1. Joints, Rheumatology, and the Shelf Steve Telloni prepared by Ryan Sanford Chief Lecture

2. The Joints 44F mother of four children ages 3-8y is evaluated for 2wk of aching in joints of wrists, hands, and knees. Pain and swelling were severe for ~ 1 week, then subsided to aching. Pain is worse in the morning and abates somewhat with activity. On physical exam there is tenderness with pressure on the dorsa of the wrists and pain with wrist motion. One side of the patient’s face shows faint redness. She has noticed patchy sloughing of the epidermis of her hands. What is the diagnosis? What is the differential diagnosis for acute arthritis?

3. Joint Pain Duration Acute Chronic 1.Infection [septic arthritis] 2.Trauma/Blood 3.Crystals! Gout and CPPD 4.Reactive 5.Parvovirus B19 6.Early Chronic Inflammation? No = OA Activity doesn’t help # Joints Involved Poly 1.RA = symmetric 2.SLE = symmetric 3.Systemic Sclerosis = symmetric Oligo 1.Spondyloarthropathy 2.Indolent infection 3.Early poly Mono 1.Indolent infection 2.Early oligo/poly Activity helps, stiff in AM [>1h]!

4. Finding?

5. Hand Pains 82F w/ chronic non-inflammatory hand pain and nodules at DIP joint -- Disease and Eponym? – OA and Heberden’s Nodes Pencil in cup Deformity on Hand X-Ray? – Psoriatic Arthritis, occurs at DIP, is erosive Ulnar Deviation? – Rheumatoid Arthritis Dactylitis? – Reactive Arthritis, Sickle Cell Anemia, Psoriasis, Ankylosing Spondylitis, Tb + anti cyclic citrullinated peptide? – RA Nodules filled with urate over fingers? – Gout MCP pain and a discoid rash? – SLE

7. Radiographic Findings and Diagnosis?

9. Osteoarthritis On Radiographs – Joint Space Narrowing – Subchondral Cysts – Osteophyte Formation – Subchondral Sclerosis The Patient Says – Not too stiff upon awakening [<30 min] – Pain gets worse with activity – Can have some effusions, esp at knees Tx: – OTC analgesia – APAP, NSAIDS. No Narcotics – Intra-articular injections – PT and periarticular muscle strengthening – Joint replacement

12. ... And I have pain with deep breaths?

13. Diagnostic Criteria for SLE Skin – Malar Rash – Discoid Rash – Photosensitivity – Oral/Nasal Ulcers MSK – Non-erosive arthritis Serologies – ANA – Anti dsDNA, anti-smith, APLA Cardiopulmonary – Serositis Renal – Proteinuria or cellular casts CNS – Seizures, psychosis, etc Heme – Hemolytic anemia OR – Leukopenia OR – Lymphopenia OR – thrombocytopenia But ALSO: constitutional complaints, abd pain, alopecia, vasculitis, raynaud’s, eye problems, etc. 1 7 6 8 9 10 11 2 3 4 5

14. Autoantibodies Most specific for SLE – Anti Smith Ab Prognostic for SLE and kidney disease – Anti ds DNA Ab APLA – bleeding or clotting? – Clotting, veins AND arteries ANCA? – Wegener’s granulomatosis (GPA), Microscopic polyangiitis, Churg-Strauss syndrome Wegener’s: c-ANCA, anti-PR3 Microscopic Polyangiitis: p-ANCA, anti-MPO Hematuria and Hemopytisis, not ANCA related – Goodpasture’s, anti-GBM Ab disease – Could also be SLE Taking hydralazine, now have arthritis and malar rash? – Anti-Histone Ab for drug induced Lupus – Also seen with procainamide, minocycline, isoniazid, quinidine Anti-Mitochondrial Ab – Primary Biliary Cirrhosis Anti-Endomysial Ab and Tissue Transglutaminase Ab – Celiac disease Autoimmune Hepatitis – Anti Smooth Muscle Ab, Anti Liver Kidney Microsomal Ab

15. Autoantibodies + Pearls Limited Scleroderma – Ab and Symptoms? – Anti-Centromere Ab – CREST [calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasias] Diffuse Scleroderma -- Ab – Anti SCL-70 Autoimmune cause of oral and genital ulcers? – Behcet’s Syndrome Young Asian female with loss of radial pulses, constitutional symptoms? – Takayasu’s Arteritis 85F with amaurosis fugax, headaches, scalp tenderness on same side, Dx? Tx? Work up? – Temporal Arteritis AKA Giant Cell Arteritis – ESR very high – Treat with high dose steroids – IMMEDIATELY; to prevent blindness – Get a temporal artery biopsy I have IBD and now an elevated bilirubin and alkaline phosphatase? – Primary sclerosing cholangitis

16. Takayasu’s Arteritis

17. I had a URI, now I have...

19. I got a URI, now I have a rash and bloody urine.. Henoch-Schonlein purpura IgA Nephropathy [synpharyngitic] Post Streptococcal GN occurs after the pharyngitis

20. 29 AA Fw/ 2mo of arthralgias of knees, elbows, hands, and swelling in legs. BP 150/95. HR 79. 2+ pitting LEE. HCT 35%; C3 60; C4 12; ANA positive; 24 Urine protein 4.6g. Urine sediment with erythrocyte casts, oval fat bodies. DDx? Likely Dx? Work-up?

21. Nephrotic Syndrome >3.5g of protein in 24h U collection Can present with either nephrosis or nephritis Causes of this Syndrome – Diabetic Nephropathy – Minimal Change Disease – think young, Kids!; heme CA – Membranous Nephropathy – HBV, solid tumors, class V SLE nephritis, NSAIDS – FSGS [obesity, HIV, idiopathic, heroin] – Myeloma – Amyloidosis Urine Sediment: oval fat bodies or benign General Tx: ACEI, diurese, treat underlying illness

22. Oval Fat Bodies

24. 66F with severe pain in L calf, sudden onset. Has RA of many joints. Has had many knee injections because of pain and effusions with triamcinolone. Now is treated with etanercept and methtotrexate. Physical exam with large R knee effusion and L knee is smaller in size. The knee was similar in size to the R until the pain began. The L calf is 5cm larger in diameter than the R. Diagnosis? – Ruptured popliteal (Baker’s) cyst

25. RA Chronic, symmetric, inflammatory, destructive Joints – PIPs, MCPs, wrists, knees, ankles, MTPs C1-C2 instability – A Classic Question S/Sx: – Constitutional: fever, weight loss, malaise – Pulm: ILD, nodules, fibrosis, pleuritis +/- effusions – Vascular: leukocytoclastic vasculitis – Cardiac: pericarditis, myocarditis

27. Seldom Seen

29. Nodules

30. Diagnostic Criteria for RA? 4 out of 7 Morning Stiffness >1h Hand Joint Arthritis >6wk Rheumatoid Nodules X-ray changes – erosions or periarticular osteopenia Arthritis of >3 joints simultaneously >6wk Symmetric involvement >6wk +RF [but check the CCP]

31. Diagnosis?

33. Gout: Negatively Birefringent Needle Shaped Crystals

34. Pseudogout = Calcium Pyrophosphate Deposition Disease Weakly Positive Birefringent Rhomboid Shaped Crystals

35. What Is This?

36. Gout SHELF: obese, drinking, male, middle aged, carnivorous Acute Monoarticular Arthritis – 1 st MTP = Podagra – Overlying skin, dusky, red, tense, red – Also at feet, ankles, knees Don’t check serum uric acid during a flare! The joint fluid: lots of WBCs [20-100k]; majority are PMNs. Find the crystals! Get a Gram Stain! Tx – Acute: NSAIDS, colchicine, maybe steroids – Chronic: decrease purine intake, daily colchicine Allopurinol or probenecid not until acute issues resolved; tx w/ colchicine or nsaids concominantly while reducing UA levels

39. Calcification of cartilage as seen on X-ray? Chondrocalcinosis of CPPD or Pseudogout

40. 26F w/ multiple sexual partners Migratory polyathralgias True inflammation  tenosynovitis Synovial fluid  50K WBC, mainly PMNs Blood Cultures growing GN diplococci Cause? Treatment?

41. Disseminated Gonococcal Infections Most common infectious arthritis of sexually active young adults Preceded by mucosal infection – can be ASx – Cervicitis – Urethritis – Pharyngitis Migratory Polyarthralgias Tx with ceftriaxone x7d, must also treat for Chlamydia – azithromycin or doxycycline

42. “Doc, since I was 20 I’ve had low back pain, especially in the morning...” SI JOINT Picture 1

43. And his spine films... PICTURE 2

44. What does Seronegative Spondyloarthropathy Mean? Absence of rheumatoid factor, autoantibodies Inflammatory! Aseptic. ESR elevated Has a tendency to affect spine, SI joint, but also other joints Also can affect eyes [uveitis, scleritis, iritis, conjunctivitis] Associated with HLA-B27 Think of 4 illnesses 1.Ankylosing spondylitis 2.Psoriatic arthritis 3.Enteropathic artritis 4.Reactive arthritis

45. Ankylosing Spondylitis Classically: starts in late teens, early 20s; gradual onset low back pain, worse in AM [inflammatory!], improves with movement/exercise Progressive involvement of spine, starting at SI Joint [picture 1]  erosions and sclerosis Also inflammation at insertion sites for tendons/ligaments  enthesitis – Achillies pain – Plantar Fasciitis – Spine  Bamboo Spine [picture 2] – spinal ligament calcification and bridging syndesmophytes Also could see uveitis

47. PIP pains and scaly papules on forearm?

48. Psoriatic Arthritis Can have various presentations... – Monoarticular/dactylitis – Esp DIP – Polyarthritis – Axial involvement – like AS Arthritis can preceded skin findings by years Enthesitis Pitting fingernails Joint Films – ‘Pencil in Cup’ deformity at DIPs

50. And the 2 Other Seronegative Spondyloarthropathies Reactive Arthritis Follows GU or GI infection The Triad – Seronegative arthritis – Urethritis – Conjunctivitis Males > Females Enteropathic IBD Associated Can look just like AS Also can see – Erythema nodusum – Pyoderma gangrenosum

52. Erythema Nodosum

53. Pyoderma Gangrenosum