|Year : 2019 | Volume
| Issue : 1 | Page : 35-38
Intermittent high-spiking fever, muscle and joint pain with sudden chest pain: Adult-onset still's disease?
Wei Li, Peng Dong, Fang Liu, Yang Jiang, Yuanzheng Li, Banghe Li
Department of Cardiology, Aviation General Hospital, China Medical University, Beijing, China
|Date of Web Publication||28-Mar-2019|
Department of Cardiology, Aviation General Hospital, China Medical University, No. 3, Beiyuan Road, Chaoyang, Beijing 100012
Source of Support: None, Conflict of Interest: None
Adult-onset Still's disease (AOSD) is a clinical syndrome characterized by long-term intermittent fever, transient polymorphic rash, arthritis or arthralgia, pharyngalgia, and accompanied by systemic involvements. The pathophysiology of AOSD has recently been classified as a complex autoinflammatory disease. Sometimes it is necessary to differentiate from coronary heart disease. In recent years, anakinra has been used to treat AOSD with promising results.
Keywords: Adult-onset Still's disease, intermittent high-spiking fever, systemic autoinflammatory disease
|How to cite this article:|
Li W, Dong P, Liu F, Jiang Y, Li Y, Li B. Intermittent high-spiking fever, muscle and joint pain with sudden chest pain: Adult-onset still's disease?. Cardiol Plus 2019;4:35-8
|How to cite this URL:|
Li W, Dong P, Liu F, Jiang Y, Li Y, Li B. Intermittent high-spiking fever, muscle and joint pain with sudden chest pain: Adult-onset still's disease?. Cardiol Plus [serial online] 2019 [cited 2019 Sep 22];4:35-8. Available from: http://www.cardiologyplus.org/text.asp?2019/4/1/35/255071
| Case Report|| |
A 51-year-old male who suffered from intermittent high-spiking fever with muscle and joint pain for the past 20 years was admitted to our hospital following recurrence of the symptoms for 2 weeks before the admission accompanied by sudden chest pain for 10 h. Initial attempts to diagnose the man carried out at his local hospital 20 years ago had uncovered no obvious cause of muscle and joint pain with high-spiking fever, but recorded his temperature as up to 40°C. After treatment with anti-inflammatory drugs, antipyretics, and hormones, etc., the patient's symptoms gradually subsided. Similar symptoms recurred repeatedly for 20 years. Two weeks before admission, the patient had a sore throat and muscle and joint pain accompanied by paroxysmal chills and a high fever (which mostly occurred at night and the highest temperature recorded was 40°C) without cough, sputum, abdominal pain, diarrhea, no frequent urination, urgency, and other discomforts. After treatment with antipyretic analgesics, his body temperature returned to normal. Ten hours before admission, the patient suddenly suffered from burning pain in the precordial area during sleep. The pain was persistent, without spreading to other areas of the body and not affects his breathing. The patient did not have a history of chronic diseases such as hypertension, heart disease, or diabetes. He also had no history of contact with infectious diseases, such as hepatitis or tuberculosis, and had no family history of inherited diseases. The patient smoked for 20 years, averaging 10 cigarettes a day, and occasionally imbibed a small amount of alcohol.
Physical examination at admission: patient was conscious, temp 37.2°C (with antipyretic drugs), pulse 102 beats/min, resp 22 beats/min, blood pressure 94/60 mmHg., and there were no pathological murmurs, pericardial frictional sounds, palms of the liver and spider nevus, rashes and bleeding spots, or varicose veins in the abdominal wall. The liver and spleen were not touched under the ribs, and there was no edema in either of the lower limbs.
A chest X-ray revealed no obvious abnormalities in the heart, lungs, or mediastinum [Figure 1]; electrocardiography (ECGs) showed extensive ST segment elevation [Figure 2] and [Figure 3]; Blood tests found total white blood cells 25 × 10^9/L [Figure 4], neutrophil ratio 93.2%, red blood cells 3.75 × 10^9/L, hemoglobin 105 g/L, and platelets 334 × 10^9/L; high-sensitivity C-reactive protein (hsCRP) >160 mg/L; plasma albumin 28.9 g/L, and erythrocyte sedimentation rate (ESR) 110 mm/H; D-dimer 1240 μg/L, troponin I, NT-ProBNP, and myocardial zymogram all in the normal range. The coronary angiography revealed the middle segment of the anterior descending artery myocardial bridge with systolic compression of 50%–70% (indicating a myocardial bridge), all the coronary artery walls were smooth with normal blood flow. Left ventricular angiography showed no significant abnormality and acute coronary syndrome was excluded. Echocardiography showed no obvious abnormality and acute myocarditis was excluded from the study [Figure 5].
|Figure 1: Chest X: No obvious abnormalities were found in the heart, lung, or diaphragm|
Click here to view
|Figure 2: Electrocardiography showed sinus rhythm, ST segment elevation 0.2–0.5mv in lead V2–V6, I, AVL, II, III, AVF, and V7–V9|
Click here to view
|Figure 4: Trend map of total leukocyte number: On admission day, the total number of white blood cells was 25.9 × 10^9/L, and then decreased to 10.3 × 10^9/L on the 4th day|
Click here to view
|Figure 5: Echocardiogram: Tricuspid regurgitation (mild) with the rest in normal range|
Click here to view
On the basis of the combination of clinical, laboratory, and imaging findings, our initial diagnosis was Adult-onset Still's disease (AOSD). After admission, the patient received nonsteroidal anti-inflammatory drugs (NSAIDs), broad-spectrum antibiotics and low-dose prednisone treatments, and the patient's body temperature gradually returned to normal [Figure 6]. His general situation improved, and he was voluntarily discharged and returned to the local hospital for further treatment. Follow-up at 10 months after discharge showed that the patient still had intermittent spiking fever, accompanied by muscle pain. A low dose of hormone could alleviate the condition.
|Figure 6: Temperature sheet: Temperature fluctuated from 36.3 o C to 39.6 o C one week after admission|
Click here to view
| Discussion|| |
Diagnosis and differential diagnosis
AOSD is a clinical syndrome characterized by long-term intermittent fever, transient polymorphic rash, arthritis or arthralgia, pharyngalgia, and accompanied by systemic involvement such as the increase of total number of peripheral leukocyte count and granulocytes and impaired liver function. Still's disease is named after the British physician Sir George Frederic Still. Adult Still's disease was first described and reported by Bywaters in 1971. Currently, our understanding of the pathological mechanism of AOSD is very limited., It has been estimated that about 1.6 per million new patients are newly diagnosed with AOSD each year.
During the past 20 years, this patient had recurrent intermittent spiking fever, up to 39.6°C, accompanied by increased leukocytes and neutrophils, and in muscles and joints pain. Laboratory tests revealed a significant increase in serum ferritin (6672 ng/ml), antinuclear antibody (IgG): 1:80, increased ESR (ESR 110 mm/H), and hsCRP increased (>160 mg/L). Chest computed tomography showed chronic inflammation of the lower left upper lobe and lower lungs and bilateral pleural effusion. In addition, the multiple enlarged mediastinal lymph nodes and bilateral cervical lymph nodes were found.
After combined treatment with antibiotics, NSAIDs and hormones, the patient's body temperature dropped, the pain of his joints was alleviated, his inflammation index decreased, and his general situation was improved. The combination of clinical, laboratory, and imaging findings all conform to the manifestations and characteristics of AOSD. The pathophysiology of AOSD has recently been classified as a complex autoinflammatory disease. Till date, there are no recognized specific diagnostic methods and standards for adult Still's disease. Many diagnostic or classification indicators have been developed. The recommended applications are the US Cush standard and the Japanese standard. AOSD is a clinical diagnosis, therefore, we must first exclude other diseases related to fever, rash, and arthritis, including various infections (viral infection, bacterial endocarditis, sepsis, tuberculosis, syphilis, Lyme disease, etc.), malignant tumors (leukemia, lymphoma, etc.) and immune diseases (systemic lupus erythematosus, mixed connective tissue disease, vasculitis, reactive arthritis, rheumatic fever, nodular erythema, etc.) and drug allergies.
Experience and lessons
Differentiation from acute coronary syndrome
This patient was 51-year-old male, and one of his main complaints was sudden chest pain for 10 h, which presented with ECG ST segments elevation. Therefore, there is no doubt that the possibility of acute coronary syndrome needed to be considered. Coronary angiography showed that the middle segment of the anterior descending artery had a significant systolic compression of 50%–70% (indicating a myocardial bridge), while the blood vessels wall were smooth with normal blood flow. Left ventricular angiography showed no obvious abnormalities. Multiple ECGs showed that ST segment elevation concave upward in most leads (I, II, aVL, aVF, and V2–V6). The myocardial enzymes and troponin were normal. Patient's chest pain was persistent continuous burning pain, without travelling into the shoulder, arm, back, neck, or jaw. After anti-inflammatory and hormone treatments, chest pain disappeared quickly and ST-ECG returned to normal. Combined with these data, acute coronary syndrome can be excluded. Presumably, it may be an immune system disease with a transient acute infection involving pericardial disease.
Previous literature contains case reports of Still's disease with pericarditis
Regarding the acute treatment of AOSD glucocorticoids should be considered in the following situations: (1) patients with NSAIDs have poor efficacy or toxic side effects such as liver damage or symptom relapse after reduction; (2) patients with serious illness such as pericardial tamponade, myocarditis, severe pneumonia, intravascular coagulation, and other organ damage., In recent years, it has been reported that Anakinra has been used to treat AOSD with promising results, and represents a promising subject of future studies.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We would like to thank Prof. Junbo Ge (Zhongshan Hospital) and Prof. Bingyin Wang (Kowloon Hospital) for their excellent support.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Bywaters EG. Still's disease in the adult. Ann Rheum Dis 1971;30:121-33.
Kadavath S, Efthimiou P. Adult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med 2015;47:6-14.
Sfriso P, Bindoli S, Galozzi P. Adult-onset Still's disease: Molecular pathophysiology and therapeutic advances. Drugs 2018;78:1187-95.
Magadur-Joly G, Billaud E, Barrier JH, Pennec YL, Masson C, Renou P, et al.
Epidemiology of adult Still's disease: Estimate of the incidence by a retrospective study in West France. Ann Rheum Dis 1995;54:587-90.
Akkara Veetil BM, Yee AH, Warrington KJ, Aksamit AJ Jr., Mason TG. Aseptic meningitis in adult onset Still's disease. Rheumatol Int 2012;32:4031-4.
Søgaard KK, Farkas DK, Ehrenstein V, Bhaskaran K, Bøtker HE, Sørensen HT, et al.
Pericarditis as a marker of occult cancer and a prognostic factor for cancer mortality. Circulation 2017;136:996-1006.
Prasad M, Hermann J, Gabriel SE, Weyand CM, Mulvagh S, Mankad R, et al.
Cardiorheumatology: Cardiac involvement in systemic rheumatic disease. Nat Rev Cardiol 2015;12:168-76.
Ocon AJ, Kwiatkowski AV, Peredo-Wende R, Blinkhorn R. Adult-onset Still's disease with haemorrhagic pericarditis and tamponade preceded by acute Lyme disease. BMJ Case Rep 2018;2018. pii: bcr-2018-225517.
Vandergheynst F, Gosset J, van de Borne P, Decaux G. Myopericarditis revealing adult-onset Still's disease. Acta Clin Belg 2005;60:205-8.
Yoo DH. Treatment of adult-onset Still's disease: Up to date. Expert Rev Clin Immunol 2017;13:849-66.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]