Still’s disease and anaesthetic concerns: A case report

The Open Rheumatology Journal, 2013

Introduction: Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder characterized by rash, leukocytosis, fevers, and arthralgias. Pulmonary involvement has been reported rarely in AOSD, but acute respiratory distress syndrome (ARDS) is extremely rare and potentially fatal and must be recognized as potential manifestation of underlying AOSD. Methods: We present a case of AOSD manifested by ARDS and review the previously reported cases in Medline/Pub med. Results: Including this case, 19 cases of AOSD complicated with ARDS have been reported in the literature. Conclusions: It is important to recognize ARDS as a manifestation of AOSD so that proper diagnostic and therapeutic management can be initiated promptly.

Journal of the Postgraduate Institute of Medicine, 2021

Clinical Rheumatology, 2014

Adult-onset Still's Disease (AOSD) since its description in 1971 has proven to be a very complex and challenging disease entity. This rare auto-inflammatory disease is classically described by the "Still's triad" of fever, rash, and arthritis, although the atypical cases frequently outnumber the typical ones. The exact pathogenesis and etiologic factors responsible for the clinical features remain largely obscure, despite recent suggestive cytokine biology findings. Diagnosis is made on clinical grounds, following the exclusion of mimickers of infectious, autoimmune or neoplastic etiology, with the additional consideration of non-specific laboratory abnormalities such as peripheral leukocytosis and elevation of serum ferritin and other acute phase reactants. The disease manifestations are protean and can include diverse complications, affecting multiple organ systems. Moreover, the severity of the organ involvement can vary considerably, representing a wide spectrum from the self-limited to severe. The mainstay of therapy has evolved from the traditional use of corticosteroids and oral immunosupressants to the newer targeted treatments with biologic agents. The scope of this review is to alert the clinician to the existence of life-threatening AOSD complications, namely the macrophage activation syndrome, disseminated intravascular coagulopathy, thrombotic thrombocytopenic purpura, diffuse alveolar hemorrhage, and pulmonary arterial hypertension. Such knowledge may lead in earlier recognition, prompt treatment, and, ideally, improved patient outcomes.

2009

Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition characterised by high fever, leukocytosis with neutrophilia, arthralgia and skin rash. Diagnosis is often clinical, and it is always necessary to exclude common disease mimics such as infections, malignancies and other rheumatic diseases. Ferritin and glycosylated ferritin are useful adjuncts to aid in the diagnosis of AOSD. Important complications to consider include fulminant hepatitis, disseminated intravascular coagulopathy and hemophagocytic syndrome. Mainstay treatment of AOSD is often with glucocorticoids. Other agents that can also be used include methotrexate, and biologic therapy, such as tumor necrosis factor-α (TNF-α) blockers and interleukin-1 (IL-1) inhibitors. A retrospective review of patients diagnosed with AOSD admitted to Singapore General Hospital (SGH) was performed, and their disease characteristics and laboratory findings were compared with that of previous published cohorts.

Clinical Rheumatology, 2007

We studied the clinical profile, laboratory parameters, disease course, and outcomes of patients with adult onset Still's disease (AOSD). A retrospective analysis of adult patients with Still's disease diagnosed from 2000 to 2004 was carried out. Their clinical features and laboratory findings at presentation, disease course, and outcomes were analyzed. Data of 14 patients with Still's disease were analyzed. The age at disease onset ranged from 16 to 59 years with a mean of 29.85, the male to female ratio being 9:5. The mean duration of illness from onset of symptoms to presentation was 14.5 months (range). The most common clinical manifestations were fever (n=14), articular symptoms (n=14), rash (n=8), weight loss (n=12), and sore throat (n=5). Elevated ESR was present in all patients with a mean of 98.3 mm at 1 h. Hepatic enzymes were elevated in seven patients at disease onset. The mean duration of follow up was 19.14 months (range). Three patients progressed to chronic arthropathy. Cyclosporine led to dramatic recovery in five patients. Macrophage activation syndrome (MAS) was present in two patients, one after sulfasalazine therapy. One patient with MAS died. Still's disease, although uncommon, has characteristic constellation of clinical and laboratory features and should be considered in the differential diagnosis of fever of unknown origin. Nonsteroidal anti-inflammatory drugs, steroids, and methotrexate may not be always effective, and cyclosporine is an effective drug in resistant cases. Sulfasalazine should be avoided in cases of AOSD.

Cureus, 2020

Background Adult-onset Still's disease (AOSD) is characterized by a classical triad of spiking fever, arthritis, and evanescent rash. It is one of the main causes of hospital admissions for fever of unknown origin and has an extended mean time to diagnosis. Therefore, it remains underdiagnosed relative to its aforementioned time frame. In this study, we attempt to highlight clinical and lab findings associated with AOSD. We then compare our diagnostic results with similar previous studies. Our results should help physicians not to miss this rare entity and make the diagnosis in a reduced time frame. Materials and methods This a retrospective, single-center study conducted at Dow University Hospital in Karachi, Pakistan. Thirty patients were enrolled in this study for six months (July 2019-December 2019). All patients were identified and recruited in the medical outpatient department using Yamaguchi's criteria. Written consent was obtained from all patients to access all their clinical charts. Demographics and prior results of laboratory investigations were retrieved from the chart files. Results In our study, the mean age of patients was 26.6 years. More specifically, males accounted for 53.3% (n=16) of cases, and females accounted for 46.6% (n=14) of total cases. The most common presenting clinical features included arthralgia (100%, n=30), fever (100%, n=30), myalgia (96.6%, n=29), large joint arthritis (66.6%, n=20), sore throat (50%, n=15), hepatomegaly (40%, n=12), splenomegaly (23.3%, n=7), skin rash (36.6%, n=11) and pericarditis (20%, n=6). Furthermore, none of our patients had cervical lymphadenopathy. The most commonly reported laboratory findings include leukocytosis (100%, n=30), hyperferritinemia (90%, n=27), elevated erythrocyte sedimentation rate (100%, n=30) and abnormal liver function tests (76.6%, n=23). Chest x-rays performed in all patients revealed no abnormalities in 83% of patients (n=25). All patients in our study sample (n=30) tested negative for antinuclear antibodies and rheumatoid factor. It was also noted that the mean duration of diagnosis in our patients was 5.98 weeks. The mean hospitalization period in our patients was 12.5 days. Furthermore, 16.7% of patients (n=5) suffered from disease relapse. Conclusion Our study ascertains that the presence of certain clinical and laboratory findings strongly indicate a diagnosis of adult-onset Still's disease.

Arch Gen Intern Med, 2019

Adult-onset Still’s Disease (AOSD) is a rare systemic auto inflammatory disorder characterized by a triad of spiking fever, rash, and arthralgia that may be associated with multiple systemic manifestations.Diagnosis is clinical, which can be challenging. While definitive diagnostic test is lacking, ferritin and glycosylated ferritin are useful adjuncts to aid in the diagnosis of AOSD. It is important to include AOSD in the differential diagnosis of prolonged fever and sore throat especially if accompanied by other systemic manifestations.Mainstay treatment of AOSD is often with glucocorticoids. Other agents that can also be used include methotrexate, and biologic therapy, such as tumor necrosis factor-α (TNF-α) blockers and interleukin-1 (IL-1) inhibitors. We hereby report a case of a thirty-six years old man who presented with a prolonged course of fever, and recurrent pharyngitis diagnosed as Adult-onset Still’s Disease (AOSD).

Case Reports, 2020

Introduction: Adult Onset Still´s Disease (AOSD) is a rare systemic inflammatory disease of unclear etiology, with low incidence and prevalence among the general population. AOSD is a common cause of fever of unknown origin (FUO) in up to 20% of cases. Due to the scarce knowledge about this disease and its diagnosis, it is usually unrecognized in the differential diagnoses, worsening the prognosis and increasing complications in some patients. Case presentation: This is the case of a 32-year-old female patient with prolonged febrile illness, who did not respond to the antimicrobial treatments previously established. She was diagnosed with AOSD according to the Yamaguchi criteria after an extensive exclusion process. She was treated with first-line treatment with corticosteroids, achieving satisfactory results. Conclusions: The diagnosis of AOSD is an exhaustive process. Regardless of the availability of cutting-edge diagnostic tools, the medical history of the patient and an adequat...

Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition characterised by high fever, leukocytosis with neutrophilia, arthralgia and skin rash. Diagnosis is often clinical, and it is always necessary to exclude common disease mimics such as infections, malignancies and other rheumatic diseases. Ferritin and glycosylated ferritin are useful adjuncts to aid in the diagnosis of AOSD. Important complications to consider include fulminant hepatitis, disseminated intravascular coagulopathy and hemophagocytic syndrome. Mainstay treatment of AOSD is often with glucocorticoids. Other agents that can also be used include methotrexate, and biologic therapy, such as tumor necrosis factor-α (TNF-α) blockers and interleukin-1 (IL-1) inhibitors. A retrospective review of patients diagnosed with AOSD admitted to Singapore General Hospital (SGH) was performed, and their disease characteristics and laboratory findings were compared with that of previous published cohorts.

American Journal of Case Reports, 2017

Rare disease Background: Adult-onset Still's Disease (AOSD) is a rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema, and arthralgia. To date, there is no definite laboratory or imaging test available for diagnosing AOSD, and the diagnosis is one of exclusion, which can be very challenging. Case Report: We report on the case of a 53-year-old female who presented with fever, arthralgia, and abdominal pain. Her initial laboratory tests showed elevated AST and ALT, and normal leukocytes with bandemia. During her hospitalization, we evaluated the patient for other potential differential diagnoses. After an extensive workup, the patient was diagnosed with AOSD based on Yamaguchi criteria. Her serum ferritin levels were measured and found to be markedly elevated, which is a non-specific finding in AOSD patients. Conclusions: This case highlights the important role of a detailed history and physical examination for timely diagnosis of AOSD to prevent complications and improve patient's prognosis.