~66% of patients present with sore throat secondary to cricothyroid perichondritis or aseptic nonexudative pharyngitis. IgG4 related disease is more common in men >50 years of age. Women are affected in 80 to 90 percent of cases, with an age of onset that is usually between 10 and 40 years. Official Valley IM Residency Site; Search for: Tag Archives: Rheumatology. The case highlighted the importance of a framework approach to diseases. Surgical excision for NBTE vegetation, can be considered in only selective cases and generally avoided. Majority men (62-83%) and > 50 years of age. Chronic: leads to fibrosis, which is NOT reversible. March 14, 2019 vmcimchiefs Leave a comment. Screen for malignancy in patients with new diagnosis of scleroderma! Fibromuscular dysplasia (look for string of beads on imaging! Thanks to Eric for presenting the case of a young woman who presented with acute onset of night sweats, chest pain, and fatigue on subacute symptoms of cough and unintended weight loss, with CT chest showing mediastinal fatty infiltration which turned out to be thickening of the aortic arch and descending arteries consistent with Takayasu arteritis! The disease is often recurrent. Described in 1897 by George Still, it is a systemic inflammatory disorder of unknown etiology. The Chief Residents' Blog. Primary Menu Skip to content. Arteriographic classification of Takayasu . Kernig and Brudzinski, when present, are highly specific. Bird fancier’s lung (feathers, bird droppings), Cheese-washer’s lung (Cheese Fancier per Sarasa), Sauna worker’s lungs (contaminated sauna water). Weaker associations: AEDs, antimicrobials, beta blockers, lithium, HCTZ, amiodarone, cipro etc. Can present with superficial thrombophlebitis, Raynaud’s, digital ischemia, other organ ischemia (cerebral, coronary, internal thoracic, renal, and mesenteric arteries), or joint complaints. Takayasu primarily affects young women with the greatest prevalence of disease noted in Asia. This was confirmed by a highly positive anti-HMGCR antibody, Adapted from a NEJM Article: https://www.nejm.org/doi/full/10.1056/NEJMra1402225. Dr. Sharp graduated from the University of Pecs Medical and Health Sciences Centre in 1999. Treatment similar to ANCA positive vasculitis, Patients typically present with constitutional symptoms, arthralgias/arthritis, and cutaneous vasculitis, Strongest association with hyperthyroidism meds, hydralazine, and minocycline (hydral is the most common), Rare, but should be aware of this association because it impacts management and because it is often not diagnosed until too late in the disease course. His clinical course was complicated by recurrent high daily fevers, a diffuse maculopapular rash, and knee arthrocenteses and joint washes that were clean leading to a diagnosis of Still’s disease! Presentation was initially concerning for septic arthritis, and joint washout revealed gross purulence from the shoulder joint. Rule in the diagnosis if at least three of the following are present: Claudication of the extremities (tends to happen later in the disease course and after systemic symptoms of fever and malaise have already started), Decreased pulsation of one or both brachial arteries aka “pulseless disease”, Bruit over one or both subclavian arteries (as in our patient!) For example, there is worse renal survival in MPO-ANCA patients and PR3-ANCA has consistently been shown to have a higher relapse rate and mortality rate compared to MPO-ANCA. MRA or CTA may not provide sufficient spatial resolution for the distal digits so it’s best to image the entire aorta and upper and lower extremities for evidence of disease that has not yet clinically manifested itself.
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