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Home , Arthropathy

10/28/2013

Disclosures Rheumatic Manifestations of Endocrine • 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 , 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 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

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A growing” epidemic of = 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 Mobility Syndrome (Cheiropathy – shown) • Stimulation of fibrous • DISH (type II DM) tissue proliferation • Adhesive Capsulitis • Neuropathic (Charcot) – DJD X 10, including unusual DJD in like ankle • Small vessel • Flexor , 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 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

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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

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DISH Can you tell the difference?

• Usually asymptomatic (curious predilection for right side of spine)

• can rarely cause impingement – Dysphagia – Back pain

• In rare instances, surgical removal necessary

• Important to recognize that this is not 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 • • 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

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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 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 , 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

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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 (), 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 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 joint of 30° or more. Even • Symptoms develop from nodule long-standing and severe contractures of the MCP joint usually are impeding tendon motion through corrected readily at surgery and may or may not recur. (A1) pulley • PIP joint contractures do not have the same prognosis (high recurrence • Local glucocorticoid injections rate as well.) Proceed with surgery as soon as PIP joint contractures into tendon sheath of benefit are observed.

Clostridium Histolyticum Collagenase Vignette #5

• Contractures of MCP or 0 PIP>20 42 year old long-standing, poorly controlled • Injection of collagenase into cord diabetic presents complaining of the abrupt • Manipulation 1 day later onset of lateral thigh pain. On exam, a • 30 day follow up with repeat injection if palpable tender mass is noted over the lateral necessary (up to 3 times total) until 0-50 thigh. Temperature and CBC are WNL. CK • Side effects: levels are mildly elevated at 250, blood – Tendon rupture (especially 5th finger) cultures are negative. The symptoms are self- – Recurrence rate 6.7% limiting and gradually subside over several

weeks with conservative therapy. Hurst LC et al. N Engl J Med 2009;361:968-979.

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Vignette #5 Diabetic Muscle Infarction • Abrupt onset of pain in affected muscle MRI is shown to right:

• Frequently with localized swelling

• Most commonly affects thigh, less involvement of the calf, almost exclusively lower extremity And the diagnosis is….. • Clinical history and localized MRI findings (occasionally bx) help make diagnosis (vs. myopathy/myositis)

T1 weighted MRI with Fat Suppression • Managed conservatively with rest, analgesics, and of Adductor Magnus tighter metabolic control of diabetes (?anticoagulation)

Vignette #6 Vignette #6

• 42 year old male previously diagnosed with type II diabetes presents with increasingly bothersome tingling in his On exam, his hand is bilateral hands that has now progressed to shown to the right. wake him up at night. His medical history Tinel’s test reveals is also notable for chronic back pain. reproducible tingling in the first three fingers.

Vignette #6: Radiographs Vignette #6

Which of the following is the next step in managing this patient’s symptoms?

A. Surgical decompression of carpal tunnel B. Carpal Tunnel Splinting C. Order an MRI for the patient D. Corticosteroid injection carpal tunnel E. Order nerve conduction studies

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Soft tissue complications of Vignette #6 Diabetes Which of the following is the next step in • Limited Joint Mobility Syndrome (Cheiropathy – managing this patient’s symptoms? shown) • DISH (type II DM) • Adhesive Capsulitis A. Surgical decompression of carpal tunnel • Neuropathic (Charcot) Arthropathy – DJD X 10, B. Carpal Tunnel Splinting including unusual DJD joints like ankle • Flexor tenosynovitis, tendon nodules (trigger C. Order an MRI for the patient finger), and dupuytren’s stenosing tenosynovitis D. Corticosteroid injection carpal tunnel • Carpal Tunnel Syndrome E. Order nerve conduction studies • Diabetic muscle infarction • Osteoporosis (type I DM, weaker association)

Diabetes and Carpal Tunnel CTS: Clinical Features Syndrome (CTS) • Both DM and CTS are prevalent in western • Numbness or paresthesias in a distribution consistent society but seem to co-exist more than would be with that innervated by the median nerve expected by chance, alone • Symptoms are often exacerbated at night and may awaken a patient from sleep. Symptoms can progress from an irritating sensory neuropathy to weakness and • Up to 20% of DM patients develop CTS wasting of the intrinsic hand muscles – Up to 75% of those with limited joint mobility • Provocation of paresthesias in a median nerve • Some studies suggest it can precede formal distribution by either a Phalen maneuver or Tinel sign 1 can help confirm the diagnosis. diagnosis of DM by up to 10 years

1Gulliford MC, Latinovic R, Charlton J, et al.: Increased incidence of carpal tunnel syndrome up to 10 • Nerve conduction studies localize the site of the nerve years before diagnosis of diabetes. Diabetes Care 9:1929–1930, 2006. compression to the wrist and differentiate carpal tunnel syndrome from other types of neuropathy.

CTS: Treatment Vignette #6: Apology for sneaky question • Why order the MRI?? • Start with conservative measures – wrist splinting, particularly at night • This patient actually has – refraining from exacerbating activities acromegaly and secondarily associated hyperglycemia

• Local corticosteroid injection into the carpal • Diabetes & acromegaly, EACH, tunnel if symptoms persist or motor signs can predispose to carpal tunnel develop. syndrome – so this patient was – Sometimes repeat injections needed at extra risk!

• Surgical decompression when injections fail • MRI is to r/o pituitary adenoma

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Acromegaly Hypertrophy of the Acromegaly Distal Tufts • Spade-like bony overgowth seen in distal tufts • Soft tissue thickening • Carpal Tunnel Syndrome • Can also see early widening of joint spaces secondary to cartilage overgrowth • Premature can follow, aggressive osteophytosis, and CPPD

Acromegaly Vignette #7

• Musculo-skeletal complaints can be the initial presentation prompting referral to a • 42 year old female presents with rheumatologist complaints of chronic weight loss, tremors,

• Back pain common presenting and pain in her hands and feet. Physical symptom examination reveals the following:

• Early, aggressive degenerative disease (CPPD, haphazard matrix deposition and fissuring) Treatment with pituitary adenoma removal, irradiation, or bromocriptine • Joint pain (38-78%), axial and may reverse some of symptoms large joint arthropathy, soft (reported) tissue overgrowth, carpel tunnel syndrome, cartilage overgrowth, DISH, and CPPD.

Hyperthyroidism: Rheumatic Thyroid Acropachy manifestations • Associated with clubbing, • Myopathy periostitis, pretibial myxedema, and diffuse soft tissue swelling – Ranges from minor aches to profound, usually and pain. proximal muscle weakness with minimal CK • Long-acting TSI may persist – Benefit to restoration of euthyroid state even after successful thyroid ablation. • : most common in shoulders

• Transition to euthyroid state • Osteopenia and osteoporosis may be associated with myalgia and arthraligias • Thyroid acropachy – Some studies suggest treatment of thyroid disorders in patients with M-S symptoms only results in transient improvement

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Hypothyroidism: The great Vignette #8 rheumatic disease mimic • 42 year old female with known Hashimoto’s thyroiditis presents with pain • Joint pain and swelling RA/SLE in her hands and feet as well as marked swelling in her knees bilaterally. Her Chem 7 and CBC are WNL, although her • SICCA Sjogren’s ESR is 32 and ANA 1:1280. • Muscle weakness, CKs Myositis

• High titers of ANAs All Above

Hypothyroidism : Rheumatic manifestations Vignette #9 • Articular manifestations – Viscous oligoarticular noninflammatory joint effusions – Polyarthralgias and myalgias 60 year old female presents – Non-erosive inflammatory (auto-immune to your office complaining of thyroid disease) fatigue, joint, and muscle pains.

• Myopathy – More often proximal Plain films of her hands are – Can have significant elevations in CK shown to the right.

• Carpal tunnel syndrome • and CPPD • Avascular necrosis (including knees)

Hyperparathyroidism (Osteomalacia) Hyperparathyroidism Rugger-Jersey Spine • Cystic areas of demineralization called “brown tumors”

• Erosive disease can occur in DIP, MCP, carpal, and AC joints

• Oseteitis Fibrosa Cystica more common in secondary hyper- Small cyst PTH, but declining

• May also be associated with diffuse bone and joint pain, proximal myopathy, neuropathy and CPPD

Areas of subperiosteal erosion

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Hypoparathyroidism Vignette #10

• Usually with history of surgical damage or 47 year old Caucasian male presents to removal your office complaining of chronic hand • Muscle fatigue and weakness and wrist pain. He reports undergoing a • Tetany and neuromuscular irritability total knee replacement for 3 years earlier. CBC, ANA, and RF are WNL. Plain films of his hands reveal: • Both hyper and hypoparathyroidism cause osteopenia and increased risk of fx.

Hemochromatosis: Rheumatic Hemochromatosis manifestations • CPPD and/or apatite deposition • Degenerative arthropathy – Predilection for the MCP joints, knees, hips • Squaring of MCP heads with tear drop osteophytes Squaring of MCP Heads • Osteopenia • Cirrhosis • “Bronze Diabetes” (increased melanin) • Congestive heart failure

Hemochromatosis: Diagnosis Summary

• Measurement of serum iron and transferrin • Rheumatic manifestations may be early saturation clues to underlying endocrine or metabolic – >60% saturation suggests diagnosis disorder • HFE gene on chromosome 6 – Some manifestations are more obvious than – C282Y or H63D mutations seen in 90% Northern others and can present later in life Europeans with hemochromatosis – Rheumatologists are expected (at least on – 1/300 Caucasians homozygous for HFE, but disease some board questions) to have appropriate is less common suspicion for these diseases – Helps in counseling relatives – Avoid possibility of being fooled by mimics • Treatment: Plebotomy. ?Fe chelation into making a diagnosis of a rheumatic disease

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Summary of Mimics Early Manifestations of Endocrine Disorders that Can Mimic Rheumatic Diseases

Manifestation Endocrine Disorder Mimics Myalgias, arthralgias, fatigue Hyperthyroidism Fibrositis Hypothyroidism SLE

Hyperparathyroidism

Proximal muscle weakness Hyperthyroidism Polymyositis Hypothyroidism

Hyperparathyroidism

Acromegaly

Shoulder girdle pain and stiffness Hyperthyroidism Polymyalgia rheumatica Degenerative arthritis Acromegaly Osteoarthritis Hypothyroidism

Distal soft tissue swelling and periosteitis Graves disease (thyroid acropachy) Hypertrophic osteoarthropathy with ANA Hashimoto thyroiditis SLE, RA CPPD, pseudogout Hyperparathyroidism, hypothyroidism, acromegaly Idiopathic CPPD

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