Today we went over a case from the HumanDx Project (Credit goes to Dr. Maki Cronin, SSM Health St. Louis University Hospital). Putting aside the CPC diagnosis for a moment, we see that there are consistent, specific associations between PR3-ANCA/MPO-ANCA and certain clinical manifestations. Predictors of poor outcome include erosive polyarthritis on presentation and shoulder/hip involvement. We reviewed that renal biopsy is not generally able to distinguish between the various AAVs, but rather will only be ‘pauci-immune’. GNR can be seen in older adults or in immunocompromised patients, 20% of cases can present as oligoarticular or polyarticular infection. PCR only required in rare cases since most non-gonococcal cultures obtained prior to antibiotics return positive. Obstruction more commonly seen in chronic. Learn what to expect during your hospital stay. NBTE (Non-bacterial thrombotic endocarditis). Serum serologies are only suggestive. Thromboangiitis obliterans (Buerger’s disease). ), Other diseases that can present with large-vessel vasculitis/aortitis such as Cogan’s syndrome, relapsing polychondritis (can cause aortic and mitral regurg), and spondyloarthropathies. IgG4 related disease is more common in men >50 years of age. It is an inflammatory and fibrotic systemic conditions where organs form tumefactive lesions rich in IgG4 plasma cells. We are conveniently located to provide high quality care to everyone in our community. Risk factors: Advanced age, pre-existing joint dz, recent surgery or injection, SSTI, IVD, indwelling catheter, immunosuppression. We know that vascular inflammation is cased by activated neutrophils and monocytes because of ANCAs. Fibromuscular dysplasia (look for string of beads on imaging! Surgical excision for NBTE vegetation, can be considered in only selective cases and generally avoided. The knee accounts for 50% of all acute CPPD flares. Patients with the latter antibody are more likely to develop scleroderma renal crisis. These ANCAs bind to a variety of antigens, most notably proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA). 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. Exclusion: Demonstration of vegetations on echo in absence of systemic infection in patients with risk factors. Schedule your visit by dialing our Valley Connection call center, or by signing in to your MyHealth Online account. Giacomelli, R. et al. Dr. Sharp works in San Jose, CA and 1 other location and specializes in Rheumatology. Raynaud’s phenomenon can be primary (idiopathic) or secondary to autoimmune conditions, hematologic disorders, drugs/toxins, environmental factors, or hypothyroidism. PR3 is tightly linked to C-ANCA and MPO to P-ANCA. Santa Clara Valley Medical Center Internal Medicine Residency. CT revealed e/o fibrosis and GGO, and BAL revealed significant lymphocytosis. Treatment of MPA/GPA (eGPA tx is slightly different), Source: National Kidney Foundation’s Primer on Kidney Diseases 7th ed, Gilbert & Weiner, 2018. Dotted line represents the diaphragm: Usually mono-microbial, and Staph aureus is the most common cause of septic arthritis in adults. Triggers: Trauma, surgery, severe medical illness. 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!) Thanks to Eric and Naina for presenting the fascinating case of an elderly man who presented to the ER with acute, progressive shoulder and neck pain/stiffness that started after a visit to the dentist, found to have Crowned Dens Syndrome (???!!!) Kernig and Brudzinski, when present, are highly specific. Need tissue biopsy for diagnosis. Joint drainage, severe infectious may require repeated aspiration or even wash out. Cultures were sent but no additional fluid studies were obtained. Higher prevalence in people with fibromyalgia and migraines though unclear linkage. For now, both systems of identification are useful (a serotype and CPC diagnosis) and in the future, I wonder if treatment will differ between the two. In relation to our traditional clinicopathological (CPC) diagnoses of MPA, GPA and eGPA, we note the following: Key takeaways are that not all “MPA, GPA and EGPA” is ANCA+, and when it is, the pattern of antigen-antibody complex is not specific! Hydralazine-induced ANCA associated vasculitis! If you like mnemonics, think Drug-REGIIME for the various categories. Some diagnostic criteria have been established to help (see below) and imaging tends to be the most helpful. The disease is often recurrent. Alcohol abuse(45%) as well as cigarette use, Systemic diseases (eg: SLE, hyperparathyroidism, hypertriglyceridemia), Epigastric abdominal pain most common symptom however, Remember that chronic pancreatitis puts you at increased risk for, Amylase/Lipase usually NORMAL so not as helpful, 72 hour quantitative fecal fat (steatorrhea alone is non-specific!). The pathophys is poorly understood but thought to be a combination of autoimmune and allergic mediated processes. Associated primarily with tobacco products but cannabis arteritis has also been reported and is clinically indistinguishable. Avoid steroids in patients with scleroderma because they can precipitate renal crisis! Morning Report . Amputation free survival was 85, 74, and 66% at 5, 10, and 15 years. September 23, 2019 vmcimchiefs Leave a comment. A form of non-infectious endocarditis characterized by deposition of thrombi on halve valves, most commonly mitral or aortic, May present with acute stroke or coronary ischemia. Search. We first went over the framework for true muscle weakness, which can be anatomically divided as follows, Source: Frameworks for Internal Medicine (Dr. Andre Mansoor from OHSU). Thanks to John for presenting the case of a middle aged man from Vietnam with history of smoking who presented to the hospital with painless jaundice with imaging concerning for malignancy, found to have IgG4 related autoimmune pancreatitis! Primary Menu Skip to content. Official Valley IM Residency Site; Search for: Tag Archives: Rheumatology. San Jose, CA 95128, Phone: (408) 885-5000 This was confirmed by a highly positive anti-HMGCR antibody, Adapted from a NEJM Article: Biologic agents for refractory cases (IL1 antagonist anakinra or canakinumab), IL6 antagonist tocilizumab, or TNF inhibitors. However, a significantly elevated level is highly sensitive/specific (>95%) for IgG4 related disease. The mainstay of treatment is immunosuppression with steroids (typically 40-60mg prednisone daily for average weight adult). Inflammatory and Fibrotic systemic condition where organs have tumefactive (tumor forming) lesions with an infiltrate rich in IgG4 plasma cells and often elevated IgG4 serum levels (but not always!). For now, regardless of the serotype of CPC diagnosis of MPA or GPA, the treatment is the same! 751 S. Bascom AveSan Jose, CA 95128Get Directions, Phone: (408) 885-5000TTY Phone: (408) 971-4068. Direct inoculation: usually due to trauma, surgery/injections, or wounds. New diagnosis in patients >60 have been reported. > 5000 eos ⇒ severe eosinophilia at risk of end organ damage, Monoclonal leukemias (eosinophil proliferation), Polyclonal: T cell lymphomas, Hodgkin lymphoma, some solid organ tumors (cervical, ovarian, gastric, colon, urotherlial, and squamous cell carcinomas), Parasites: remember that only multicellular parasites can cause eosiniphilia, M:F is 1:1 but hydralazine induced lupus is more common in women, Mechanism is poorly understood and genetic predisposition may play a role. 751 S Bascom Ave Bimodal age distribution with peak between 15-25 and another 36-46 years of age. Rheumatology Schedule ===== A Quick Summary of Key Rheumatic Diseases Acute Arthritis Adult Onset Stills Disease Antiphospholipid Antibody Syndrome Fibromyalgia Gout and CPPD MCTD NSAIDs Osteoarthritis Osteoporosis Polymyalgia Rheumatica and Temporal Arteritis Polymyositis and Dermatomyositis Raynaud Phenomenon Rheumatoid arthritis Scleroderma Sjogrens SLE … The causes can be divided as follows. Knee is affected in over 50% of all acute attacks followed by wrists, shoulders, ankles, feet, and elbows. Could perform digit plethysmography or toe pressures to confirm. This is a rare case of culture negative endocarditis which is later thought to be more likely Libman Sacs! Bronchiectasis is a chronic condition where the walls of the bronchi are thickened from inflammation and infection. GCA primarily affects older patients (>50 years). 30% of those with autoimmune pancreatitis also have tubulointerstitial nephritis at presentation. Treatment involves immunosuppression with steroids and cytoxan or rituxan. Santa Clara Valley Medical Center Internal Medicine Residency, 6. Imaging of the arterial tree by MRA or CTA is important to help with diagnosis, evaluate the arterial lumen, mintor disease course, and decide on need for surgical intervention. Enter your email address to follow this blog and receive notifications of new posts by email. The exam maneuvers we use to determine nuchal rigidity (neck stiffness, Kernig, Brudzinski signs) are not sensitive for meningitis. In fact, ANCA positivity without clinical vasculitis is common especially in cases involving PTU. zoonotic microbes, fungal), Farm animal exposure: Brucella, Coxiella (Q-fever), Ingestion of unpasteurized milk: Brucella, Coxiella, HACEK: Most common agents of culture negative endocarditis. Classification criteria have been developed for Takayasu as a means of categorizing patients for research studies. Once we narrowed the differential to an inflammatory myopathy, we utilized the following chart that guided us to the probable conclusion that it was an immune-mediated necrotizing myopathy (also known as necrotizing autoimmune myopathy). The Chief Residents' Blog. Visit the Library Negative cultures can result due to recent abx or atypical organism. Enter your email address to follow this blog and receive notifications of new posts by email. PCR, histology, special cultures are helpful.