[Remission of steroid-resistant Stills disease treated with anakinra, evidenced by FDG-PET/CT examination: case report]. / Remise steroid-rezistentní Stillovy nemoci pri lécbe anakinrou dokumentovaná FDG-PET/CT vysetrením: kazuistika.

After elimination of infectious causes, neoplastic causes and the systemic autoimmune disease of connective tissue, a patient with high fevers over 39 °C was diagnosed with Stills disease. High doses of prednisone led to resolution of symptoms, however after reducing the doses of prednisone to 15 mg, high fevers over 39 °C returned, as well as joint pains. The high doses of prednisone led to decompensation of diabetes mellitus even with 4 daily insulin dosages. Therefore it was proceeded to regular subcutaneous administration of anakinra once a day. Anakinra enabled the reduction of prednisone to as much as the currently administered 2.5 mg a day, but it has not so far allowed for removing glucocorticoids from the treatment completely. Activity of the disease is shown by the findings within the FDG-PET/CT examination. At the time of maximum activity of the disease there was distinct lymphadenopathy with pathological accumulation of FDG visible as well as increased accumulation of FDG in the hematopoietic bone marrow. As the disease activity decreased, the size of nodules regressed and FDG accumulation in both the lymphatic nodes and bone marrow declined. FDG-PET/CT is a suitable method for monitoring the activity of Stills disease.Key words: anakinra - Adult-onset Stills disease.

Atypical form of Adult-onset Still's Disease with Distal Interphalangeal Joints Involvement.

BACKGROUND: A distal interphalangeal (DIP) joint involvement in the adult-onset Still's disease (AOSD) has been described in some publications but is rarely reported to be severe. We report severe DIP joints damages in a young patient with AOSD. CASE REPORT: A 22 years old patient presented to our department complaining of inflammatory joints pain associated with prolonged fever and cutaneous rash. Physical examination identified polyarthritis and hepatosplenomegaly but no lymphadenopathies. After an extensive screening for neoplastic, infectious or hematologic diseases, the patient was finally diagnosed with AOSD. Treatment based on corticosteroids was then initiated with a good response on systemic signs. However, the patient continued to have recurrent arthritis affecting wrists and proximal interphalangeal joints. A Few years later, he developed a severe and disabling DIP arthritis with signs of joint destruction on conventional radiographs and MRI. Despite the initiation of methotrexate with optimal dosage, the patient continued to have polyarticular flares. The combination of methotrexate and sulfasalazine was responsible for drug-induced hepatotoxicity and this treatment was stopped. Anti-TNFα treatment was then indicated as general signs improved but severe joints damage persisted. Unfortunately, and due to healthcare system considerations, the patient was not able to benefit from TNFα inhibitors, and remained on methotrexate treatment only. Conculsion: The distal destructive arthritis during AOSD is rare and controversial. Our patient had a severe form with resistance to conventional therapies.

Imaging Characteristics of Chemotherapy Related Adult-Onset Still Disease.

A 60-year-old man with lymphoma completed chemotherapy on October 21, 2016, with complete remission. He then received rituximab maintenance therapy. Since March 2017, he has had progressive fatigue, myalgias, rash, weight loss, diarrhea, and recurrent low-grade fever. Subsequent bone marrow biopsy and FDG PET/CT demonstrated no active lymphoma. An In-white blood cell scan showed abnormal tracer uptake on 20-hour postinjection, but not on 3-hour postinjection images, including innumerable skeleton muscle foci, multiple cutaneous foci, and persistent diffuse increased uptake in the lungs. Diagnosis of adult-onset Still disease was made accordingly. The patient's cytopenia was deemed a chemotherapy-related adverse effect.

Imaging characteristics of adult onset Still's disease demonstrated with 18F-FDG PET/CT.

The diagnosis of adult onset Still's disease (AOSD) is non­specific, and requires the exclusion of other diseases including infectious, inflammatory and malignant diseases. The current study aimed to summarize the imaging characteristics of fluorodeoxyglucose (18F­FDG) positron emission tomography (PET)/computerized tomography (CT) in patients with AOSD. The 18F­FDG PET/CT characteristic observations of 32 patients with definite AOSD were retrospectively reviewed based on visual interpretation and the semi­quantitative index of standard uptake value of maximum (SUVmax). Among 32 patients, no normal case was observed. Abnormal FDG accumulation by the spleen, bone marrow and lymph nodes was the main observation of the PET/CT images. Totals of 27 (84.4%) and 26 cases (81.3%) were identified with diffusely elevated FDG uptake by the spleen and bone marrow, respectively, and the average SUVmax was 4.2±1.1 and 4.6±0.6, respectively. A total of 20 cases (62.5%) showed lymphadenopathy with FDG uptake, with the range of SUVmax from 2.2­13.9. In addition, 7 patients (21.9%) were observed to exhibit effusion without FDG uptake, 1 case presented with abnormal FDG uptake by the skin, and another by the right shoulder joint. In addition, no abnormally elevated FDG uptake by the liver or large vessels was observed. Due to non­specific imaging features, 18F­FDG PET/CT could not be directly helpful in diagnosing AOSD. However, 18F­FDG PET/CT serves important roles in evaluating the involved extent of AOSD, and guiding the biopsy of lymph nodes, bone marrow or other tissues, which may aid in the development of novel clinical management strategies.

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease: Case report and review of literature.

RATIONALE: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure. PATIENT CONCERNS: A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema. Five days later, the patient presented with dyspnea, hypotension, and anuria. Anasarca developed with massive pleural effusion, ascites, and systemic edema, resulting in an increase of 47 kg in body weight. DIAGNOSES: The patient was diagnosed as AOSD after infection, malignancy, hematologic disorders, and other autoimmune diseases were excluded. INTERVENTIONS: We administered tocilizumab, an IL-6 receptor inhibitor, intravenously in addition to cyclosporine, prednisolone, plasma exchange, and continuous hemodiafiltration. OUTCOMES: The patient's systemic condition improved. After stabilization by all medications, the patient was managed and responded to tocilizumab alone. To the best of our knowledge, this was the first case of severe SIRS complicating AOSD that was successfully treated with an anti- IL-6 receptor antibody. LESSONS: SIRS should not be overlooked in a patient with steroid-resistant AOSD and edema. Inhibitors of the IL-6 receptor can be used safely and effectively to control AOSD complicated with severe SIRS.

[Pseudo-adult Still's disease, anasarca, thrombotic thrombocytopenic purpura and dysautonomia: An atypical presentation of multicentric Castleman's disease. Discussion of TAFRO syndrome]. / Pseudo-maladie de Still, anasarque, microangiopathie thrombotique et dysautonomie : présentation atypique d'une maladie de Castleman multicentrique. Discussion du syndrome TAFRO.

INTRODUCTION: Multicentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia. CASE REPORT: We report a 32-year-old woman with an association of oligoanuria, anasarca, thrombotic microangiopathy with features compatible with adult-onset Still disease. The outcome was initially favorable with corticosteroids, immunoglobulins and plasmapheresis but with the persistence of relapses marked by severe autonomic syndrome and necessity of high dose corticosteroids. The diagnosis of mixed type Castleman's disease, HHV8 and HIV negative, was obtained four years after the onset of symptoms by a lymph node biopsy. The outcome was favorable after tocilizumab and corticosteroids but tocilizumab had to be switched to anakinra to ensure a proper and long-lasting control of the disease. CONCLUSION: Our patient partially fits the description of TAFRO syndrome (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly), a MCM rare variant, recently described in Japanese patients.

18F-FDG PET/CT in patients with adult-onset Still's disease.

(18)F-fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG PET/CT) has become useful for the detection and diagnosis of inflammatory conditions, including rheumatic diseases, immunoglobulin (Ig) G4-related disease and giant cell arteritis. However, few articles based on small sample sizes (n = 7) diagnosed as adult-onset Still's disease (AOSD) have been published. The study aim was to observe the reliable characteristics and usefulness of (18)F-FDG PET/CT for the evaluation of consecutive patients with AOSD. Eligible patients were selected from among those who had undergone (18)F-FDG PET/CT between May 2007 and June 2014. Twenty-six consecutive AOSD patients were recruited retrospectively according to criteria set by Yamaguchi et al. All patients underwent evaluation by (18)F-FDG PET/CT. The characteristics and usefulness of (18)F-FDG PET/CT for evaluation of consecutive patients with AOSD were evaluated. All 26 patients had (18)F-FDG-avid lesion(s) related to their particular disease. Diffuse and homogeneous accumulation of (18)F-FDG was seen in the bone marrow (26/26; 100 %; maximum standardized uptake (SUVmax), 2.10-6.73) and spleen (25/26; 96.15 %). The SUVmax of affected lymph nodes was 1.3-9.53 (mean ± SD, 4.12 ± 2.24). The SUVmax and size factors (maximum diameter and areas) of affected lymph nodes were significantly different (P = 0.033 and P = 0.012, respectively). (18)F-FDG PET/CT showed the general distribution of (18)F-FDG accumulation. This factor helped to exclude malignant disease and aided the diagnosis of AOSD (42.3 %) in 11 cases when combined with clinical features and aided decisions regarding appropriate biopsy sites, such as the lymph nodes (n = 9) and bone marrow (n = 13). (18)F-FDG PET/CT is a unique imaging method for the assessment of metabolic activity throughout the body in subjects with AOSD. Characteristics or patterns of AOSD observed on (18)F-FDG PET/CT can be used for the indication and diagnosis or to guide the clinical management of ASOD.

Clinical value of ¹8F-fluoro-dexoxyglucose positron emission tomography/computed tomography in patients with adult-onset Still's disease: a seven-case series and review of the literature.

OBJECTIVES: While there are a few reports describing 18F-fluoro-dexoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) findings in patients with adult-onset Still's disease (AOSD), no summary report has yet been published. In this study, we evaluated the usefulness of FDG-PET/CT for diagnosis and activity evaluation in patients with AOSD by summarizing the findings of our patients and those reported in the literature. METHODS: Seven consecutive AOSD patients who had undergone PET/CT at our department between 2007 and 2012 were included. We evaluated FDG uptake for characteristic findings in patients with AOSD. In addition, we reviewed the literature on seven previously reported AOSD patients who had undergone PET/CT. RESULTS: FDG accumulation was positive mainly in the bone marrow (100%), spleen (90.9%), lymph nodes (80.0%) and joints (75.0%). In addition, FDG uptake was positive in the pericardium, pleura, salivary glands, eyelids, muscle and major blood vessels. Six patients underwent follow-up FDG PET/CT for evaluation of treatment efficacy. Follow-up PET/CT showed diminished FDG accumulation in the bone marrow, spleen and lymph nodes, with maximum standardized uptake value (SUVmax) being substantially reduced from 4.03 ± 0.95 to 2.20 ± 0.75 (p = 0.04), 4.04 ± 1.10 to 2.55 ± 1.13 (p = 0.04) and 5.63 ± 4.99 to 2.10 ± 1.91 (p = 0.11), respectively. No significant correlation was found between SUVmax in each lesion and the laboratory data, except for a significant correlation between lactate dehydrogenase (LDH) and spleen SUV. CONCLUSIONS: FDG-PET/CT is useful for long-term assessments of AOSD activity in individual patients. However, PET/CT findings alone are not sufficient to make a differential diagnosis of AOSD versus malignant lymphoma.

Elevated FDG activity in lymph nodes as well as the spleen and liver in a patient with adult-onset still disease.

A 66-year-old woman had fever, rash, and joint pain. Physical examination revealed multiple enlarged lymph nodes. A possibility of lymphoma was considered and FDG PET/CT was performed, which demonstrated elevated FDG activity not only in many lymph nodes but also in the spleen and liver. However, adult-onset Still disease was diagnosed, and the patient responded well to therapy.

[Adult refractory Still disease with atypical articular manifestations: efficacity of interleukin-6 antagonists (tocilizumab)]. / Atteintes articulaires atypiques au cours d'une maladie de Still de l'adulte réfractaire : efficacité des antagonistes de l'interleukine-6 (tocilizumab).

INTRODUCTION: Tocilizumab is a humanized monoclonal antibody directed against interleukin-6 receptor and is beginning to be reported as effective in some cases of Still's disease refractory in adults (ASD). ASD is rare, heterogeneous, with unpredictable evolution. The distal destructive arthritis represents a possible complication. PATIENT: We report an unusual case of adult-onset Still's disease with severe distal interphalangeal destructive arthritis with refractory early and prolonged remission after the first tocilizumab infusion. CONCLUSION: Tocilizumab can be used in patients with refractory ASD after failure or intolerance of conventional treatments.

Is Still's disease still one disease? A case of Adult-onset Still's disease showing accumulation in the carotids and the large vessels of the legs on positron emission tomography: CT images.

Adult-onset Still's disease (AOSD) is known as a systemic inflammatory disease of unknown etiology and pathogenesis, characterized by fever, skin eruptions, systemic organ involvement, and arthralgias. AOSD is difficult to diagnose because of its heterogeneous clinical manifestations and prevalence (although more prevalent in the young, onset of AOSD after the age of 60 has also been described), and absence of pathognomonic clinical features. The disease also lacks a specific diagnostic test. To date, association studies between AOSD and HLA loci have failed to indentify a genetic predisposition. The recent publication of entirely different PET-CT manifestations found in three patients who were supposed to have the same disease (AOSD), as well as the surprisingly different PET-CT images of our AOSD patient (accumulation in the carotids and large vessels of the legs), raises our suspicion that AOSD is actually not one entity but a constellation of disorders whose varying underlying pathologies are now being revealed by new imaging techniques.

Delayed ascending aortic pseudoaneurysm in an adult onset Still's disease patient with previous mitral valve and aortic root replacement.

A 59-year-old female patient with adult onset Still's disease (AOSD) presented with hemoptysis and pseudoaneurysm from an aortic root vent cannulation site that was created 4 years earlier for combined mitral and aortic valve surgery. The pseudoaneurysm was successfully repaired and the patient remained well during a follow-up period of 20 months.

Adult-Onset Still Disease (AOSD).

Adult-onset Still disease (AOSD) is an uncommon clinical entity that predominantly affects young adults. One of the most common presentations of the disease is fever of unknown origin. Early diagnosis can be difficult because fever of unknown origin is more commonly seen with other conditions such as malignancy or infection. Ambiguity in presentation and lack of serologic markers make diagnosis difficult. We describe here an 18-year-old African-American man who presented with fever, sore throat, and arthritis at initial admission, with serology positive for both Mycoplasma pneumonia and Epstein-Barr infection. The patient was discharged without improvement. He was then readmitted with persistence of initial symptoms and, at that stage, he fulfilled the proposed diagnostic criteria of AOSD. The purpose of this case report is to describe the triggering infections that can initially mislead diagnosis and to review the literature about AOSD from a primary care perspective.

A case of adult onset Still's disease showing marked accumulation in the liver and spleen, on positron emission tomography-CT images.

A 35-year-old woman was admitted to our hospital because of high fever and skin rash, and subsequently diagnosed as having adult onset Still's disease (AOSD). Because of resistance to the steroid hormones, high levels of the serum-soluble form of the interleukin-2 receptor and splenomegaly, we suspected a possible diagnosis of malignant lymphoma and performed positron emission tomography (PET), which disclosed an intense accumulation of 2-deoxy-2 [F18] fluoro-D-glucose (FDG) in the liver and spleen. However, bone marrow aspiration and liver biopsy did not reveal any malignant cells. After the treatment of high-dose adrenocorticosteroids and plasma exchange, her symptoms and laboratory data, including PET findings, gradually improved. This is a rare case of severe AOSD in which an intense accumulation of FDG was detected by PET, and a differential diagnosis from malignant lymphoma may be difficult by FDG-PET alone, so that careful evaluation by techniques including histopathological examination may be necessary.

Inhibition of the TNF-pathway: use of infliximab and etanercept as remission-inducing agents in cases of therapy-resistant chronic inflammatory disorders.

OBJECTIVE: To examine the potential of the two tumour necrosis factor (TNF) inhibitors infliximab and etanercept as remission-inducing agents in chronic therapy-resistant inflammatory disorders of immune or non-immune pathogenesis. METHODS: 14 patients with adult Still's disease/macrophage activation syndrome (4), Wegener's disease (3), Behçet's disease (3), keratoscleritis (1), lymphomatous tracheo-bronchitis (1) Cogan's syndrome (1), and rapidly destructive crystal arthropathy (1) were treated with infliximab (n = 10) and etanercept (n = 4). All patients showed organ-threatening progression of their diseases with resistance to conventional immunosuppressive medication. Therapeutic benefit was assessed clinically and by documenting organ-specific functional and morphological alterations. Side effects were compared with the data of our clinic's rheumatoid arthritis (RA) patients treated by TNF inhibitors. RESULTS: A rapid and dramatic beneficial effect was documented in 9 patients and a moderate one in 5. Best responses (clinical and laboratory parameters) were seen in patients with macrophage activation syndrome/adult Still's disease and Behçet's disease, while the results were less impressive in those with Wegener's disease, Cogan's syndrome, idiopathic cerato-scleritis and lymphomatous tracheobronchitis. In all cases immunosuppressive agents and systemic glucocorticoids could be reduced or discontinued. CONCLUSIONS: TNF inhibition may be highly effective in patients with severe, therapy-resistant chronic inflammatory disorders.

[Arthrodesis of the wrist in inflammatory arthropathy. Effects of fusion of intracarpal joint spaces on functional results]. / Arthrodèses du poignet pour arthropathie inflammatoire. Influence de la fusion des interlignes intra-carpiens sur le résultat fonctionnel.

We retrospectively analysed 25 wrist arthrodeses performed in 23 patients because of inflammatory joint disease (21 rheumatoid arthritis, 1 case of Still's disease, 1 case of psoriatic arthritis) to assess: 1) the functional result, the position and the fusion rate; 2) the correlation between the radiographic features and the results on pain. The results were evaluated after an average of 56 months (12-121) by an observer not involved in surgery. 8 wrists were pain-free, 12 caused occasional pain, 4 caused frequent pain and 1 wrist was responsible for continuous pain at follow-up. The position of the arthrodesis was acceptable in the sagittal plane (mean extension 4.3 degrees), but with a slight ulnar tilt (mean ulnar tilt 12.8 degrees). Fusion was achieved in all cases after a mean of 8.2 weeks (5-16). All the intracarpal joints had united in only 8 cases, while the scaphotrapezo-trapezoid joint had not united in 17 cases, but fusion was spontaneously obtained in 8 cases. We identified 5 non-unions between lunatum and triquetrum, 5 non-unions between hamatum and capitatum and 3 non-unions between triquetrum and hamatum. Pain at follow-up was related to non-union of triquetro-lunate joints (p = 0.035). Wrist arthrodesis remains appropriate for severe lesions of the rheumatoid wrist in order to restore function and relieve pain.