Extremely High Serum Ferritin: An Instrumental Marker of Masquerading Adult-Onset Still’s Disease with Hemophagocytic Syndrome

Abstract

Patient: Female, 88Final Diagnosis: Adult onset still’s diseaseSymptoms: Fever • rashMedication: —Clinical Procedure: —Specialty: RhaumatologyObjective:Unusual clinical courseBackground:Adult-onset Still’s disease (AOSD) is a rare multi-systemic inflammatory disorder of unknown etiology characterized by spiking fever, characteristic rash, and arthritis. It often associates with high serum ferritin levels.Case Report:An 88-year-old woman had fever of over 39°C without response to extended-spectrum antibiotics for 6 days. She had non-specific erythema with infiltration on her trunk. She had leukocytosis with neutrophilia of 80%, mild hepatic dysfunction, normal level of rheumatoid factor and antinuclear antibody, thrombocytopenia, elevated d-dimer and soluble interleukin2 receptor, extremely high serum ferritin (78 662 ng/mL), and splenomegaly. Although she had no arthritis or specific erythema, we made the diagnosis of AOSD according to Yamaguchi’s criteria with disseminated intravascular coagulation (DIC) and hemophagocytic syndrome (HPS) after ruling out infections, malignancies, or other connective tissue diseases. Twelve percent of AOSD patients have HPS. The mean serum ferritin of AOSD with HPS was reported at 18 179 ng/mL, which supported the diagnosis of AOSD because only a few other diseases could show such extremely high serum ferritin. Although she was treated with prednisolone (30 mg/day), her condition deteriorated and her left pleural effusion increased. Therefore, methylprednisolone 500 mg/day for 3 days was started followed by prednisolone 30 mg/day and immunosuppressive agent (Cyclosporine 50 mg/day), which improved her general condition, elevated C-reactive protein levels, and extremely high serum ferritin levels.Conclusions:We report the case of an elderly patient with severe AOSD, who developed HPS and DIC, whose extremely high serum ferritin level was useful in diagnosis.

Elderly-onset Still's Disease Complicated by Macrophage Activation Syndrome: A Case Report and Review of the Literature

“…Regarding the clinical outcome, the rates of remission, relapse and death were similar, but the observation period was longer in the overall AOSD group. Next, we identified 5 EOSD cases complicated by MAS, including the present case (8,15,28,29), and compared the findings with AOSD cases complicated by MAS from the literature review (46) ( Table 4). The mean onset age of MAS was 76.8±7.3 and 40.2±16.0 years old in the EOSD and AOSD groups, respectively.…”

“…However, some cases of elderlyonset Still's disease (EOSD) have also been reported . The oldest such case was 88 years old at the disease onset (8). The frequency of EOSD is so low that the literature is limited to only a few case reports, with no reviews available, so the overall clinical features of EOSD remain unclear.…”

<p>Status Quo of Diagnostic Procedures and Treatment of Inpatients with Infective Endocarditis at the Department of General Medicine at a University Hospital in a Suburban City in Japan: A Single-Hospital-Based Retrospective Study</p>

“…The general medicine department of this hospital manages the first-touch practice of outpatients without a reference from their primary doctors, in addition to treating both in-and outpatients with common diseases, fevers of unknown origin, diseases that are difficult to correctly diagnose, and complicated pathological situations requiring intensive care. [4][5][6][7] In this study, patients suspected of having, or diagnosed with IE before admission would, in principle, be seen by the endocarditis team of the department of cardiology and cardiovascular surgery. In comparison, patients with non-specific symptoms and signs such as fever of unknown origin, consciousness disorder, paralysis, or rash, who were not suspected of having, or were not diagnosed with IE prior to referral, would initially be seen by the department of general medicine, emergency medicine, neurology, or dermatology or whatever department seemed appropriate.…”

“…3 Among these departments, the general medicine departments, especially at university hospitals in Japan, which are often in charge of diagnosing diagnostically challenging diseases, including fevers of unknown origin, are likely to see patients with IE. [4][5][6][7] Our previous research showed that the general medicine department of Saga University Hospital in Japan diagnosed and treated the third-highest number of inpatients with IE after the departments of cardiology and cardiovascular surgery. 4 The department of cardiovascular surgery plays a clearly delineated role in treating IE in terms of when and how to operate and how to manage patients after surgery.…”

Adult-Onset Still’s Disease

“…The first disorder, disseminated intravascular coagulation (DIC), is not rare and may occur at a frequency of 1-5% [8,53,[59][60][61][62][63][64][65][66][67]. This diagnosis should be suspected with the combination of thrombotic events and cutaneous or mucosal bleeding and sometimes specific organ involvements, such as fulminant hepatitis, cardiac or respiratory failure, or stroke.…”