A 67–year-old white woman with primary Raynaud’s phenomenon presented following a week of progressively worsening shortness of breath, dry cough and generalized malaise. An avid tennis player, she first noticed dyspnea while playing, but a few days later grew short of breath even at rest. She went to an urgent care center, where a computed…
In a multicenter, prospective, open-label study, ustekinumab therapy was effective in treating oral ulcers resistant to colchicine in patients with Behçet’s disease, according to study author David Saadoun, MD, PhD, Department of Internal Medicine and Clinical Immunology, Sorbonne University, Paris, and fellow researchers.1 Researchers focused on the topic because oral ulcers are often disabling, have…
A 13-year-old, adopted girl of unknown ancestry with social anxiety, selective mutism and Takayasu arteritis presented for evaluation of severe, painful, gingival hyperplasia, which limited her oral intake and resulted in weight loss. The young patient was diagnosed with Takayasu arteritis at age 8, when she presented with a persistently elevated erythrocyte sedimentation rate (ESR),…
Primary hemophagocytic lymphohistiocytosis (HLH) is caused by genetic mutations and inherited syndromes; it therefore occurs in the pediatric age group. Secondary HLH, however, is more common in adults and is often triggered by other disease states, such as malignancies, chronic immunosuppression, infections and autoimmune disease.1,2 Macrophage activation syndrome (MAS) is a subset of secondary HLH…
SNOWMASS VILLAGE, COLO.—The study of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is ongoing, and research results should help improve treatment for this patient population. Key trials and therapeutic options were discussed at the 2020 ACR Winter Symposium during the session, Update on the Treatment of ANCA-Associated Vasculitis, by Peter A. Merkel, MD, MPH, chief of…
Since it was first described, the spectrum of disease caused by deficiency of adenosine deaminase 2 (DADA2) has been broadening. Features described include systemic vascular and inflammatory features and recurrent stroke, which overlap with childhood-onset polyarteritis nodosa (PAN). Previous data show that DADA2 has extensive genotypic and phenotypic variation.1 Results from a recently published study…
Researchers suggest antiviral therapy is not appropriate for patients with GCA, based on their study findings and related research…
A rare form of large vessel vasculitis, Takayasu arteritis persents with no clear patterns, with patients experiencing vascular symptoms, as well as such systemic symptoms as fever and weight loss. A systematic literature review found evidence to guide rheumatologists in monitoring and treating their patients with Takayasu arteritis…
A systematic literature review identified novel evidence on the treatment and management of GCA that was incorporated into the most recent EULAR recommendations on the management of large vessel vasculitis. Investigators confirmed the efficacy of prompt initiation of glucocorticoids and found fast-track approaches to diagnosis lowered the risk of ischemic complications…
A 42-year-old man with a history of ulcerative colitis (UC), primary sclerosing cholangitis (PSC) and chronic sinusitis was referred to a rheumatologist to evaluate for a possible diagnosis of systemic vasculitis. This patient had developed new skin lesions, gingival hypertrophy and ulcerating tracheobronchitis, concerning for possible granulomatosis with polyangiitis (GPA). Since 1994, the patient had…