Rheumatoid factor (RF) is found commonly in patients with systemic lupus erythematosus (SLE), and has been associated with a more benign disease course.1,2 Anti-citrullinated peptide antibodies (ACPA) are more specific for rheumatoid arthritis (RA).3–5 Several assays for ACPA detection have been developed: among others, an enzyme linked immunosorbent assay (ELISA) for anti-cyclic citrullinated peptide (anti-CCP) antibodies3 and a line immunoassay (LIA) for antibodies to peptide A (pepA) and peptide B (pepB), two synthetic citrullinated peptides.4 Few reports exist about the presence of ACPA in SLE. Although patients with SLE are often part of the control group when determining the specificity of ACPA for RA, SLE alone is seldom studied. Mediwake et al found that 3/66 …

https://ard.bmj.com/content/annrheumdis/64/2/330.full.pdf

Presence of rheumatoid factor and antibodies to citrullinated peptides in systemic lupus erythematosus

This article presents an updated overview of hypertrophic osteoarthropathy and digital clubbing for the practicing rheumatologist. Discussion includes a brief historical perspective, its definition, incidence and prevalence, classification, pathology and pathophysiology, clinical manifestations, demographics, findings on physical examination, imaging techniques for its detection, differential diagnosis, and treatment modalities.

https://www.rheumatic.theclinics.com/article/S0889-857X(13)00009-4/pdf

Hypertrophic Osteoarthropathy: What a Rheumatologist Should Know About this Uncommon Condition

Giant cell arteritis (GCA) is a medium and large-vessel vasculitis, which is an important cause of secondary headache in older adults. While GCA has a classic presentation occurring after the age of 50, atypical presentations (eg, fever of unknown origin, cough, low or normal erythrocyte sedimentation rate) may lead to a delay in diagnosis. The topography of vascular involvement has implications for disease-related complications, which can result in neurologic disease at multiple levels of the nervous system. The most feared complication, vision loss, fortunately becomes uncommon after initiation of corticosteroids. Corticosteroid treatment should not be withheld while waiting the results of a temporal artery biopsy (TAB), which remains the gold standard for GCA diagnosis. Newer diagnostic modalities, including ultrasound, magnetic resonance imaging, and positron emission tomography can play an important role in directing treatment in cases with negative TAB. After successful control of the disorder, patients should be gradually tapered off corticosteroids, with careful monitoring using both clinical and laboratory parameters to assess for relapse. Corticosteroid-related treatment complications are not uncommon in GCA. There is mixed evidence for use of adjunct corticosteroid-sparing agents (eg, methotrexate), although these should be initiated in the setting of corticosteroid-related morbidity and/or cases with frequent relapse.

Giant Cell Arteritis

Felty’s syndrome (FS) is defined by the coexistence of rheumatoid arthritis (RA), neutropenia, and splenomegaly. The mechanisms underlying the neutropenia of FS may involve both cellular and humoral immunity, with a possible role of granulocyte-colony stimulating factor (G-CSF) antibodies.1 Various disease modifying antirheumatic drugs have been used to treat FS, but with varying success2 as this syndrome may arise in response to the excessive immune reaction found in RA. Interest has focused recently on a new biological tool in the treatment of RA, rituximab, a chimeric monoclonal antibody specific for human CD20 which targets B lymphocytes.3 Accordingly, we investigated here the safety and efficacy of rituximab in two patients presenting with active RA and severe and refractory FS.

Two men, …

https://ard.bmj.com/content/annrheumdis/64/2/332.full.pdf

Lack of efficacy of rituximab in Felty’s syndrome

As knowledge of the epidemiology of primary systemic vasculitides (PSV) is fragmentary, we attempted to investigate the incidence of temporal arteritis (TA), Takayasu’s arteritis (TAA), polyarteritis nodosa (PAN), Wegener’s granulomatosis (WG), Churg-Strauss syndrome (CSS), Henoch-Schonlein purpura (HSP), and hypersensitivity vasculitis (HSV) in Vilnius according to the American College of Rheumatology (ACR) 1990 criteria and to compare the data with the results from selected European studies.1

To be included in this study the patients had to ( a ) have been diagnosed with systemic vasculitides in the 10 year period from 1990 to 1999 and ( b ) have been resident in Vilnius at the time of diagnosis. Patients referred to Vilnius University Hospital rheumatology department were prospectively included in the study. Also, the patients’ registration books from tertiary nephrology, dermatology, and …

https://ard.bmj.com/content/annrheumdis/64/2/335.full.pdf

Incidence of primary systemic vasculitides in Vilnius: a university hospital population based study

https://www.jrheum.org/content/jrheum/28/12/2758.full.pdf

Localized polyarteritis nodosa with periostitis.

Resume Introduction La pericardite est un mode de revelation rare de la maladie de Horton. Nous rapportons deux nouveaux cas. Observations Un homme et une femme de plus de 70 ans etaient hospitalises pour douleur thoracique. Le diagnostic retenu etait celui de pericardite confirmee par l’echocardiographie. Le syndrome inflammatoire etait marque. L’interrogatoire retrouvait a posteriori des signes cephaliques de maladie de Horton. Les biopsies d’artere temporale etaient caracteristiques de la maladie. Le traitement par prednisone (20 mg/j) permettait une remission complete, rapide et sans rechute clinique ulterieure de la pericardite, avec respectivement un an et trois ans de suivi. Conclusion La maladie de Horton doit etre discutee et recherchee cliniquement en cas de pericardite chez le sujet âge car la corticotherapie s’impose afin de prevenir les complications vasculaires de cette maladie. Sous corticoides, il n’a jamais ete rapporte de tamponnade ni d’evolution vers la constriction.

Péricardite inaugurale d’une maladie de Horton

Maladie de Hodgkin avec marqueurs cellulaires du virus Epstein-Barr compliquant l'évolution d'un syndrome de Gougerot-Sjögren

Resume Les tumeurs primitives de l'aorte sont extremement rares. Une patiente de 73 ans presente une alteration de l'etat general avec un amaigrissement de 8 kg et des troubles digestifs a type de douleurs abdominales. L'examen clinique est normal. Le bilan biologique retrouve un important syndrome inflammatoire. Le scanner thoracoabdominal montre des hypodensites diffuses spleniques, des hypodensites renales et un aspect de thrombus de l'aorte thoracique s'etendant sur 9 cm. L'echocardiographie transœsophagienne montre une importante masse heterogene flottante dans la lumiere arterielle de l'aorte thoracique descendante evoquant un thrombus developpe aux depens de plaques d'atherome. Du fait du fort risque potentiel emboligene et dans le but d'obtenir un diagnostic, est realise un remplacement de l'aorte thoracique descendante. L'anatomopathologie de la piece operatoire retrouve un angiosarcome epithelioide de l'aorte. Il faut evoquer la possibilite d'une tumeur primitive de l'aorte devant un processus d'allure thrombotique intra-aortique.

Angiosarcome épithélioïde de l'aorte

Des douleurs abdominales au cours d’une thrombocytémie essentielle

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