Joints, Rheumatology, and the Shelf Paul Johnson prepared by Ryan Sanford Chief Lecture 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 PE 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 DDx for acute arthritis? Joint Pain Duration Chronic Acute 1. 2. 3. 4. 5. 6. Infection [septic arthritis] Trauma/Blood Crystals! Gout and CPPD Reactive Parvovirus B19 Early Chronic Inflammation? Activity helps, stiff in AM [>1h]! Activity doesn’t help No = OA # Joints Involved Mono 1. 2. Indolent infection Early oligo/poly Oligo 1. 2. 3. Spondyloarthropathy Indolent infection Early poly Poly 1. 2. 3. RA = symmetric SLE = symmetric Systemic Sclerosis = symmetric Finding? 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? AKA? – Reactive Arthritis, Sickle Cell Anemia, Psoriasis, Akylosising Spondylitis, Tb • + anti cyclic citrullinated peptide? – RA • Nodules filled with urate over fingers? – Gout • MCP pain and a discoid rash? – SLE Radiographic Findings and Dx? 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 . . . And I have pain with deep breaths? Diagnostic Criteria for SLE • Skin – – – – Malar Rash 1 Discoid Rash 2 Photosensitivity 3 Oral/Nasal Ulcers 4 • MSK – Non-erosive arthritis 5 • Serologies – ANA 6 – Anti dsDNA, anti-smith, 7 APLA • Cardiopulmonary – Serositis 8 • Renal 9 – Proteinuria or cellular casts • CNS 10 – Seizures, psychosis, etc • Heme 11 – – – – Hemolytic anemia OR Leukopenia OR Lymphopenia OR thrombocytopenia But ALSO: constitutional complaints, abd pain, alopecia, vasculitis, raynaud’s, eye problems, etc. Autoantibodies • Most specific for SLE – • Prognositic for SLE and kidney disease – • Anti ds DNA Ab APLA – bleeding or clotting? – • Anti Smith Ab Clotting, veins AND arteries ANCA? – Wegener’s granulomatosis, Microscopic polyangiitis, Churg-Strauss syndrome • • • Hematuria and Hemopytisis, not ANCA related – – • Primary Biliary Cirrhosis Anti-Endomysial Ab and Tissue Transglutaminase Ab – • Anti-Histone Ab for drug induced Lupus Anti-Mitochondrial Ab – • Goodpasture’s, anti-GBM Ab disease Could also be SLE Taking hydralazine, now have arthritis and malar rash? – • Wegener’s: c-ANCA, anti-PR3 Microscopic Polyangiitis: p-ANCA, anti-MPO Celiac disease Autoimmune Hepatitis – Anti Smooth Muscle Ab Wegener’s Granulomatosis Autoantibodies + Pearls • Limted 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 Takayasu’s Arteritis I had a URI, now I have . . . I got a URI, now I have a rash and bloody urine . . • Henonch Shonlein purpurua • IgA Nephropathy [synpharyngitic] • Post Streptococcal GN occurs after the pharyngitis • 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? 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 Oval Fat Bodies • 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. PE 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? 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 Seldom Seen Nodules Diagnostic Criteria for RA? 4 out of 7 • • • • AM 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] Diagnosis? Diagnosis? Gout: Negatively Birefringent Needle Shaped Crystals Pseudogout = Calcium Pyrophosphate Deposition Disease Weakly Positive Birefringent Rhomboid Shaped Crystals What Is This? Gout • SHELF: obese, drinking, male, middle aged, carnivorous • Acute Monoarticular Arthritis – 1st MTP = Podagra – Overlying skin, dusky, red, tense, red – Also at feet, ankles, knees • Don’t check serum uric acid during a flair! • 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 Calcification of cartilage as seen on X-ray? Chondrocalcinosis of CPPD or Pseudogout 26F w/ multiple sexual partners • • • • Migratory polyathralgias True inflammation tenosynovitis Synovial fluid 50K WBC, mainly PMNs Blood Cultures growing GN diploocci Cause? Treatment? 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 “Doc, since I was 20 I’ve had low back pain, especially in the morning . . .” SI JOINT Picture 1 And his spine films . . . PICTURE 2 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, conjuntivitis] • Associated with HLA-B27 • Think of 4 illnesses 1. 2. 3. 4. Ankylosing spondylitis Psoriatic arthritis Enteropathic artritis Reactive arthritis 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 PIP pains and scaly papules on forearm? 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 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 Erythema Nodosum Pyoderma Gangrenosum