intra-articular steroid injection to alleviate joint pain in SLE patients with or without FM.

Lupus enteritis as a flare up of systemic lupus erythematosus- a case report and review

Systemic lupus erythematosus (SLE) generally affects young to middle-aged women, commonly presenting as a triad of fever, rash, and joint pain. Abdominal pain is a common symptom in patients with SLE. The leading causes of abdominal pain in SLE are lupus enteritis, pancreatitis, pseudo-obstruction, acalculous cholecystitis, mesenteric thrombosis, hepatic thrombosis, medications like (NSAIDS, MMF, steroids, HCQ), colon perforation. The incidence of abdominal pain in patients with SLE ranges from 8-40%, and the commonest cause is lupus enteritis. The following case describes a young woman presenting with lupus enteritis as a manifestation of SLE, the importance of early disease recognition, utilities of abdominal computed tomography (CT) in diagnosis, and current treatment protocols for lupus enteritis.

A nomogram to predict the risk of lupus enteritis in systemic lupus erythematosus patients with gastroinctestinal involvement.

BackgroundLupus enteritis (LE), a main cause of acute abdominal pain in systemic lupus erythematosus (SLE) patients, is a serious and potentially fatal complication. This study aimed to identify clinical serological indicators to establish a nomogram to assess LE in SLE patients with gastrointestinal manifestations.MethodsThe clinical and laboratory data of SLE patients with gastrointestinal manifestations that were hospitalized in the West China Hospital from January 2010 to January 2020 were retrospectively analyzed. The least absolute shrinkage and selection operator logistic regression model was used to select potentially relevant features. Subsequently, a nomogram was developed using multivariable logistic analysis. The performance of the nomogram was evaluated using a receiver operating characteristic curve, a calibration curve, and decision curve analysis (DCA).FindingsWe included a total of 8,505 SLE patients, of which 251 had experienced gastrointestinal manifestations. The patients were randomly divided into training (n = 176) and validation (n = 75) groups. The LRA (LE Risk Assessment) model consisted of 11 significantly associated variables, which included complement 4, antineutrophil cytoplasmic antibody, albumin, anion gap, age, d-dimer, platelet, serum chlorine, anti-Sjögren's-syndrome-related antigen A, anti-ribosomal P protein, and anti-ribonucleoprotein. In the training and validation cohorts, the areas under the curve were 0.919 (95% confidence interval [CI]: 0.876–0.962) and 0.870 (95% CI: 0.775–0.964), respectively. The nomogram demonstrated excellent performance in the calibration curve and DCA.InterpretationThe LRA model exhibits good predictive ability in assessing LE risk in SLE patients with gastrointestinal manifestations.

Protocolo diagnóstico del dolor abdominal en un paciente con lupus eritematoso sistémico

Abdominal pain in systemic lupus erythematosus (SLE) is a common symptom. Forty percent of patients will present with abdominal pain during the course of the disease, although it may also be an initial manifestation of undiagnosed disease. The origin of the pain is diverse, as it can be similar to that of the general population or be the result of activity specific to SLE or a concomitant disease. As part of the initial approach, the need for urgent surgery should be ruled out; otherwise, a complete work-up should be undertaken, including clinical history taking with physical examination, laboratory tests, phase reactants and SLE activity parameters, and imaging on suspicion. A high index of suspicion should be kept for infection and neoplasia, and immunosuppressive treatment instituted once these possibilities have been discounted.

The Problem of Pain in Systemic Lupus Erythematosus: An Explication of the Role of Biopsychosocial Mechanisms.

To define biopsychosocial mechanisms of pain that go above and beyond disease activity and organ damage in systemic lupus erythematosus (SLE). We conducted a cross-sectional analysis of patient-reported data in a population-based registry of 766 people with SLE. Predictors of pain intensity and interference were examined using hierarchical linear regression. We built 2 main hierarchical regression models with pain intensity and interference as outcomes, both regressed on disease activity and organ damage. For each model, we sought to establish the relationship between pain outcomes and the primary exposures using sequential steps comprising the inclusion of each construct in 6 stages: demographic, socioeconomic, physical, psychological, behavioral, and social factors. We also conducted sensitivity analyses eliminating all overt aspects of pain in the disease activity measure and reestimated the models. Disease activity and organ damage explained 32-33% of the variance in pain intensity and interference. Sociodemographic factors accounted for an additional 4-9% of variance in pain outcomes, whereas psychosocial/behavioral factors accounted for the final 4% of variance. In the sensitivity analyses, we found that disease activity and organ damage explained 25% of the variance in pain outcomes. Disease activity only explained 33% of the variance in pain outcomes. However, there was an attenuation in these associations after accounting for psychosocial/behavioral factors, highlighting their roles in modifying the relationship between disease activity and pain. These findings suggest that multilevel interventions may be needed to tackle the negative effect of pain in SLE.

Physiopathology of pain in systemic erythematosus lupus.

Systemic lupus erythematosus is an autoimmune disease that promotes chronic inflammation, with periods of activation and remission. Pain is commonly one of the first symptoms reported by patients with lupus. It interferes with patients' quality of life, leading to a decrease in strength and difficulty in performing daily activities. Given the chronic nature of the disease, the high prevalence of pain and its negative impact on the quality of life of patients with lupus, the present literature review study aims to explain the pathophysiology of pain in systemic lupus erythematosus and the implications of the chronic process and contributing factors. During periods of systemic lupus erythematosus activity, pain is a significant symptom. Despite this, several studies show that severe pain can occur in patients with in mild to moderate disease activity. Also, in the early stages of the disease, the pain may be accentuated by the greater activity of the disease. However, even when advanced disease is under control, there can be comorbidities and accumulated damage that can also cause high levels of pain. This sensitivity is due to the overlap of primary, secondary, and tertiary pain pathologies, which feedback and make this symptom one of the main concerns of patients with lupus. Understanding the pathophysiology of pain in systemic lupus erythematosus, as well as its chronification and contribution factors, is essential to identify effective therapeutic alternatives in these patients at each stage of pain pathology (primary, secondary, and tertiary).

CLINICAL AND IMMUNOLOGICAL ASPECTS OF CHRONIC HEADACHES IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Objective: to study the clinical and immunologica features of the manifestation of chronic pain in systemic lupus erythematosus (SLE) patients with neurological symptoms.Methods. We examined 30 healthy individuals and 38 patients with SLE. Beck’s depression questionnaire was used to assess the presence of depressive symptoms. Antibodies to adenosine deaminase (anti-ADA), β2-glycoprotein-I-dependent antibodies to phospholipids of the IgG class (anti-FL) and antibodies to double-stranded DNA (anti-dsDNA) were determined in the serum of patients with SLE. Doppler sonography of the brachiocephalic arteries was performed for all patients with SLE.Results. Complaints about the presence of headaches of varying severity presented 35 people (92.1%). Migraine was recorded in 63.2% patients with SLE. Doppler ultrasound in patients with SLE with chronic headaches in 66.7% of cases showed signs of reduced blood flow in the arteries of the vertebrobasilar basin, which may indicate chronic brain ischemia. Signs of depressive disorder of varying severity were found in 36.8% of patients with SLE, and in patients with neurological disorders, moderate (p = 0.027) and severe (p = 0.041) depression were more often detected. Elevated levels of anti-ADA were found in 36.8%, and anti-FL in 44.7% of patients with SLE. It was noted that “migraine-like” manifestations of chronic pain syndrome were more common in the group of patients with SLE, who had a combined increase in anti-ADA and anti-FL (χ2 = 4.5; p = 0.024). Since a certain part of ADA is concentrated in the plasma membranes of vascular and platelet endothelium cells, it can be assumed that there is a conformational effect of anti-ADA on the β2-glycoprotein-I, leading to increased synthesis of anti-FL and undesirable activation of coagulation cascade in vessels.Conclusion. The combination of severe chronic headache with high levels of anti-ADA and anti-FL can precede the development of stroke and transient ischemic attacks, which emphasizes the need for additional immunological examination of patients with SLE with neurological symptoms.

Acute Abdominal Pain in Systemic Lupus Erythematosus: A Broad Differential

Acute Abdominal Pain in Systemic Lupus Erythematosus: A Broad Differential

Chronic pain and impaired glial glutamate transporter function in lupus-prone mice are ameliorated by blocking macrophage colony-stimulating factor-1 receptors.

Systemic lupus erythematosus (SLE) is a multi-organ disease of unknown etiology in which the normal immune responses are directed against the body's own healthy tissues. Patients with SLE often suffer from chronic pain. Currently, no animal studies have been reported about the mechanisms underlying pain in SLE. In this study, the development of chronic pain in MRL lupus-prone (MRL/lpr) mice, a well-established lupus mouse model, was characterized for the first time. We found that female MRL/lpr mice developed thermal hyperalgesia at the age of 13weeks, and mechanical allodynia at the age of 16weeks. MRL/lpr mice with chronic pain had activation of microglia and astrocytes, over-expression of macrophage colony-stimulating factor-1 (CSF-1) and interleukin-1 beta (IL-1β), as well as suppression of glial glutamate transport function in the spinal cord. Intrathecal injection of either the CSF-1 blocker or IL-1 inhibitor attenuated thermal hyperalgesia in MRL/lpr mice. We provide evidence that the suppressed activity of glial glutamate transporters in the spinal dorsal horn in MRL/lpr mice is caused by activation of the CSF-1 and IL-1β signaling pathways. Our findings suggest that targeting the CSF-1 and IL-1β signaling pathways or the glial glutamate transporter in the spinal cord is an effective approach for the management of chronic pain caused by SLE.

Severe Lupus Enteritis Responding to High Dose Steroids, Cyclophosphamide and Intravenous Immunoglobulin: A Case Report

Introduction: Lupus enteritis is a rare cause of abdominal pain in systemic lupus erythematosus (SLE) patients. It is a very serious complication that can lead to bowel perforation. It is generally steroid responsive. In this report we present a case of steroid resistant lupus enteritis which resolved with intravenous immunoglobulin and cyclophosphamide. Case report: A 31-years-old female who is known to have SLE of two years' duration of only joint involvement. She presented with increasing abdominal distention. She was lethargic and her abdomen was diffusely rigid. CT scan of abdomen and pelvis was showed diffuse abdominal ascites with few small foci of gas in the fluid with increased attenuation of mesenteric fat. She was taken emergently to surgery for a concern of peritonitis. There were patchy areas of ischemia throughout the small and large bowels. It was decided to leave the patient with an open abdomen and come back for further looks to determine bowel viability. CT scan angiography of the abdomen failed to show any vascular occlusion. Labs showed a CRP of 13.7 mg/dL, C3 of 37 mg/dL, C4 of 8 mg/dL. SLE enteritis was considered as the most likely cause and she was started on high-dose methylprednisone 1 g/day IV for a total of 5 days. Re-exploration was done on the next day and showed similar findings compared to first time. The abdomen was irrigated and closed. On the third day post admission, the patient developed worsening lactic acidosis, anuria, transaminitis, and features of DIC. She was taken emergently to surgery. Again, the bowel appeared unchanged with no obvious perforation. The abdomen was left open with the decision of doing re-exploration every 48 hours. As the patient had been on the high dose steroids; intravenous cyclophosphamide 1 g for one dose and intravenous immunoglobulin at 500 mg/kg for four days were added. The bowel started to develop gradual improvement of the patchy ischemic areas on successive laparotomies and on the 9th day post-admission it looked very close to normal and the abdomen was closed. She was extubated on the 12th day post-admission. She demonstrated gradually improving mentation and alertness levels and was discharged to rehabilitation 3 weeks later. Conclusion: Lupus enteritis should be always considered in an SLE patient presenting with abdominal pain. Diagnosis is based on typical CT scan findings. It is steroid-responsive. Immunosuppressive agents should be considered in unresponsive cases.

Pain and systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous clinical manifestations involving virtually the entire body. The pain in SLE can have different causes. The SLE classification criteria include mainly the musculoskeletal manifestations of pain, which are commonly reported as initial symptoms of SLE, such as arthralgia, arthritis and/or myalgia. Chronic widespread pain, which is typical of fibromyalgia (FM), is frequently associated with SLE. The aim of this review is to describe widespread pain and fatigue in SLE, and the association of SLE and FM. Although secondary FM is not correlated with the disease activity, it may interfere with the daily activities of SLE patients. Therefore it is necessary to identify its symptoms and treat them promptly to improve the quality of life of patients. In conclusion, it is essential to identify the origin of pain in SLE in order to avoid dangerous over-treatment in patients with co-existing widespread pain and FM.

SAT0013 Lupus Enteritis: Clinical Characteristics and Risk Factors Associated with Recurrence

BackgroundLupus enteritis is a severe complication of systemic lupus erythematosus (SLE) cases with acute abdominal pain. Some studies have identified clinical characteristics and risk factors for relapse in lupus enteritis...

Clinical Features and Associated Factors of Abdominal Pain in Systemic Lupus Erythematosus

Clinical Features and Associated Factors of Abdominal Pain in Systemic Lupus Erythematosus

Clinical Features and Associated Factors of Abdominal Pain in Systemic Lupus Erythematosus

To evaluate the clinical characteristics of systemic lupus erythematosus (SLE)-induced abdominal pain in a cohort in South China and identify the risk factors for SLE-induced abdominal pain. This is a retrospective cohort study of SLE patients with complaint of abdominal pain admitted to the first affiliated university hospital of Sun Yat-sen University between 2002 and 2011. Demographic information, clinical features, laboratory findings, SLE Disease Activity Index, and imaging characteristics were documented. Of the 3823 SLE patients reviewed, 213 patients complained of abdominal pain and 132 cases were considered SLE-induced. The most common causes were lupus mesenteric vasculitis (LMV; 73.5%, 97/132) and lupus pancreatitis (LP; 17.4%, 23/132). Other causes included appendicitis, acute gastroenteritis, and peritonitis. Univariate and multivariate logistic regression analysis indicated the European Consensus Lupus Activity Measurement (ECLAM) score was significantly associated with lupus-induced abdominal pain (OR = 1.858, 95% CI: 1.441-2.394, p < 0.001), LMV (OR = 1.713, 95% CI: 1.308-2.244, p < 0.001), and LP (OR = 2.153, 95% CI: 1.282, 3.617, p = 0.004). The serum D-dimer level (OR = 1.004, 95% CI: 1.002-1.005, p < 0.001) was a strongly associated factor for lupus-induced abdominal pain. Moderate and large amounts of ascetic fluid was significantly associated with lupus-induced abdominal pain and LMV. Elevated liver enzymes was a risk factor for LP (OR = 34.605, 95% CI: 3.591-333.472, p = 0.002). LMV and LP were the leading causes of SLE-induced abdominal pain. The serum D-dimer was a strongly associated factor for lupus-induced abdominal pain. ECLAM score was a reliable index in assessment of SLE-associated abdominal pain. Elevated liver enzymes, and moderate or large amounts of ascites, were positively associated with lupus-induced abdominal pain.

Acute abdominal pain in systemic lupus erythematosus

A 28-year-old woman with a history of systemic lupus erythematosus and antiphospholipid antibody syndrome presented with acute, severe, left-sided abdominal pain, hematemesis, and hematochezia. On physical examination, there was significant left-sided abdominal tenderness with guarding. Laboratory test analysis revealed leukocytosis of 30,800/mm3 (normal 4000-10,8000/mm3), anemia with a hemoglobin of 9.6 g/dL (12-16 g/dL), elevated C-reactive protein level of 140 mg/dL (<3 mg/dL), and a normal serum lactate level of 1.1 mmol/L (<2 mmol/L).

Colitis Associated With Active Systemic Lupus Erythematosus Successfully Treated With Rituximab

Intestinal vasculitis is a serious and often underrecognized complication of systemic lupus erythematosus (SLE) usually managed with high-dose corticosteroids. We report a case of corticosteroid refractory colitis (likely due to intestinal vasculitis) that achieved remission with a single cycle of high-dose rituximab. This has not previously been described to our knowledge. A 46-year-old woman with SLE presented with 1-week history of bloody diarrhea, abdominal pain, worsening joint pains, fatigue, and mouth ulcers. There was evidence of increased SLE activity with low C4, raised anti-double-stranded DNA antibody, leukopenia, and anemia. Colonoscopy showed active pancolitis (confirmed histologically). A diagnosis of acute colitis associated with active SLE was made, and she received intravenous methylprednisolone 1 g daily for 3 days followed by 60 mg oral prednisolone daily. She continued to have symptomatic colitis with worsening anemia requiring frequent blood transfusions. She then received 2 doses of rituximab 1 g (750 mg/m(2)) 2 weeks apart that led to improvement in colitis symptoms and SLE disease activity over the next 4 weeks. A repeat colonoscopy (and histology) confirmed good resolution of colitis. Six months later, she continued to be in remission with low-dose prednisolone. Intestinal vasculitis should be considered as a possible cause of acute abdominal pain in SLE as early recognition and treatment can improve long-term survival. Rituximab can be an effective alternative for patients refractory to conventional treatment.

Differences in Disease Features Between Childhood-Onset and Adult-Onset Systemic Lupus Erythematosus Patients Presenting with Acute Abdominal Pain

Abdominal pain in systemic lupus erythematosus (SLE) patients has rarely been analyzed in pediatric populations. We planned to investigate the potential differences between childhood-onset and adult-onset SLE patients who were hospitalized because of acute abdominal pain. A retrospective study including 23 childhood-onset SLE patients with 38 admissions and 88 adult-onset SLE patients with 108 admissions from 1999 to 2008 were conducted in our hospital. All of them had the chief complaint of diffuse abdominal pain. The etiologies of acute abdominal pain in adult-onset SLE patients were more diverse than childhood-onset SLE patients. The most common cause of acute abdominal pain in SLE patients was lupus mesenteric vasculitis (LMV) (18.5%), followed by acute gastroenteritis (14.4%), pancreatitis (10.3%), appendicitis (7.5%), and cholecystitis (6.2%). Compared with adults, children were admitted more often due to LMV (31.6% versus 13.9%; P = 0.016), had more frequently recurrent episodes (39.1% versus 14.8%; P = 0.009), and were more often treated with immunosuppressive agents (31.6% versus 7.4%; P < 0.001) at the time of admission. The overall case fatality rate of acute abdomen in SLE patients was 9.4%. The extra-gastrointestinal symptoms, laboratory evaluation, disease activity, and organ damage measured by the SLE Disease Activity Index and outcomes were comparable between children and adults. Various etiologies of acute abdominal pain should be considered in SLE patients. LMV is the most common cause of acute abdomen in childhood-onset SLE patients with low mortality and morbidity provided by prompt diagnosis and timely administration of high-dose intravenous corticosteroids after excluding real surgical abdomen.

L’entérite : une manifestation peu fréquente et corticosensible du lupus érythémateux systémique

Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis. We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded. Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.