Elevated Acute Phase Reactants Research Articles

Exclusively ocular and cardiac manifestation of granulomatosis with polyangiitis \u2013 a case report

BackgroundGranulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibodies (ANCA)-associated necrotizing granulomatous vasculitis that affects small to medium size vessels. While the classical form with renal and respiratory tract involvement is mainly seen, a limited form (i.e., with no renal disease) may also occur. We present an unusual case of GPA manifesting merely as a bilateral ocular involvement and complete heart block.Case presentationWe report a case of a 60-year-old male patient with a limited form of GPA who initially presented with bilateral chronic conjunctivitis and complete atrioventricular block. His visual acuity subsequently declined due to progression to bilateral panuveitis with exudative retinal detachment. The laboratory investigation revealed the elevation of acute phase reactants and strongly positive cytoplasmic ANCA (c-ANCA). Despite negative conjunctival and musculocutaneous biopsy results, the positive c-ANCA, and the clinical manifestation, i.e., heart and ocular involvement, led to the diagnosis of GPA. The remission was achieved with cyclophosphamide and methylprednisolone systemic therapy.ConclusionsA limited form of GPA may be a diagnostic chameleon. Though rare, it is essential to consider even extremely uncommon findings. Our patient is the first case of such a unique demonstration of the limited GPA manifesting as a bilateral ocular involvement and complete heart block.

Bilateral suppurative parotitis in a neonate: a case report

Acute suppurative parotitis is a very rare ailment to be encountered in the neonatal period. Here, we report a 13-day old hemodynamically stable neonate, who presented to us with bilateral tender, erythematous parotid swelling and purulent discharge exuding from stensen’s duct. He was exclusively breast fed and had no other risk factors. Provisional diagnosis of acute suppurative parotitis was made with clinical examination. On investigating, there was neutrophilic leukocytosis, elevated acute phase reactants, ultrasonogram showed hypoechoic areas and a heavy growth of Staphylococcus aureus was isolated on pus culture. Baby underwent incision and drainage on the day of admission and was started on appropriate parenteral antibiotics along with supportive management. There was a significant clinical response over next 24 hours. He was doing well throughout the hospital stay and antibiotic course was completed based on culture sensitivity pattern. He neither had any immediate complications during hospital stay nor developed any late complications on follow up. Prognosis of the condition is excellent with adequate timely management. Authors would like to report this case for its rarity and to emphasize on fact that though the condition is uncommon, acute neonatal parotitis should be included in the differentials of any unilateral or bilateral neonatal parotid swelling, as early diagnosis and prompt management of this entity is mandated for a favorable outcome and to limit complications drastically.

The impact of temporal artery biopsy for the diagnosis of giant cell arteritis in clinical practice in a tertiary university hospital.

BackgroundTemporal artery biopsy (TAB) is useful in assisting with giant cell arteritis (GCA) diagnosis but lacks sensitivity. The aim of our study was to assess the diagnostic impact of TAB histology in patients with suspected GCA on hospital admission.MethodsA prospectively maintained database was queried for all TABs performed between 1-1-2000 until 31-12-2017 at the University Hospital of Ioannina. Thus, inclusion criteria were made on the grounds of every patient that underwent a TAB during the above-mentioned period, regardless of demographic, clinical and laboratory data.ResultsTwo hundred forty-five TABs were included (149 females and 96 males), with a mean age of 64.5 (±3.5) years. The mean symptoms duration until admission to the hospital was 8.6 (±1.3) weeks and all had elevated acute phase reactants on admission. The reasons of admission were fever of unknown origin (FUO) in 114 (46.5%) patients, symptoms of polymyalgia rheumatica (PMR) in 84 (34.3%), new headache in 33 (13.5%), anemia of chronic disease (ACD) in 8 (3.32%) and eye disturbances in 6 (2.5%) patients. Positive results were found in 49 (20%) TABs. More specifically, in 14% of patients with FUO, 21% in those with PMR, while in patients with a new headache the percentage was 27%. Finally, 5 out of 6 (83.3%) of patients with ocular symptoms and only one (12.5%) of those suffering from ACD. Visual manifestations and FUO are correlated with a positive TAB.ConclusionIt seems that TAB is useful in assisting with GCA diagnosis, but lacks sensitivity.

Is it required to routinely check fibrinogen level in patients with rheumatic diseases on tocilizumab? Case-based review

Tocilizumab (TCZ) may rarely cause hematological side effects including neutropenia and thrombocytopenia. TCZ is essentially expected to lower the fibrinogen levels to stay within the normal range, but TCZ-induced hypofibrinogenemia has been rarely reported in literature. Although it may remain asymptomatic, hypofibrinogenemia has clinical significance owing to the tendency of the condition to result in bleeding. A 65-year-old female patient with known polymyositis was, approximately 20years after the diagnosis was made, examined due to elevated acute phase reactants leading to the diagnosis of giant cell arteritis (GCA) and TCZ treatment was initiated as she had former steroid-induced osteoporotic fractures. 1month after the initial dose of intravenous (IV) TCZ, she presented with ecchymosis and was detected to have hypofibrinogenemia. Following the administration of the second dose, hypofibrinogenemia was detected again. In this review, we have analyzed this patient in addition to the cases in six other articles of TCZ induced hypofibrinogenemia which we found out based on our search strategy. Our aim is to point out a rare side effect of TCZ, hypofibrinogenemia, thus to emphasize a possible bleeding disorder and discuss probable underlying mechanisms.

Chronic inflammation in adult familial Mediterranean fever patients: underlying causes and association with amyloidosis

Background: Chronic inflammation, as determined by persistently elevated acute-phase reactants in attack-free periods, can occasionally be observed in patients with familial Mediterranean fever (FMF) and is suggested to be a risk factor for the development of amyloidosis. We aimed to investigate the underlying causes of chronic inflammation in FMF patients and its association with amyloidosis in long-term follow-up.Method: Electronic medical records of FMF patients who had regular follow-up for ≥ 5 years in our cohort were utilized. As part of routine evaluation, detailed history, physical examination, and pertinent laboratory and radiographic investigations were performed in all patients to determine potential causes of elevated C-reactive protein (CRP) levels.Results: The study included 146 FMF patients who had no evidence of amyloidosis at baseline and had regular follow-up for ≥ 5 years. Thirty-seven patients (25.3%) were found to have chronic inflammation in the disease course. Twenty-five (67.5%) of them had either very frequent attacks or chronic manifestations of disease. In the entire study group, amyloidosis developed in five patients (3.42%) during the 5 year follow-up, four in the FMF with chronic inflammation group (10.8%), and only one of the 109 patients without chronic inflammation (odds ratio 13.09, 95% confidence interval 1.41–121.2).Conclusions: The results suggest that persistently high CRP levels during the attack-free periods may be a strong risk factor for the development of amyloidosis in patients with FMF. The vast majority of FMF patients with chronic inflammation had active FMF.

SCHNITZLER SYNDROME – IS ANTI-IL-1 THERAPY ALWAYS THE RIGHT CHOICE?

Introduction Schnitzler syndrome is a periodic fever syndrome presenting with urticarial skin rash, a monoclonal IgM component and at least two of the following: fever, joint or bone pain, lymphadenopathy, hepatosplenomegaly and elevated acute phase reactants. IL-1 is typically elevated, making anakinra a successful therapy. However, sarilumab (IL-6 antagonist) may be efficacious in certain patients. Case Description A 69-year-old female experienced monthly episodes of facial swelling and a non-pruritic, erythematous rash with high fevers, nausea, headache and neck pain over 1 year. During episodes, white cell count, neutrophils, monocytes, eosinophils, ESR and CRP were elevated but would resolve after episodes. Facial cellulitis, odontogenic and neurologic infections were excluded. Previous medical history was significant for breast cancer and uterine cancer in remission and Hashimoto's thyroiditis. Tryptase levels were normal, arguing against mast cell activation syndrome. Complement C2 (less than 1.3 mg/dL) and C4 (10mg/dL) levels were slightly low. The patient had normal immunoglobulin levels, IL-2, TNF-α, IL-1β levels and negative anti-SSA and anti-SSB, anti-Smith, anti- dsDNA, anti-mitochondrial and anti-actin antibodies but isolated elevated IL-6 levels and a monoclonal IgM component. Isolated elevated IL-6 made tocilizumab appropriate treatment with symptom resolution in days. However, after 4 months of therapy anaphylactic symptoms required changing to sarilumab with good effect. Discussion Although a decade older than expected and despite the presence of an atypical rash, symptoms were consistent with Schnitzler syndrome. Patients with elevated IL-6 levels who do not tolerate tocilizumab may respond well to other IL-6 antagonists such as sarilumab.

On demand use of anakinra for attacks of familial Mediterranean fever (FMF).

To evaluate the efficacy of on-demand use of anakinra in patients with crFMF. The Gazi FMF cohort was established in the year 2010, and from that date, 689 patients with FMF diagnosed according to the Tel Hashomer criteria were registered. Attack type, duration, severity, and their impact on life were collected either by disease diaries or a mobile phone application (FMF AIDD, AppStore, and Playstore). A retrospective cohort analysis was made from records of patients who have ever been treated with IL-1 inhibitors. A total of 78 patients were treated with IL-1 inhibitors in our cohort. Among these, 15 patients were identified who received on-demand anakinra. Rationale for on-demand use was prominent prodrome or trigger for attacks and patient's personal claim. Six patients were switched from regular use and nine were directly started as on-demand use. All were using background colchicine in maximum tolerated doses. None of the patients had evidence of persistently elevated acute phase reactants or proteinuria. The median duration of on-demand anakinra use was 6 (3-36)months. Pre- and post- on-demand anakinra periods were compared. Patient reported attack severity (p = 0.002), duration (p = 0.001), frequency (p = 0.001), absenteeism (p = 0.002), and presenteeism (p = 0.002) were significantly improved. On-demand anakinra prevented progression of prodromes to full-blown attacks which was demonstrated by decrease in the rate of attack/prodrome ratio (p = 0.02). On-demand anakinra can be continued in ten subjects on long-term. On-demand anakinra significantly improved FMF attacks in certain patients which suggest this approach would be of benefit in daily practice in selected patients.

A Case of Familial Mediterranean Fever Having Intermittent Leukopenia

Familial Mediterranean fever (FMF) is a genetically inherited autoinflammatory disorder characterized by inflammatory attacks and may result in amyloidosis as a severe complication. Elevation of acute phase reactants, including leukocytosis, is seen during attack periods. Here we describe a 13-year-old female patient with a very rare clinical presentation of FMF, who would experience FMF attacks when she did not regularly take her colchicine. During these attacks she had leukopenia and neutropenia instead of leukocytosis. The leukocyte count returned to normal when she continued the medication and avoided attacks. Ethnicity and clinical signs are important in leukopenic patientsand should be investigated for FMF to avoid unnecessary procedures and complications.

A20 haploinsufficiency (HA20): clinical phenotypes and disease course of patients with a newly recognised NF-kB-mediated autoinflammatory disease

ObjectivesThe association between mutations in TNFAIP3, encoding the NF-kB regulatory protein A20, and a new autoinflammatory disease has recently been recognised. This study aims at describing the clinical phenotypes and...

Síndrome del hombre en el barril: manifestación atípica de la arteritis de células gigantes

«Man-in-the-barrel» syndrome refers to diplegia of the upper extremities in which mobility of the head and lower limbs is preserved. Brachial plexitis that presents as «man-in-the-barrel» syndrome is an unusual manifestation of giant cell arteritis. We report a case of C5-C6 plexitis as part of the clinical features of a patient with giant cell arteritis. A 70-year-old male with a two-month history of weight loss, headache, facial pain and jaw claudication, associated with a persistent elevation of acute phase reactants and bilateral brachial plexopathy, with no evidence of neck or brain injuries or occult neoplasm and with negative autoimmunity tests. Results of the biopsy study of the temporal artery were compatible with giant cell arteritis, and the positron emission tomography scan revealed extensive vascular involvement of the aorta and its branches. Although the typical clinical manifestations of giant cell arteritis are headache, jaw claudication, loss of sight, constitutional symptoms and polymyalgia rheumatica, its presence must be suspected in patients over the age of 50 who manifest alterations affecting the peripheral nerve, including brachial diplegia with no other demonstrable cause.

Effects of an Anti-RANKL Antibody Denosumab on Joint Structural Damage in Patients with Rheumatoid Arthritis Treated with Conventional Synthetic Disease-Modifying Anti-Rheumatic Drugs (DESIRABLE Study): A Randomised, Double-Blind, Placebo-Controlled Phase 3 Trial

Background Rheumatoid arthritis (RA) is commonly treated with conventional synthetic disease-modifying antirheumatic drugs (csDMARDs); however, joint destruction is not always fully suppressed. We evaluated the efficacy of denosumab that was added to csDMARD therapy for RA patients. Methods This randomised, double-blind, parallel-group, placebo-controlled phase 3 study was conducted at 104 hospitals in Japan. We enrolled RA patients aged ≥20 years receiving csDMARDs, with erosive disease and elevated acute phase reactants. Participants were randomly assigned (1:1:1) to subcutaneously receive denosumab 60 mg every 3 months (Q3M), a matching placebo every 3 months, and denosumab 60 mg or the placebo every other 3 months (Q6M). The primary endpoint was the change in the modified total Sharp score (mTSS) from baseline to 12 months and analysed using the van Elteren stratified rank test adjusted for randomised strata. The effect of denosumab on bone mineral density (BMD) was also evaluated. Findings In total, 654 patients received treatments (218 had placebo; 217 had denosumab Q6M; and 219 had denosumab Q3M). Compared with the placebo group, both denosumab groups showed significantly less progression of joint destruction. The mean changes in the mTSS were 1·49±3·76 in the placebo group, 0·99±3·77 in the Q6M group (p=0·0235), and 0·72±2·32 in the Q3M group (p=0·0055). The percentage changes in lumbar spine (L1-L4) BMD from baseline in the placebo, Q6M, and Q3M groups were -1·03%, 3·99% (p<0·0001) and 4·88% (p<0·0001), respectively. No major differences were observed between the safety profiles of denosumab and placebo. Interpretation Denosumab inhibits the progression of joint destruction, increases BMD, and is generally well tolerated in RA patients taking csDMARD. This offers a potentially novel therapeutic option for preventing the onset and progress of structural damage and increasing BMD in RA patients treated with csDMARD. Trial Registration Number: This study is registered with ClinicalTrials.gov, number NCT01973569. Funding Daiichi Sankyo Co., Ltd. Declaration of Interest: TT has received research grants from Astellas, Chugai, Daiichi Sankyo, Takeda, AbbVie, Asahi Kasei, Mitsubishi Tanabe, Pfizer, Eisai, AYUMI, Nippon Kayaku, and Novartis, has received consulting fees from Astra Zeneca, Eli Lilly, Novartis, Mitsubishi Tanabe, AbbVie, Nippon Kayaku, Janssen, Astellas, Taiho, Chugai, Taisho Toyama, GlaxoSmithKline, and UCB, and has served on speakers’ fees for AbbVie, Bristol-Myers Squibb, Chugai, Mitsubishi Tanabe, Pfizer, Astellas, Daiichi Sankyo, Eisai, Sanofi, Teijin, Takeda, and Novartis. YT has received research grants from Mitsubishi Tanabe, Takeda, Bristol-Myers Squibb, Chugai, Astellas, AbbVie, MSD, Daiichi Sankyo, Pfizer, Kyowa Hakko Kirin, Eisai and Ono, and has served on speakers’ bureaus for Daiichi Sankyo, Astellas, Pfizer, Mitsubishi Tanabe, Bristol-Myers Squibb, Chugai, YL Biologics, Eli Lilly, Sanofi, Janssen, and UCB. SS has received grant/research support from Chugai and Daiichi Sankyo, and has served on speakers’ bureaus for Asahi-Kasei Pharma, Astellas, MSD, Daiichi Sankyo, Takeda, Chugai, Teijin Pharma, Pfizer, Eli-Lilly Japan, and Mitsubishi-Tanabe. HY has received research grants from MSD, Astellas, AbbVie, BMS, Kaken, UCB, Ono, AYUMI, Eisai, Daiichi Sankyo, Takeda, Mitsubishi Tanabe, Chugai, Teijin, Torii, Nippon Shinyaku and Pfizer, and has received consulting fees from Pfizer, YL Biologics, Takeda, Teijin, BMS, Nippon Kayaku, Chugai, Mitsubishi Tanabe, Daiichi Sankyo, and Astellas. TY has received Grants-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology of Japan (MEXT #17H04377), and has received consulting fees from Daiichi Sankyo. ST has acted as a consultant for Amgen, Astellas, MSD, AbbVie, Daiichi Sankyo, Eli-Lilly, Ono, Asahi Kasei Pharma, and Teijin Pharma. TN is an employee of Daiichi Sankyo. NO is a shareholder and employee of Daiichi Sankyo. HKG. has received consulting fees from Daiichi Sanyo, Pfizer, Amgen, Bioclinica, Eli Lilly, Janssen, Servier, Novartis, l Takeda, Merck, Biomarin, Clementia, Agnovos, Regeneron and Medimmune. DvdH. has received consulting fees from AbbVie, Amgen, Astellas, AstraZeneca, BMS, Boehringer Ingelheim, Celgene, Daiichi Sankyo, Eli Lilly, Galapagos, Gilead, GlaxoSmithKline, Janssen, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi, Takeda and UCB, and is the director of Imaging Rheumatology BV. Ethical Approval: The study was approved by the institutional review boards of all participating sites and was conducted in accordance with the principles of the Declaration of Helsinki. Written informed consent was obtained from all participants.

Approach to Growing Pains in Childhood in a Familial Mediterranean Fever Endemic Region

: Growing pain, still preserving its charm, is the most common form of recurrent musculoskeletal pain in childhood. Approach to diagnosis requires significant care in geographical regions with familial Mediterranean fever (FMF) endemicity. We discuss data and a current approach to growing pains in children in an FMF endemic region. The symptomatology may be confused with different diseases. Although unnecessary tests may be performed to reach diagnosis, sometimes patients may be misdiagnosed as having growing pain without revealing the primary underlying pathology. Definite algorithms are not developed for diagnosis of growing pains. Diagnosis is mainly based on typical clinical features of the condition. However laboratory tests and imaging methods may be used for diagnosis and differential diagnosis especially in atypical course suggesting diseases such as inflammatory ones or malignancies. Vitamin D deficiency should be investigated to give supplementation if necessary. Some helpful algorithms may be used in evaluation of patients. In order to evaluate myalgia as growing pain in areas where FMF is endemic, fever, abdominal pain, elevated acute phase reactants and history of FMF and/or amyloidosis in family should be carefully excluded. It is important to inform parents about the alarming symptoms of extremity pain and when to urge medical advice again.

Long-term follow-up of paediatric MEFV carriers.

Although familial Mediterranean fever (FMF) is inherited autosomal recessively, some heterozygotes may express disease phenotype and require therapy. To date, there is no study in the literature about how to follow-up Mediterranean fever (MEFV) heterozygotes who do not fulfil FMF criteria in the paediatric age group. This study aims to share a single-centre experience of the long-term clinical and laboratory follow-up of paediatric MEFV carriers. We reviewed the charts of 69 children who were heterozygous for MEFV variants. All children were followed-up with their routine analysis and serum amyloid A levels every 6months. Thirty-nine children had pathogenic mutations and 30 children had variants of unknown significance. The mean follow-up was 3.2±1.6years (min 2years, max 6years). The children with pathogenic mutations had significantly higher mean SAA levels than the children with variants of unknown significance (p=0.018); however, the mean CRP and ESR were similar. Besides, the children with pathogenic mutations complained of fever episodes significantly more than the children with variants of unknown significance (p=0.04). None of the children had persistent proteinuria in the follow-up. We started colchicine in only two patients who were M694V heterozygous. Both patients had family history for FMF and fulfilled the disease criteria after 2years of follow-up. Neither of these patients had persistently elevated acute phase reactants in their routine follow-up. This study suggested that routine clinical follow-up is useful; however, routine periodic laboratory workup is not necessary among MEFV carriers.

Roles of Loss of Chromosome 14q Allele in the Prognosis of Renal Cell Carcinoma with C-reactive Protein Abnormity.

Background:Renal cell carcinoma (RCC) is frequently associated with paraneoplastic inflammatory syndrome (PIS). This study aimed at exploring the connections between the survival rate and specific gene alterations and the potential mechanism.Methods:We retrospectively studied 69 surgical RCC cases from August 2014 to February 2016, including 18 cases of clear cell RCC (ccRCC) demonstrating elevated pretreatment serum C-reactive protein (CRP, Group A). Twelve of the 18 cases were symptomized with febrile episode. We also selected 49 cases of ccRCC with normal pretreatment CRP (Group B). Using 22 microsatellite markers, we compared the incidence of loss of heterozygosity (LOH) between Group A and Group B. All statistical tests are two-sided.Results:The 3p LOH was common in both Group A (89%) and Group B (92%). The frequency of 14q LOH in Group A (16 of 18) was higher than Group B (4 of 49, χ2 = 40.97 P < 0.0001). The 3p and 14q LOH were the characteristics of ccRCC with elevated acute phase reactants, including PIS, regardless of the presence of metastasis. On the contrary, 14q LOH was a rare genomic alternation in advanced-staged ccRCC without PIS. The overall survival of patients with elevated CRP (33.3%) was lower than its counterparts (6.1%, hazard ratio=1.852, P < 0.0001) in Kaplan-Meier curve.Conclusions:The results imply that the disruption of a 14q gene(s) might result in not only the inflammatory manifestations in the tumor host but also the poor survival rate as well. The isolation of the gene(s) on 14q might be a vital goal in the treatment of PIS-associated RCC.

Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain.

The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9.7years. In the overall cohort, the 2-, 5-, and 10-year survival probabilities were 91.9, 86.7, and 77%, respectively. Main causes of death were infections and cancer (24% each). Multivariate model revealed that CAM (HR=24.06), OM (HR=12.00), DM (HR=7.26), higher age at diagnosis (HR=1.02), severe infections (HR=3.66), interstitial lung disease (HR=1.61), and baseline elevation of acute phase reactants (HR=3.03) were associated with a worse prognosis, while edema of the hands (HR=0.39), female gender (HR=0.39), and longer disease duration (HR=0.73) were associated with a better prognosis. The standardized mortality ratio was 1.56 (95% CI 1.28-1.87) compared to the Spanish general population. Our findings indicate that IIM has a high long-term mortality, with an excess of mortality compared to the Spanish population. A more aggressive therapy may be required in IIM patients presenting with poor predictive factors.

46-Year-Old Man With Lower Back Pain

46-Year-Old Man With Lower Back Pain

Characteristics of Turkish patients with elderly onset psoriatic arthritis: A retrospective cohort study.

Psoriatic arthritis (PsA) is a chronic inflamatory disease characterized with axial and peripheral joints involvement. It rarely affects patients older than 65 years old.The purpose of this study is to compare and evaluate the demographic, clinical and laboratory features of elderly-onset psoriatic arthritis (EOPsA) and young-onset (YOPsA) patients.A total of 180 patients diagnosed with PsA according to CASPAR criteria and followed-up in single center were included in this study. The patients with initial symptoms started after age 65 were accepted as EOPsA. Demographic, clinic, and laboratory data and the medications which the patients received were recorded and retrospectively evaluated.Nineteen (10.5%) of 180 patients were diagnosed as EOPsA, and 161 (89.5%) patients were evaluated as YOPsA. The mean patient age was 42.1years for the YOPsA group and 68.3 years for the elderly onset group. Mean duration of disease was 5.6 years for the early onset group and 1.3 years for the elderly onset group (P = .001). Fourteen (73.3%) of 19 EOPsA patients were female and 5 of them were male. Higher rates of fatique, pain scores, comorbid diseases, and acute phase reactants elevation were detected in EOPsA patients comparing to YOPsA (P = .000, P = .000, P = .001, and P = .001, respectively). YOPsA patients have more dactilitis, nail involvement, elevated PASI scores, and smoking habitus when compared with EOPsA patients (P = .019, P = .03, P = .005, P = .004, respectively). In terms of the treatment options chosen, there was no significant difference in the use of nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids (CS), methotrexate (MTX), and sulfasalazine (SSL), but there was a more frequent use of anti-tumor necrosis factor-alpha in the YOPsA group.YOPsA and EOPsA patients may presented with different clinical and laboratory features. EOPsA patients are characterized with higher rates of fatigue, pain scores, comorbid diseases, and acute phase reactants and less dactilitis, nail involvement, and anti-TNF-alpha usage.

Complete form pachydermoperiostosis in Tunisia – A case series and literature review

IntroductionPachydermoperiostosis (PDP) or hypertrophic osteoarthropathy (HOA) is a rare disease that has genetic predisposition and defined clinical features. Aim of the workTo study and analyse the clinical features of Tunisian PDP patients and review the literature. Patients and methodsThe PDP cases attending the Rheumatology Department of the University Hospital Farhat Hached of Sousse in Tunisia were retrospectively studied over a period of 17years (2000–2017). Secondary causes were excluded. ResultsThe PDP cases had a primary and complete form of the disease and included 6 men at a frequency of 0.03% (6/20,000) of the total number of rheumatic diseases cases attending the rheumatology clinic. The mean age was 27±12.3years [18–46years]. Five patients had arthralgia and one polyarthritis. Thickening of the skin of the head and distal extremities (pachyderma) and deep folds and furrows of the skin of the forehead was observed in all cases. Digital clubbing of the fingers and toes and spade-like enlargement of the hands and feet was noted in 5 cases. Hyperhidrosis of the hands and feet was observed in 4 cases and seborrhea in 2. Elevated acute phase reactants were found in 4 cases. Hypergammaglobulinemia was present in one case. Periostosis of the long bones was observed in all cases. Treatment consisted of analgesics in all cases, non-steroidal anti-inflammatory drugs in 2 cases and tamoxifen in 2 cases. ConclusionsComplete form of primary PDP is rarely present among rheumatic diseases patients in Tunisia. A multicenter larger number longitudinal study is recommended.

Primary Pyomyositis as Unusual Cause of Limp: Three Cases in Immunocompetent Children and Literature Review

AbstractPyomyositis (PM) is an uncommon primary skeletal muscle infection caused mainly by Staphylococcus aureus that is characterized by single or multiple intramuscular abscess formation. In our ward, between 2013 and 2015, three children (two females and one male) aged from 2 to 12 years were diagnosed and treated for PM. Patients' medical records and imaging studies were examined retrospectively. All patients, otherwise healthy, complained of limp, fever, and severe lower limb pain. Skin scratch lesions were detected in two cases; one of them showed an edematous appearance of the affected area. Multifocal bilateral abscesses of gemini and gastrocnemius were detected in the youngest patient; right obturator and iliac muscles were affected in the second patient; and right gluteus and pyriform muscles were involved in the third patient. All patients showed elevated acute phase reactants and had normal serum creatinine kinase levels. Blood cultures and polymerase chain reaction (PCR) investigations were negative in all cases. Magnetic resonance imaging (MRI) findings included muscle enlargement, deep fascia, high signal in subcutaneous tissues, and postgadolinium abscess formation. No patient required surgical or percutaneous drainage. All three were treated conservatively with intravenous oxacillin, associated with ceftriaxone in the first patient and ceftazidime in the other two, followed by oral antibiotic therapy for a period ranging from 5 to 6 weeks. Pyomyositis must be considered as an unusual cause of limp in children of all ages. When promptly diagnosed and adequately treated, it has a favorable outcome without relapses or sequelae as occurred in all our patients.

New Markers for Adult-Onset Still's Disease

Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder (SAID). Although the pathogenesis of the disease is complex and far from being fully understood, recent progresses in pathophysiological knowledge have paved the way to new diagnostic approaches. Indeed, AOSD diagnosis can be a real challenge, owing to its infrequency, and to the lack of specificity of the principal clinical features (high fever, arthralgia or arthritis, skin rash) and laboratory findings (elevated acute phase reactants, hyperleukocytosis≥10,000 cells/mm3 with neutrophils≥80%). None of these manifestations is disease-specific, so clinicians must first rule out neoplastic, infectious or inflammatory conditions. Besides these diagnostic difficulties, several other challenges remain. AOSD is very heterogeneous in terms of clinical presentation, evolution and severity. Thus, new biomarkers are required to assess: (i) disease activity; (ii) disease severity (through the identification of patients at risk of severe organ failure, and eventually of life-threatening complications, such as reactive haemophagocytic lymphohistiocytosis); (iii) disease evolution (which can be monophasic, relapsing, or progressive, with either systemic inflammation or chronic erosive arthritis); (iv) and treatment efficacy. The identification of new markers can only be done through a better understanding of the pathogenesis of the disease. After a short focus on the current AOSD pathophysiological knowledge, this article reviews the main biomarkers that have been proposed in the literature over the last few years.