Adult Still’s disease and respiratory failure in a 74 year old woman

Stoica, George S.; Cohen, Rubin I.; Rossoff, Leonard J.

doi:10.1136/pmj.78.916.97

Cited by 26 publications

(23 citation statements)

References 3 publications

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“…1,2,7 Pleural effusions and respiratory failure have been observed in patients with AOSD. 1,8 In the case that we describe, CT of the thorax revealed bilateral small pleural effusions. AOSD is a clinical diagnosis.…”

Section: Discussionmentioning

confidence: 73%

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A rare presentation of adult onset Still’s disease in an elderly patient

2011

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Adult onset Still's disease (AOSD) is a rare inflammatory disorder of unknown etiology that usually affects young adults. Very few patients older than 70-year-old have been reported. Clinical features include quotidian fevers, arthralgias, arthritis, pharyngitis, lymphadenopathy and an evanescent rash. AOSD should be considered in the differential diagnosis of fever of unknown origin. Early diagnosis is often difficult since it is a diagnosis of exclusion and the presence of infectious, neoplastic and autoimmune conditions needs to be ruled out before the diagnosis is made. No specific laboratory tests are available to aid in the diagnosis of AOSD. As a result, a set of diagnostic criteria that define the clinical features of this condition, termed the Yamaguchi criteria, have been most commonly used to establish the diagnosis. We describe the case of a 72-year-old Caucasian male with past medical history significant for generalized anxiety disorder, depression, BPH, and hypertriglyceridemia, ho was evaluated at a tertiary institution for profound generalized weakness and weight loss that started three weeks prior to his initial presentation. Initial laboratory studies showed leukocytosis, elevated ESR, CRP, ferritin and liver dysfunction. Cultures, ANA and rheumatoid factor studies were negative. The patient underwent further extensive workup that excluded the presence of infectious, neoplastic and autoimmune disorders and was subsequently diagnosed with AOSD and new onset diabetes mellitus. For the management of AOSD he was started on prednisone with significant improvement in markers of inflammation, symptoms and level of function. Case ReportA 72-year-old Caucasian male with past medical history significant for generalized anxiety disorder, depression, BPH, and hypertriglyceridemia presented with profound generalized weakness, significant weight loss of 30 lbs, fever and arthralgia of three weeks duration. Physical exam was significant for apparent muscle weakness, lethargy, 2 + edema of the hands and 1+ edema of the lower extremities. Initial vital signs included temperature 38.2 C, pulse 95 beats/min, respiratory rate 16 breaths/min, blood pressure 103/67, oxygen saturation 97% on room air. Blood cultures obtained upon admission showed no growth. Chest X-ray revealed no acute cardiopulmonary process. Urinalysis revealed trace bacteria, WBC3-4 HPF, glucose >1000 mg/dL, protein 30 mg/dL, rare epithelial cells. Laboratory studies included sodium 132 mEq/L, potassium 3.8 mEq/L, chloride 95 mEq/L, carbon dioxide 26 mEq/L, glucose 384 mg/dL, BUN 20 mg/dL, creatinine 0.9 mg/dL, calcium 8.6 mg/dL, GFR 83 mL/min, albumin 2.4 gm/dL, lipase 125 IU/L, amylase 162 U/L, WBC 23.2 thou/cumm, 91% polys, RBC 4.32 mill/cumm, Hgb 13 gm/dL, Hct 39%, MCV 89 FL, platelet count 365 thou/cumm. EBV IgG level was 4 and EBV IgM level was 0.1, suggestive of latent infection. CMV IgG results were also elevated, again suggesting latent exposure. Liver function tests on admission were within normal limits with the exception of el...

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Section: Discussionmentioning

confidence: 73%

“…1,5 A limited number of cases in the elderly have been reported. [6][7][8] Patients often present with fever (usually higher than 39C), evanescent rash, lymphadenopathy, sore throat, arthralgia, myalgia, arthritis and serositis. [1][2][3][4]9 In contrast, our patient presented initially with profound fatigue and weight loss.…”

Section: Discussionmentioning

confidence: 99%

A rare presentation of adult onset Still’s disease in an elderly patient

2011

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“…Tamponade, myocarditis [39,40], pulmonary fibrosis, pleural effusions and adult respiratory distress syndrome have also been described [41].…”

Section: Clinical Featuresmentioning

confidence: 99%

Adult-onset Still’s disease

et al. 2009

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“…The patient was 57 Autoimmun Highlights (2010) 1:53-59 dyspnoeic and hypoxic and showed multiple pulmonary infiltrates as is seen in ARDS. Three other cases of fatal AOSD and ARDS have been described in one case associated with RHS [22,25,26]. Myocarditis [14,16,20,35,46,[53][54][55], and in particular cardiac tamponade [45], can also very rarely complicate the course of AOSD, more frequently appearing in the paediatric form.…”

Section: Casementioning

confidence: 99%

“…Moreover, hepatomegaly, splenomegaly and lymphoadenopathy can be seen [12,13]. Although mortality in AOSD is quite low, a fatal outcome has been reported in some patients [2,[14][15][16][17][18][19][20][21][22][23][24][25][26][27][28].…”

Section: Introductionmentioning

confidence: 99%

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

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Colafrancesco

Gattamelata

et al. 2010

Autoimmun Highlights

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Purpose The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. Methods The clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature. Results The most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm 3 (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease). Conclusion The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.

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Adult Still’s disease and respiratory failure in a 74 year old woman

Abstract: A case of adult onset Still’s disease in an elderly woman, that was associated with severe respiratory failure and multiorgan dysfunction, is reported. Histopathology was confirmed on open lung biopsy.

Cited by 26 publications

References 3 publications

A rare presentation of adult onset Still’s disease in an elderly patient

A rare presentation of adult onset Still’s disease in an elderly patient

Adult-onset Still’s disease

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

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