Still Disease is Still Hard to Diagnosis: A Case Report

Adult-onset Still Disease (AOSD) is a rare rheumatologic condition with unrecognized etiology. Spiking fevers, joint involvement (arthralgia or arthritis), rashes, lymphadenopathy, abnormal liver function test data, and leukocytosis are the main features of this disease. Yamaguchi’s criteria with the sensitivity and specificity of 96.2% and 92.1%, respectively, is the most beneficial tool for the diagnosis of AOSD, as a rare disease. The uncommon features of the still disease remain confusing for clinicians. about the present study reported a 27-year-old patient who was referred to our hospital with an unknown diagnosis, because of the rare features of Still disease. The explored characteristics of rashes, fever, and para-clinical data, as well as laboratory and imaging data, are described in detail; such features have led to long hospitalization to confirm the diagnosis in this patient. The positive family history of the rheumatologic disease and dramatic response to low dose corticosteroids were other outstanding features of our case. This report highlighted the necessity of conducting randomized clinical trials to address the management of AOSD. Besides, the present study signified the need for providing further awareness among clinicians to prevent long hospitalization.

Brain Abscess Due To Asian Nocardia in a 63 Years-Old Patient with Adult Still´S Disease: A Rare Case Report and Literature Review

Cerebral nocardiosis is a rare and opportunistic pathology caused by a gram-positive, aerobic bacterium of the order of Actinomycetes called Nocardia spp. It appears in patients with immunodeficiencies affecting cellular immunity. Nocardia can affect an organ (lungs in 75% of cases) or manifest itself as a disseminated infection (central nervous system in 25% of cases). Here we present a rare case of 63-years-old patient immunocompromised by monoclonal biologic treatment for Adult-Onset Still disease (AOSD), who subsequently presents fever, signs of motor focality and resonance imaging findings which show supra and infratentorial lesions. The patient is managed in intensive therapy for sensory impairment, receiving standardized antibiotic scheme, getting through to complicated evolution. Cerebral nocardiosis is a rare infectious disease that requires an early diagnostic study and its respective treatment.

Adult Onset Still Disease

Introduction. Adult Onset Still Disease (AOSD) is a systemic inflammatory disease, of unknown etiology, affecting young adults. It is, at least in part, a diagnosis of exclusion. Characterized by high fever with spikes, with or without complete defervescence outside of said spikes, arthritis, and evanescent rash. Also, sore throat, hepatomegaly, splenomegaly, serositis, lymphadenopathy might be present. Fatal risk is mainly attributed to Macrophage Activation Syndrome. Biologically its main trait is a marked inflammatory syndrome with unusually elevated ferritin levels. Case presentation. 31 year old male, known with a history of alopecia areata, treated with Diprophos, last dose one month prior to admission, with fever, odynophagia, sore throat, diffuse myalgia, debuted twelve days before. On blood samples, at admission and during his hospital stay, marked inflammatory signs are present (elevated ESR up to 100mm/h, C-reactive protein up to 37.4mg/dl and marked ferritin of 6240 ng/ml) accompanied by leukocytosis with neutrophilia, lymphopenia, low grade normochromic, normocytic anemia, thrombocytosis, slightly elevated liver function tests, cholestasis (GGT 502 U/L, ALKP 255 U/L) with normal bilirubin, D-dimers over 3000 with lowering values to 1344, spontaneous INR at 1.57. Normal values for rheumatoid factor, no antinuclear antibodies present, negative serology for hepatitis B, C, HIV, atypical bacterial infections (Chlamydia, Coxiella, Mycoplasma) Epstein-Barr Virus, syphilis, vasculitis markers (pANCA, cANCA) and anti-double stranded DNA, all coupled with 5 different hemocultures and 3 different urocultures all negative. Repeated ENT evaluation was within normal, chest X-Ray, echocardiography and CT scans of neck, thorax and abdomen reported back no significant abnormalities. Thus, by applying Yamaguchi criteria, after a complex evaluation of the case, the diagnosis is AOSD, and during his stay at the Internal Medicine department of Col ea Clinical Hospital, despite antiinflammatory, antithermic, antibiotic and corticosteroid treatment, the patient remains symptomatic, with high fever (up to 38.8 degrees Celsius) with chills and diffuse myalgias. At the indication of a rheumatologist, inside a specialty clinic, pulse-therapy with Methylprednisolone is initiated (500mgs a day for 5 days) with fever remission for more than 72 hours. The corticosteroid treatment is continued at home, with the patient self-monitoring for symptoms. Two months after corticoid therapy was initiated, the patient is almost without any symptoms (alopecia areata still present partially) and the inflammatory syndrome is greatly diminished. Conclusion. We present the case of a 31 year old male, with AOSD, defined by Yamaguchi criteria, with persistent symptomatology, mainly as high fever, without response to usual treatment, with the exception of pulse-therapy with Methylprednisolone. The diagnosis proved to be difficult, in part being one of exclusion, but also the clinical presentation, so unspecific, might easily lead to a different diagnosis.

Neutrophil-to-Lymphocyte Ratio and Platelet-to-Lymphocyte Ratio in Adult-Onset Still Disease, their Relationship with Baseline Disease Activity and Subsequent Disease Course: A Single-Center Retrospective Cohort Study

Introduction: Adult-onset Still Disease (AoSD) is a rare systemic polygenic nonfamilial autoinflammatory disease of unknown aetiology. The long-term course of the disease can be categorised in three different definitions including self-limited course, intermittent course or chronic course. Recently, Neutrophil-to-Lymphocyte Ratio (NLR) and Platelet-to-Lymphocyte Ratio (PLR) were investigated in various rheumatic diseases as an informative markers in evaluating severity of inflammation and disease activity. Aim: To explore association between baseline NLR, PLR, disease activity score and subsequent disease course in patients with AoSD. Materials and Methods: This retrospective cohort study enrolled 61 patients with AoSD and 61 age-matched patients with Fibromyalgia Syndrome (FMS). Pouchot score was specifically used in AoSD patients to assess disease activity based on symptoms, physical examination findings and laboratory results from April 2020 to July 2020. Patients with AoSD were subgrouped into three groups: self-limited; intermittent; and chronic course. The association of NLR and PLR with disease activity score was analysed between groups by using independent samples t-test, Mann-Whitney U test and Kruskal Wallis Variance Analysis. Differences between categorical variables were analysed using chi-square test. A p≤0.05 was considered statistically significant. Results: The mean follow-up of the 61 patients with AoSD was 74 months (range, 14-169). Eighteen patients (29.5%) had a self-limited disease course, nine (14.8%) an intermittent disease course and thirty four (55.7%) a chronic disease course. AoSD patients had significantly higher serum NLR, PLR and lower Mean Platelet Volume (MPV) values than FMS patients {6.68 (1.67-19.7), 1.83 (1.1-4) p=0.0001; 187 (82.9-549), 114 (72-246) p=0.0001; 8.3 (6.4-11.3), 9.3 (7.7-11.7) p=0.0001, respectively}. NLR, PLR and Pouchot score were similar among AoSD subgroups, which were grouped according to disease pattern. The majority of patients in the self-limited and chronic course groups had higher baseline Pouchot score without statistical significance. The NLR and C-Reactive Protein (CRP) were significantly higher in AoSD patients with active disease than inactive disease {7.02 (1.8-19.7), 4.17 (1.67-14.8) p=0.06; 13 (1.9-29.5), 9 (1.6-20.9) p=0.046, respectively)}. Conclusion: High NLR and elevated CRP levels are related to active disease in AoSD patients. Although NLR, PLR and Pouchot score were similar among subgroups, patients with a chronic course or self-limited course had higher NLR values and more active disease at diagnosis compared with patients with an intermittent course.