A rare clinical manifestation of adult-onset Stills disease:polyserositis and effusions
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《中华医药杂志》英文版
Department of Infection and Liver Disease, The First Affiliated Hospital of Wenzhou Medical College,Wenzhou,Zhejiang Province 325000,China
Correspondence to Professor CHEN Yong-ping, Department of Infection and Liver Disease, The First Affiliated Hospital of Wenzhou Medical College, Wenzhou, Zhejiang Province 325000,China
Tel: +86-577-88078232, Fax:+86-577-88203006,E-mail: ypchen106@yahoo.com.cn
INTRODUCTION
Adult-onset Stills disease (AOSD) is a systemic inflammatory disorder of unknown cause. There is no diagnostic test or pathognomonic histopathology, so the diagnosis of AOSD is often a diagnosis of exclusion. Polyserositis or effusions are seldom reported and exudative effusions are found in thoracentesis or pericardiocentesis. Herein we report a case of atypical AOSD which had been misdiagnosed for a long time since it attacked.
CASE REPORT
A 29-year-old male was admitted to hospital because of a seven-day history of severe febrile illness, accompanying with chest distress, chest pain and sore throat. There was no other significant past medical history and he didnt take any drugs. Examination revealed a fever of 40 ℃ and the mild swelling knee joints. Other abnormal findings included slightly quiet heart sounds and the breath sound was low without obvious rhonchi and moist rales in the lung. In percussion, the lower chest was dull. Initial investigations showed that the patients white blood cell count was 24.3×109/L, neutrophil accounted for 92.6%, erythrocyte sedimentation rate (ESR) 98 mm/h, C-reactive protein (CRP) 168 mg/L and albumin 30 g/L. His liver function tests, antistreptolysin-O test and complement levels were within normal range. He had a normal electrocardiogram and chest computed tomography (CT) revealed pneumonia in the left lower lobe accompanying with bilateral moderate pleural effusions and a little pericardial effusion. Slight splenomegaly and a small quantity of ascites were found with abdominal ultrasound and pericardial effusion ranging from small to moderate was confirmed with echocardiogram. So the preliminary diagnosis was pneumonia and polyseromembrane fluid except for sepsis. He was administered with sulperazone plus azithromycin.
During the first 4 days of his hospitalization, he had a daily spiking fever up to 41 ℃, usually in the evening, then returned to normal or below normal gradually. Arthralgia became worsening, involving the wrist joints, ankle joints and so on. Dyspnea appeared little by little, especially in exertion. Further investigation of pleural fluid showed white blood cell count was 14 240/μl, neutrophil and lymphocyte accounted for 97% and 3% respectively, protein level 20.0 g/L, lactic dehydrogenase 1 036 U/L, adenosine deaminase 13 U/L and carcino-embryonic antigen 0.9 μg/L. Rivalta test was positive and other tests revealed that rheumatoid factor, antinuclear antibody, antineutrophil cytoplasmic antibody and homologous leucocytic antigen B27 were negative. He had a normal bone marrow examination and negative blood culture. Now, ESR had risen to 125 mm/h. The symptoms continued to exacerbate so we chose tienam instead of the former drugs which had been used for four days, but after 5 days the symptoms didnt improve. So adult-onset Stills disease (AOSD) was suspected. Then he was treated with prednisolone and all symptoms relieved after three days. Repeated echocardiogram did not show any reaccumulation of the pericardial fluid and chest computed tomography showed pericardial thickening, but pleural fluid disappeared. ESR dropped to 33 mm/h and CRP dropped to 16 mg/L. So AOSD was finally diagnosed. During one years following-up period, the patients condition maintains stable.
DISCUSSION
The diagnosis of AOSD rests on the combination of typical clinical and laboratory findings and the diagnostic criteria developed by Cush[1, 2].A triad of quotidian fever, evanescent rash and polyarthritis presents. Pharyngitis, splenomegaly, lymphadenopathy, cardiopulmonary involvement and hepatomegaly are sometimes seen. Not all the eventual symptoms and signs may be apparent at presentation and the condition can evolve over period of weeks or months. Polyserositis or effusions are seldom reported and exudative effusions are found in thoracentesis or pericardiocentesis[1~4 ].
In this case, effusions are found in bilateral pleural cavities and the pleural fluid is an exudates with increased numbers of inflammatory cells, particularly neutrophils. Although uncommon, a worrisome manifestation of AOSD is acute pericardial tamponade[5].Pneumonitis is found in more than 20% of cases and may present as bilateral alveolar and interstitial infiltrations on radiographs[1,2].Another character of this case is rapidly response to glucocorticosteroid. It may be a hint for the final diagnosis. Stills rash is seen in more than 92% of patients. It is evanescent, frequently appears during febrile attacks, lasts for hours, and tends to change from day to day. But in this case, rash is absence. According to the above characteristic, it can be easily misdiagnosed as sepsis, purulent serositis or tuberculosis in multiserous cavity. Awareness of the condition is important when investigating patients with polyserositis or effusions.
REFERENCES
1. Cush JJ, Medsger TA Jr, Christy WC, Herbert DC, Cooperstein LA. Adult-onset Stills disease. Clinical course and outcome. Arthritis Rheum, 1987,30:186-194.
2. Cush JJ. Adult-onset Stills disease. Bull Rheum Dis,2000,49:1-4.
3. Pasteur M, Laroche C, Keogan M. Pleuropericardial effusion in a 50 year old woman. Pleuropericardial effusion caused by adult inset Stills disease. Postgrad Med J, 2001,77:346, 355-357.
4. Pouchot J, Sampalis JS, Beaudet F, Carette S, Decary F, Salusinsky-Sternbach M, et al. Adult Stills disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore), 1991,70:118-136.
5. Moder KG, Miller TD, Allen GL. Cardiac tamponade: an unusual feature of adult onset Stills disease. J Rheumatol,1995,22:180-182.
(Editor Anne)(ZHENG Ming-hua, CHEN Yong)
Correspondence to Professor CHEN Yong-ping, Department of Infection and Liver Disease, The First Affiliated Hospital of Wenzhou Medical College, Wenzhou, Zhejiang Province 325000,China
Tel: +86-577-88078232, Fax:+86-577-88203006,E-mail: ypchen106@yahoo.com.cn
INTRODUCTION
Adult-onset Stills disease (AOSD) is a systemic inflammatory disorder of unknown cause. There is no diagnostic test or pathognomonic histopathology, so the diagnosis of AOSD is often a diagnosis of exclusion. Polyserositis or effusions are seldom reported and exudative effusions are found in thoracentesis or pericardiocentesis. Herein we report a case of atypical AOSD which had been misdiagnosed for a long time since it attacked.
CASE REPORT
A 29-year-old male was admitted to hospital because of a seven-day history of severe febrile illness, accompanying with chest distress, chest pain and sore throat. There was no other significant past medical history and he didnt take any drugs. Examination revealed a fever of 40 ℃ and the mild swelling knee joints. Other abnormal findings included slightly quiet heart sounds and the breath sound was low without obvious rhonchi and moist rales in the lung. In percussion, the lower chest was dull. Initial investigations showed that the patients white blood cell count was 24.3×109/L, neutrophil accounted for 92.6%, erythrocyte sedimentation rate (ESR) 98 mm/h, C-reactive protein (CRP) 168 mg/L and albumin 30 g/L. His liver function tests, antistreptolysin-O test and complement levels were within normal range. He had a normal electrocardiogram and chest computed tomography (CT) revealed pneumonia in the left lower lobe accompanying with bilateral moderate pleural effusions and a little pericardial effusion. Slight splenomegaly and a small quantity of ascites were found with abdominal ultrasound and pericardial effusion ranging from small to moderate was confirmed with echocardiogram. So the preliminary diagnosis was pneumonia and polyseromembrane fluid except for sepsis. He was administered with sulperazone plus azithromycin.
During the first 4 days of his hospitalization, he had a daily spiking fever up to 41 ℃, usually in the evening, then returned to normal or below normal gradually. Arthralgia became worsening, involving the wrist joints, ankle joints and so on. Dyspnea appeared little by little, especially in exertion. Further investigation of pleural fluid showed white blood cell count was 14 240/μl, neutrophil and lymphocyte accounted for 97% and 3% respectively, protein level 20.0 g/L, lactic dehydrogenase 1 036 U/L, adenosine deaminase 13 U/L and carcino-embryonic antigen 0.9 μg/L. Rivalta test was positive and other tests revealed that rheumatoid factor, antinuclear antibody, antineutrophil cytoplasmic antibody and homologous leucocytic antigen B27 were negative. He had a normal bone marrow examination and negative blood culture. Now, ESR had risen to 125 mm/h. The symptoms continued to exacerbate so we chose tienam instead of the former drugs which had been used for four days, but after 5 days the symptoms didnt improve. So adult-onset Stills disease (AOSD) was suspected. Then he was treated with prednisolone and all symptoms relieved after three days. Repeated echocardiogram did not show any reaccumulation of the pericardial fluid and chest computed tomography showed pericardial thickening, but pleural fluid disappeared. ESR dropped to 33 mm/h and CRP dropped to 16 mg/L. So AOSD was finally diagnosed. During one years following-up period, the patients condition maintains stable.
DISCUSSION
The diagnosis of AOSD rests on the combination of typical clinical and laboratory findings and the diagnostic criteria developed by Cush[1, 2].A triad of quotidian fever, evanescent rash and polyarthritis presents. Pharyngitis, splenomegaly, lymphadenopathy, cardiopulmonary involvement and hepatomegaly are sometimes seen. Not all the eventual symptoms and signs may be apparent at presentation and the condition can evolve over period of weeks or months. Polyserositis or effusions are seldom reported and exudative effusions are found in thoracentesis or pericardiocentesis[1~4 ].
In this case, effusions are found in bilateral pleural cavities and the pleural fluid is an exudates with increased numbers of inflammatory cells, particularly neutrophils. Although uncommon, a worrisome manifestation of AOSD is acute pericardial tamponade[5].Pneumonitis is found in more than 20% of cases and may present as bilateral alveolar and interstitial infiltrations on radiographs[1,2].Another character of this case is rapidly response to glucocorticosteroid. It may be a hint for the final diagnosis. Stills rash is seen in more than 92% of patients. It is evanescent, frequently appears during febrile attacks, lasts for hours, and tends to change from day to day. But in this case, rash is absence. According to the above characteristic, it can be easily misdiagnosed as sepsis, purulent serositis or tuberculosis in multiserous cavity. Awareness of the condition is important when investigating patients with polyserositis or effusions.
REFERENCES
1. Cush JJ, Medsger TA Jr, Christy WC, Herbert DC, Cooperstein LA. Adult-onset Stills disease. Clinical course and outcome. Arthritis Rheum, 1987,30:186-194.
2. Cush JJ. Adult-onset Stills disease. Bull Rheum Dis,2000,49:1-4.
3. Pasteur M, Laroche C, Keogan M. Pleuropericardial effusion in a 50 year old woman. Pleuropericardial effusion caused by adult inset Stills disease. Postgrad Med J, 2001,77:346, 355-357.
4. Pouchot J, Sampalis JS, Beaudet F, Carette S, Decary F, Salusinsky-Sternbach M, et al. Adult Stills disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore), 1991,70:118-136.
5. Moder KG, Miller TD, Allen GL. Cardiac tamponade: an unusual feature of adult onset Stills disease. J Rheumatol,1995,22:180-182.
(Editor Anne)(ZHENG Ming-hua, CHEN Yong)