I think one of the nice things about starting with atypical arthritides is that when I reviewed the questions, I noticed that they're what I call first-order type questions. And so once you read the question stem, it'll be pretty apparent to you what the answer is, I hope, after having listened to this talk and prepared some questions. But in the scale of taxonomy, typically first-order type questions, which is nice. Okay. Okay, so we're basically going to look at eight conditions. Very briefly, psoriatic arthritis, lupus, scleroderma, gout, pseudogout, and what we call some of the zebras. Calcific periarthritis, sarcoidosis, and Lyme disease. As the name implies, psoriatic arthritis is an arthropathy that afflicts individuals who have the skin condition known as psoriasis. Very briefly, it's a condition in which there's an alteration in the skin cell cycle. And so there's an order of ten times the amount of skin cell turnover. And basically, as it builds up on the epidermis, it forms these thick, silvery plaques. They can be red, indurated, and very painful. Typically, we see them on the extensor surfaces, the elbows, the knees, the scalp also, sometimes on the torso. What's interesting is that in the majority of cases, most individuals who have psoriatic arthritis have precursor skin lesions. So if a patient comes in and you see these characteristic lesions, and they have some of the joint manifestations that we'll talk about, that's the norm. However, a minority of patients will present to your office with arthralgias and have not experienced skin lesions at that time. Classically, there's dactylitis, what we call sausage fingers, nail pits, PIP flexion contractures. And when I highlight in red, it's typically for a reason, as you've become accustomed to this morning. I've seen test questions, and I've tagged them that in this disease process, the NP joints can be stiff and hyperextended. Arthritis mutilans with severe digital shortening occurs in a subset of patients. And as is the case in many of these disorders, there are both medical and surgical treatment options. There are some classic radiographic findings, including periostitis, or basically inflammation of the connective tissue, periosteum around the small joints. And typically what you can see is what's termed a pencil and cup deformity at the DIP joints. And you can get this acro osteolysis, resorption of the bone, particularly at the base of the distal phalanx, culminating in this pencil and cup deformity. Very classic finding. Medical management, as you all know, there are non-steroidals. I didn't list it here, but steroids, obviously. DMARDs, disease-modifying anti-rheumatic drugs. And the term anti-rheumatic implies not just for rheumatoid disease, but for many other autoimmune inflammatory arthropathies. So you'll see DMARDs in the treatment of IBS, I'm sorry, IBD, ulcerative colitis, UC, Hashimoto's, ITP. So these DMARDs sort of span a broad spectrum of autoimmune inflammatory arthropathies. The three most classic are methotrexate, sulfasalazine, and I don't have it up here, but the hydroxychloroquine. Oftentimes they're used in triple combination. The newer generation, from the late 1980s to the current time, the biologic DMARDs, they include things like Humira, Enbrel, Remicade. They're basically biologic alterations, and they have a more robust response at specific cycles in the immune system. But basically, non-steroidal steroids, DMARDs, and tumor necrosis factor blocking agents. Surgical treatment for psoriatic arthritis. You're going to learn a lot of great principles from Dr. Benson in the rheumatoid arthritis talk. But essentially, fusion and arthroplasty. There can be issues with bone loss, as you may recall from the pencil and cup deformity. So sometimes they may require supplemental bone graft or fusions. And I alluded earlier to the hyperextension of the MP joints. They often have less motion overall with arthroplasty compared to its sister inflammatory arthropathies as a result of MP hyperextension. Moving on to lupus. This is a, in some cases, a highly debilitating process that affects women on a 9 to 1 scale. It's an autoimmune disorder that affects young women in particular. ANA positive findings with serologic testing. Oftentimes, individuals present with symmetric hand swelling and characteristic malar rash, pleuritis, and other systemic conditions. From a musculoskeletal perspective, what's interesting is that the joint spaces are often preserved. And what we see is subluxation, laxity of joints. So if you'll notice in the radiograph to the right, pristine appearing joints, but subluxation of the digits. Initial treatment is often affected with splinting. You'll see silver ring splints. In this particular case, swan neck deformity and lupus is highly characteristic. So swan neck and lupus. For debilitating postures, limited or total fusions are recommended. And sometimes you'll see thumb IP subluxation, MP instability. Again, you want to consider fusion surgery. Moving on to scleroderma. Two broad types, limited cutaneous and then the diffused cutaneous systemic. The limited cutaneous, as you may expect, typically affects the skin and joints. Diffused cutaneous can affect internal organ systems, the kidneys, the pleura. And this is a highly debilitating pathology for individuals who have the diffused form. Also positive ANA. And you'll forgive me, but if you want to jot down, because I think it's important. The other serologic testing is anti-SCL70. That's an antibody, an autoantibody that often is used in diagnostics. SCL70, the antibody for that. In the limited cutaneous, classically it's the Crest syndrome, where you see calcinosis many times at the vulva pulp that can become painful. Raynaud's phenomena, esophageal dysfunction, sclerodactyly, thin, shiny, taut skin, and telangiectasis, those little dilated blood vessels. In both scleroderma and lupus, there is an association with Raynaud's. And you'll see that in test questions. It's the phenomena because it's associated with a distinct disease process. Just as a matter of terminology, the disease is when there is an unknown primary disorder. But oftentimes, there's symmetric vasospasm associated in the digits with scleroderma in the limited variety, as well as the diffuse, as well as lupus patients. Again, you can see progressive joint contractures, skin ulceration as a result of the Raynaud's, stiff swan neck deformities. And there are oftentimes issues with surgical intervention as a result of the compromised circulation. Systemic sclerosis is more commonly treated with surgery. It's indicative of a more advanced disease process. And as mentioned, as a result of the vasospasm, oftentimes, sympathectomies or arterial reconstruction is necessary. And, of course, correction of joint contractures and excision of those calcifications that we saw. Moving on to gout. This is, in many ways, the great mimicker. I'm sure in your office, people are coming, are referred to you for what's suspected to be a septic joint. And it ends up being gout a great portion of the time. Basically, it's the deposition of crystals, monoarticular inflammation. They often deposit at the DIP joint, perhaps the radiocarpal joint. As I mentioned, it can mimic septic arthritis. Classic radiographic presentation are these erosions. You can see them in the carpus, oftentimes inundated throughout the carpus. Periarticular erosions, in this case, at the DIP joint, and these punched-out lytic lesions. And the first thing I would do if this individual presented at my office, as you probably would, would be get that ring off. But oftentimes, it looks like a hot, shiny, swollen digit. The pathology exists because the crystals deposit in and around the joints. Oftentimes, they can create this thick, cream-cheese-type consistency, known as tephageous gout. It has a predilection for abnormal joints, as opposed to normal joints. So as we age, and there's the typical degenerative process occurs, you'll find that gout tends to attack those areas. So it's got a predilection for diseased articular cartilage. A few words on the pathophysiology. And I don't think you have to know this, but you may recall from your biochemistry days, purine metabolism leads to a buildup of uric acid. When it's in the bloodstream, we call it hyperuricemia. When we find in the urine, hyperuricosauria. It usually requires a long time. It doesn't happen while the flare may be acute. The process has evolved over a period of months and years. The presence of hyperuricemia in and of itself does not necessarily result in gout. It's the deposition, the precipitation of the uric crystals within the joint that results in significant pain and discomfort. This is a very important slide. From a diagnostic standpoint, aspiration of the joint in question is most diagnostic, has the greatest sensitivity and specificity. And without getting into the principles of refraction and light microscopy, what we typically see is negatively bifringent needle-shaped crystals in gout, as opposed to the positively bifringent rhomboids that we'll talk about in a moment with pseudogout. And again, the definitive diagnosis is a joint aspiration and microscopy that reveals this negative bifringence with crystals. Some of the classic photos showing subcutaneous tophi that can become very painful and can erupt from the skin surface. Also, in the wrist and carpus, here is a coronal MRI T2 image, and you can see these little punched out lytic lesions throughout the carpus. But as far as the intrinsic wrist ligaments, gout has a predilection for the scapho-lunate and lunotrichoetral ligaments. They can lead to rupture over time. And so the progression of a slack wrist in the absence of deformity, one of the things one ought to consider, is gout. Here's an arthroscopic photo. This is the mid-carpal joint looking down at the scapho-lunate interval. And basically what you see is alterations in the articular cartilage, what appears to be depositions of material consistent with gout. In addition, it can result in tendon pathology. Sometimes patients present with painless mobile masses and tendon rupture. And here is an extensile approach to the wrist that reveals thick, inflamed tissue, and on the bottom you'll see a ruptured digital flexor tendon. Management includes the use of anti-inflammatories. Historically, indicin has been the anti-inflammatory of choice, but in review of studies, there's essentially no major difference between a standard NSAID and indicin. Colchicine has a mechanism of action that's a little bit different. It inhibits the production of lactic acid, which ultimately limits the amount of uric acid deposition. The other thing that colchicine does is it inhibits phagocytosis, thereby minimizing the inflammatory cascade. So I think first line, you want to think anti-inflammatories of your choice. Colchicine for its unique mechanism of action. And then for chronic management, we get into xanthine oxidase inhibitors. And basically, it prevents a pathway of the conversion of xanthine to uric acid. And those are medications that you're familiar with, allopurinol. I thought one of the most esoteric questions I saw is for patients who have renal pathology. The answer is uloric has a fairly good safety profile. I was kind of floored when I saw this question, but if you can put that in the memory bank. Uloric has a xanthine oxidase inhibitor for patients with renal issues. Uricasoric medication are basically those that allows the kidneys to excrete more uric acid, probenicid, et cetera. Another very important concept is dietary restrictions. Gout tends to flare with meat, alcohol, seafood, beer, the so-called rich man's diet. Surgical options are aspiration of the early liquid form and the bulking of tophi. The bulking is the key rather than complete excision. It's oftentimes intertwined around tendons and the neurovascular structures. Pseudogout, also present in large joints. Essentially a problem with metabolism resulting in deposition of calcium pyrophosphate. X-rays classically show calcifications within the TFCC, but also can afflict the intrinsic wrist ligaments, SL and LT. STT arthritis has also shown an association with pseudogout. And again, I would remark positively bifringent rhomboids for pseudogout. Okay, the zebras. Acute calcific periorthritis, often an acute flare of symptoms, pain and swelling. And radiographically what you'll see are calcifications adjacent to the affected joint. You see a very small sliver of calcifications, often misdiagnosed as an infection, gout or pseudogout. And the treatment essentially is watchful waiting and anti-inflammatories. Sarcoidosis. This is a systemic granulomatous disease. Basically, the body tries to wall off bacteria, viral or other tissues, creating granulomas. This can also afflict many of the internal organs, particularly the kidneys and lungs. Ten times more frequent in women than men. Also much more common in African-American individuals. Classically, chest radiographs will show bilateral hyaluronymphadenopathy. Operative excision often leads to recurrence. And unfortunately, there is no cure. But steroids are the mainstay for treatment to keep the symptoms at bay. Lyme disease is a regional or geographic entity. It's a tick-borne disease, most commonly in the northeast and upper midwest and mountainous areas. It's caused by the spirochete Borrelia burgdorferi. Three stages. Again, not very critical to know. The early, mild, late. Early is when you see that classic bull's-eye lesion. The mid-disease is when you start to get migratory arthralgias. And finally, polyneuropathy in the late stage. Testing for Lyme is ELISA testing, western blot. And again, testing should be reserved for populations with highest pretest probability. And here's a classic example of that bull's-eye lesion in stage one. Stage two becomes a little bit more subacute. Arthralgias in three. Chronic or late presentation. That can include polyneuropathy. Treatment or antibiotics. Doxyamoxicillin. Again, this is probably under the auspices of our primary care friends. But just to have an awareness that antibiotics are appropriate. So basically, I think for these test questions, if you know the characteristics of each of those disorders we went through, think the question through carefully. Because sometimes there are distractors and they'll get you. But these, for the most part, I would consider lay-up type questions. And the identifiers are typically given. And you don't have to go hunt for them. Just take your time. And I seem to be delving into my last talk of the day, test-taking strategies. I have to reserve my impulse to talk about that. But as with most of the questions on this exam, I would be cautious. And for these questions in particular, they're pretty apparent. I have a few minutes, so we'll do some of them if that's okay. So which of the following consumables is linked with affecting the onset of an acute gout attack? Again, it's meat, beer, seafood. Chocolate does not. Dairy products have actually been shown to limit the amount of uric acid. So the answer is beer. This is a question that I consider not a first-order question. It's a second-order question. Here's why. After resection of the tissue shown, the specimen should be sent to the lab in which of the following solutions? So this requires you to know that this is a gouty TOFI, step one. Step two, you have to know how to transport it to the lab. And so the answer is ethanol. Formalin and water-based preservatives will result in degrezation of the crystals. And so I think of all the questions that I have posed for you, there's maybe 20 or 25. This is really the only one that's a second-order question. Okay. This is a 45-year-old woman with slowly increasing ring finger pain and swelling. Physical examination is notable for diffuse digital swelling, decreased mobility, and nail pits. And you see this classic radiographic presentation, and you can jump right at psoriatic arthritis. Which of the following risk conditions is gout most associated with? I'll let you take it in for a moment. But when I first saw it, I wasn't thinking gout. I was thinking pseudo-gout. And I went right to TFCC. But again, if you take your time and think about it, this is gout, and we know that it afflicts the scapulonate ligament. When examining arthrosynthesis fluid under a polarizing microscope, monosodium urate crystals typically have what shape? Right away, we know we're talking about gout, and then we know we're talking about needle. They can flip this question and talk about CPPD, and you know that it's rhomboid. Okay. This will be the last question. This is a 19-year-old male with no known comorbidities, five-day history of increasing small finger pain, began spontaneously, no trauma. He denies fever, no constitutional symptoms. Exam reveals swelling of the digit, which is held in a slightly flexed posture, focal tenderness and swelling over the flexor sheath of the PIP joint, marked pain with passive extension. And I believe it's very hard to see. However, there is a sliver at the PIP joint, a very subtle hint of a calcification. And so the answer would be non-steroidals. If you were inclined to go with an infectious or a suppurative tenosynovitis, you might be thinking oral antibiotics. But I think the stem and the radiographs together allow this to be viewed as an acute periarticular calcification. Thank you for your time. Good luck.

psoriatic arthritis

lupus

scleroderma

gout

pseudogout

calcific periarthritis