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Systemic Diseases with Rheumatic Manifestations

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Systemic Diseases with Rheumatic Manifestations 10/28/2013 Disclosures Rheumatic Manifestations of Endocrine Diseases • No financial disclosures • 1 relevant disclosure to this talk: Jonathan Graf, MD Associate Professor of Medicine, UCSF • I am a not only a rheumatologist, I am also an Division of Rheumatology, San Francisco General Hospital internist Overview Vignette #1 • Musculoskeletal symptoms are common in endocrine and metabolic disorders • 45 Year old male with – Some are easy to recognize while others can be progressive skin more subtle tightening of his – Can mimic those of actual rheumatic disease hands, no history of GERD or Raynaud’s • Recognition is important phenomenon, and – May be some of the earliest clues to underlying negative ANA. endocrine or metabolic disorder – Avoid pitfall of misdiagnosis Vignette #1 Vignette #1 • The most likely positive test result in this • The most likely positive test result in this patient would be: patient would be: • A. Rheumatoid Factor • A. Rheumatoid Factor • B. Anti Scl-70 • B. Anti Scl-70 • C. Anti-Centromere Antibody • C. Anti-Centromere Antibody • D. Elevated HgA1C • D. Elevated HgA1C 1 10/28/2013 A growing” epidemic of diabetes= more MSK A growing epidemic of diabetes complications Source: Data for 1960-1998 from the National Health Interview Survey, NCHS, CDC Projected data for 2000-2050 from the Behavioral Risk Factor Surveillance System, Division of Diabetes Translation, CDC Soft tissue complications of Diabetes: Proposed Effects of hyperglycemia Diabetes on the musculo-skeletal system • Limited Joint Mobility Syndrome (Cheiropathy – shown) • Stimulation of fibrous • DISH (type II DM) tissue proliferation • Adhesive Capsulitis • Neuropathic (Charcot) Arthropathy – DJD X 10, including unusual DJD in joints like ankle • Small vessel • Flexor tenosynovitis, tendon nodules (trigger vasculopathy and finger), and dupuytren’s stenosing tenosynovitis tissue ischemia • Carpal Tunnel Syndrome • Diabetic muscle infarction • Neuropathy: Direct • Osteoporosis (type I DM, weaker association) toxic effect (Really a bunch of hand waving!) Soft tissue complications of Limited Joint Mobility Syndrome: Diabetic Cheiropathy • Incidence correlates Diabetes – Disease duration (usually >10 yrs) – Suboptimal glycemic control • Limited Joint Mobility Syndrome (Cheiropathy – shown) • Most commonly involves the hands • DISH (type II DM) – Can also involve shoulders, knees, and feet • Adhesive Capsulitis • Etiology: Palmar fasciitis leads to • Neuropathic (Charcot) Arthropathy – DJD X 10, progressive thickening and including unusual DJD in joints like ankle tightening of skin • Flexor tenosynovitis, tendon nodules (trigger • Excellent mimic of scleroderma finger), and dupuytren’s stenosing tenosynovitis and sclerodactyly • Carpal Tunnel Syndrome • Can become quite disabling • Diabetic muscle infarction • Glycemic control and physical/ • Osteoporosis (type I DM, weaker association) occupational therapy may slow Progressively thickened, waxy, and shiny skin progression 2 10/28/2013 Prayer Sign Vignette #2 • The patient places his 48 YO male with a history of DMII presents or her hands together with a two year history of mild-moderate as if in prayer pain and stiffness in his mid and low back. He now notes 6 months of increasing neck • Patients with limited pain and dysphagia. mobility syndrome are unable to make complete contact between the palmar surfaces of their fingers Vignette #2 Vignette #2 Which of the following statements about this patient’s condition is LEAST correct? A. He likely has involvement of the thoracic spine B. TNF-a blocking therapy will improve his symptoms C. Type II diabetes is associated with this condition D. It is more commonly reported in males DISH: Diffuse idiopathic skeletal Vignette #2 hyperostosis Which of the following statements about this • Excess calcification along spinal ligaments and patient’s condition is LEAST correct? bone formation at insertion sites of tendons and ligaments A. He likely has involvement of the thoracic spine • Higher prevalence in pts with DMII (up to 25%) B. TNF-a blocking therapy will improve his but can be seen on its own symptoms C. Type II diabetes is associated with this condition • Most commonly affects mid-thoracic spine: D. It is more commonly reported in males Formal definition – Flowing ligamentous calcifications of at least 4 contiguous vertebrae – Minimal loss of disc space – Absence of sacroiliitis 3 10/28/2013 DISH Can you tell the difference? • Usually asymptomatic (curious predilection for right side of spine) • Osteophytes can rarely cause impingement – Dysphagia – Back pain • In rare instances, surgical removal necessary • Important to recognize that this is not ankylosing spondylitis Diffuse Idiopathic Skeletal Hyperostosis Ankylosing Spondylitis Soft tissue complications of Vignette #3 Diabetes • 52 year old patient with a history of • Limited Joint Mobility Syndrome (Cheiropathy – type II diabetes and HTN complains of progressively shown) increasing pain in what had been a • DISH (type II DM) normal left shoulder after falling. • Adhesive Capsulitis • Physical examination • Neuropathic (Charcot) Arthropathy – DJD X 10, demonstrates pain and near total including unusual DJD in joints like ankle loss of motion with abduction/adduction/external and • Flexor tenosynovitis, tendon nodules (trigger internal rotation of her L. shoulder. finger), and dupuytren’s stenosing tenosynovitis The R. is normal. • Carpal Tunnel Syndrome • Diabetic muscle infarction • Plain films are shown to the right • Osteoporosis (type I DM, weaker association) • MRI reveals no tearing of the rotator cuff Adhesive capsulitis: frozen shoulder Adhesive capsulitis: frozen shoulder • May affect up to 12% of patients with types I and • May/may not co-exist with rotator cuff pathology II diabetes cumulatively • May/may not be preceded by minor trauma of • Usually benign, self limited which patient is unaware • Pain and limited range of motion are common • Thought due to thickening and shrinkage of the and can rapidly progress shoulder joint capsule (not stress fractures or other pathology) • Most patients respond to physical therapy as first line: Other invasive procedures only for • Clinical diagnosis: plain films and MRI to rule out refractory cases other internal derangements 4 10/28/2013 Soft tissue complications of Vignette #4 Diabetes • Limited Joint Mobility Syndrome (Cheiropathy – • 51 year old male with shown) type II diabetes presents • DISH (type II DM) complaining of painless • Adhesive Capsulitis swelling in his right ankle • Neuropathic (Charcot) Arthropathy – DJD X 10, and foot including unusual DJD joints like ankle • Flexor tenosynovitis, tendon nodules (trigger • Exam shown to right: finger), and dupuytren’s stenosing tenosynovitis Afebrile with diffuse • Carpal Tunnel Syndrome swelling, mild tenderness, • Diabetic muscle infarction and mild erythema over • Osteoporosis (type I DM, weaker association) dorsum of foot extending to ankle Vignette #4 Charcot Arthropathy • Xray shown to right • Diabetes is the leading cause reveals extreme bony of neuropathic arthropathy destruction, fracture, • Often painless • Progressive sensory and osteolysis neuropathy (sometimes worrisome for subclinical) • Preferentially affects axons of osteomyelitis greatest length (stocking- glove) • Impairment of normal joint • Often can be difficult protection to distinguish from • Microtraumas • Microfractures infection, especially if • Exuberant “healing”osteolysis overlying ulcerations and hyperostosis Neuropathic (Charcot) Arthropathy Non-Surgical Treatment Options • Key signs: – DJD X10! (degenerative joint disease) – OA in a privileged joint (ankle) – Involvement of the metatarsal, tarsal, and talar joints most common – Can involve knees shoulders, etc… • Treatment: limiting weight bearing and orthotic protective devices – Total contact casting 3-6 months until stable – Orthotic ambulation devices – Possible surgical fixation and stabilization if medical therapy fails Total Contact Casting CROW: Charcot restraint orthotic walker 5 10/28/2013 Soft tissue complications of Soft tissue complications of Diabetes Diabetes • Limited Joint Mobility Syndrome (Cheiropathy – • Limited Joint Mobility Syndrome (Cheiropathy – shown) shown) • DISH (type II DM) • DISH (type II DM) • Adhesive Capsulitis • Adhesive Capsulitis • Neuropathic (Charcot) Arthropathy – DJD X 10, • Neuropathic (Charcot) Arthropathy – DJD X 10, including unusual DJD joints like ankle including unusual DJD joints like ankle • Flexor tenosynovitis, tendon nodules (trigger • Flexor tenosynovitis, tendon nodules (trigger finger), and dupuytren’s stenosing tenosynovitis finger), and dupuytren’s stenosing tenosynovitis • Carpal Tunnel Syndrome • Carpal Tunnel Syndrome • Diabetic muscle infarction • Diabetic muscle infarction • Osteoporosis (type I DM, weaker association) • Osteoporosis (type I DM, weaker association) Flexor Tendon Nodules and Flexor Tendon Nodules and Dupuytren’s Contractures Dupuytren’s Contractures • Flexor tenosynovitis reported to • Chronic tenosynovitis of flexor tendons can progress to flexor affect between 12-15% of all contractures Diabetic patients • 4th-5th digits most commonly involved • Also seen in alcoholic, HIV, epilepsy, previous trauma, “normals” • Local GC injection of little benefit • The condition is multifocal – Removing one lesion does not prevent others • Early in course, presents with isolated nodule(s) on flexor – Recurrence may occur after surgery tendons of hand • Surgery is indicated with MCP
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