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Prof.Mohamed Elwy Professor of Rheumatology and physical medicine Ain Shams University

Prof.Mohamed Elwy Professor of Rheumatology and physical medicine Ain Shams University. Prof.Mohamed Elwy AinShams U. 2015. Soft Tissue (Non-articular) Rheumatism. Prof.Mohamed Elwy AinShams U., 2015.

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Prof.Mohamed Elwy Professor of Rheumatology and physical medicine Ain Shams University

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  1. Prof.Mohamed Elwy Professor of Rheumatology and physical medicine Ain Shams University Prof.Mohamed Elwy AinShams U. 2015

  2. Soft Tissue (Non-articular) Rheumatism Prof.Mohamed Elwy AinShams U., 2015

  3. Soft-tissue rheumatic disorders causing pain outside joints may be classified as follows: 1. Generalized rheumatic pain due to systemic disorders. 2. Tendinitis and tenosynovitis. 3. Enthesopathies. 4. Bursitis. 5. Capsulitis. 6. Myositis. 7. Muscle cramps. 8. Fasciitis. 9. Fibromyalgia. 10. Panniculitis. 11. Neuritis. 12. Soft-tissue injury.

  4. The Normal Pain Processing Pathway Glutamate Substance P 4. The descending tract carries modulating impulses back to the dorsal horn • A signal is sent via the ascending tract to the brain, and perceived as pain Pain Perceived • Impulses from afferents depolarize dorsal horn neurons, then, extracellular Ca2+ diffuse into neurons causing the release of Pain Associated Neurotransmitters – Glutamate and Substance P • Stimulus sensed by the peripheral nerve (ie, skin) 1. Staud R and Rodriguez ME. Nat Clin Pract Rheumatol. 2006;2:90-98. 2. Gottschalk A and Smith DS. Am Fam Physician. 2001;63:1979-1984.

  5. Central Sensitization: A Theory for Pain Amplification in FM Central sensitization is believed to be an underlying cause of the amplified pain perception that results from dysfunction in the CNS1 May explain hallmark features of generalized heightened pain sensitivity2 Hyperalgesia – Amplified response to painful stimuli Allodynia - Pain resulting from normal stimuli Theory of central sensitization is supported by: Increased levels of pain neurotransmitters3,4 Glutamate Substance P fMRI data demonstrates low intensity stimuli in patients with FM comparable to high intensity stimuli in controls5 fMRI = functional magnetic resonance imaging 1. Staud R and Rodriguez ME. Nat Clin Pract Rheumatol. 2006;2:90-98. 2. Williams DA and Clauw DJ. J Pain. 2009;10(8):777-791. 3. Sarchielli P, et al. J Pain. 2007;8:737-745. 4. Vaerøy H, et al. Pain. 1988;32:21-26. 5. Gracely RH, et al. Arthritis Rheum. 2002;46:1333-1343.

  6. Central Sensitization Produces Abnormal Pain Signaling After nerve injury, increased input to the dorsal horn can induce central sensitization Perceived pain Nerve dysfunction Ascendinginput Descendingmodulation Nociceptive afferent fiber Induction of central sensitization Perceived pain(hyperalgesia/allodynia) Increased release of pain neurotransmitters glutamate and substance P Minimalstimuli Pain amplification Increased pain perception Increased pain perception 1. Adapted from Gottschalk A and Smith DS. Am Fam Physician. 2001;63:1979-1984. 2. Woolf CJ. Ann Intern Med. 2004;140:441-451.

  7. FM: An Amplified Pain Response 10 Pain in FM Normal pain response 8 Pain amplification response Hyperalgesia (when a pinprick causes an intense stabbing sensation) 6 Subjective pain intensity 4 Allodynia (hugs that feel painful) 2 0 Stimulus intensity Adapted from Gottschalk A and Smith DS. Am Fam Physician. 2001;63:1979-1986.

  8. 2.5% Why FM is important: Clauw, Daniel J. (16 April 2014). "Fibromyalgia". JAMA 311 (15): 1547. Very Common: *2-8% of the general population(Clauw and Daniel, 2014). *It is the 2nd most common Rhic. Dis. after OA. General population Ptn History 25% Misdiag.: *25% of patients referred to Rheuma.Clinics with +ve ANA had FMS. +ve ANA 25% Other speciality: In FM, anxiety/depression are present in ~25% of patients. FM

  9. ('Fibromyalgia Syndrome') (Fibrositis) ACR Rheumatol

  10. Patient presentation: Soft-tissue pain and tenderness + Sometimes with little palpable lumps Particularly in the neck and back regions (These are areas of pain but without redness, swelling nor hotness) Ptn History

  11. Clinical Feature: Exaggerated tenderness of normal tender points'. Tender point Exam: In normal subjects,  uncomfortable to firm pressure, But in fibromyalgia patients,  produce wince or withdrawal. The degree of pressure is important. “Dolorimeter” = “Standard pressure spring device” is ideal, but reasonable palpation (at 4kg) is enough for clinical exam. ولتشخيص المرض يكون 11 نقطة أو أكثر من ال18 مؤلمين ويمكن استعمال الضغط اليدوى حتى يحدث ابيضاض لجلد الأظافر. Exam. Deltoid LL Low Back UL

  12. Physical Exam Requirement Systematic palpation of the 18 tender point sites Palpation force is 4 kg or equal to the force needed to just blanch your thumbnail

  13. Clue to diagnose & Associated diagnostic findings

  14. Clue to diagnose: Fibromyalgia affects patients at 40s or 50s. Female preponderance (7/1 to 9/1) (Hawkins, Sept.2013). Clue to diagnose & Associated C/P Prof.Mohamed Elwy AinShams U., 2015

  15. Patients With FM are More Likely to HaveConcomitant Chronic Pain Conditions FM Patients Female n=906 Baseline† Male n=1689 Associations of pain-related conditions among patients diagnosed with FM in the DMBA database between 1997 and 2002 ‡ • 20% of patients with SLE, RA and OA have concomitant FM2 • Because patients with FM are often diagnosed with other pain-related conditions, FM may go undetected DMBA = Deseret Mutual Benefits Administration SLE = Systemic lupus erythematosus; RA = Rheumatoid Arthritis; IBS = Irritable Bowel Syndrome *Headache = headache, tension headache, migraine †Baseline from 52,698 females and 52,232 males without FM ‡Risk ratio = The probability of each condition occurring as compared to a normal, healthy control group (baseline=1) 1. Weir PT, et al. J Clin Rheumatology.2006;12(3):124-128. 2. Wolfe F and Rasker JJ. Fibromyalgia. In: Firestein, ed. Kelly’s Textbook of Rheumatology, 8th Edition. St. Louis, MO: WB Saunders Co; 2008.

  16. Associated diagnostic findings Day&Night Pain Clue to diagnose & Associated C/P Other Systems Psychol-

  17. Associated diagnostic findings Pain 1. Local tender points at several sites (NB: normal sleep-deprived patients exhibit tender points). 2. Occasionally, 'fibrositic nodules'. Clue to diagnose & Associated C/P #. Negative control sites (non-tender), such as forehead, distal forearm and lateral fibular head. 3. Chronic aching, aggravated by cold, stress and activity. Sometime felt allover 4 Subjective swelling of extremities.

  18. Associated diagnostic findings Day&Night 5. Sleep disturbance Pain 1. Local tender points at several sites (NB: normal sleep-deprived patients exhibit tender points). 6. Morning stiffness (They awake exhausted). 2. Occasionally, 'fibrositic nodules'. 7. Marked fatigability (feel more tired in the morning> later). Clue to diagnose & Associated C/P #. Negative control sites (non-tender), such as forehead, distal forearm and lateral fibular head. 3. Chronic aching, aggravated by cold, stress and activity.. Sometimes felt allover 4. Subjective swelling of extremities.

  19. Associated diagnostic findings Day&Night 5. Sleep disturbance Pain 1. Local tender points at several sites (NB: normal sleep-deprived patients exhibit tender points). 6. Morning stiffness (They awake exhausted). 2. Occasionally, 'fibrositic nodules'. 7. Marked fatigability (feel more tired in the morning> later). Clue to diagnose & Associated C/P @. Negative control sites (non-tender), such as forehead, distal forearm and lateral fibular head. 3. Chronic aching, aggravated by cold, stress and activity.. Sometimes felt allover 4. Subjective swelling of extremities. Psychol- 8. Headache: Occipital and bifrontal. & migrain 9. Irritable and weepy. & Chronic Depression 10. Poor concentration and forgetfulness. 11- Can not cope with a job or household activities

  20. Associated diagnostic findings Day&Night 5. Sleep disturbance Pain 1. Local tender points at several sites (NB: normal sleep-deprived patients exhibit tender points). 6. Morning stiffness (They awake exhausted). 2. Occasionally, 'fibrositic nodules'. 7. Marked fatigability (feel more tired in the morning> later). Clue to diagnose & Associated C/P #. Negative control sites (non-tender), such as forehead, distal forearm and lateral fibular head. 3. Chronic aching, aggravated by cold, stress and activity.. Sometimes felt allover Other Systems 4. Subjective swelling of extremities. Psychol- 12. Urinary & GIT: -Urinary: Nocturnal freq..& urgency. -Abdominal diffuse pain & irritable bowel $. - Dysmenorrhoea, 8. Headache: Occipital and bifrontal. & migrain 9. Irritable and weepy. & Chronic Depression 10. Poor concentration and forgetfulness. 11- Can not cope with a job or household activities

  21. Qualitative Studies in FMS Physical Domain Pain Fatigue Disturbed sleep Emotional/Cognitive Domains Depression, anxiety Cognitive impairment (decreased concentration, disorganization) Memory problems Social Domain Disrupted family relationships Social isolation Disrupted relationships with friends Work/Activity Domains Reduced activities of daily living Reduced leisure activities/avoidance of physical activity Loss of career/inability to advance in career or education Arnold, L., Crofford, L., Mease, P., Burgess, S., Palmer, S., Abetz, L., Martin, S. (2008). Patient perspectives on the impact of fibromyalgia. Patient Education and Counseling 73: 114-120.

  22. Qualitative Studies in FMS Katz, R., Heard, A., Mills, M., Leavitt, F. (2004). The prevalence and clinical impact of reported cognitive difficulties (fibrofog) in patients with rheumatic disease with and without fibromyalgia. Journal of Clinic Rheumatology 10(2): 53-58.

  23. “STRESS” GENES ENVIRONMENT Interaction between Symptoms and Function in FM • Psychological and Behavioral Consequences • Decreased activity • Poor sleep • Increased distress • Maladaptive illness behaviors Symptoms

  24. Overlap between Fibromyalgia and Other “Systemic” Syndromes: Chronic Multi-symptom Illnesses FIBROMYALGIA 2 - 4% of population; defined by widespread pain and tenderness MULTIPLE CHEMICAL SENSITIVITY - symptoms in multiple organ systems in response to multiple substances CHRONIC FATIGUE SYNDROME 1% of population; fatigue and 4/8 “minor criteria” SOMATOFORM DISORDERS 4% of population; multiple unexplained symptoms - no organic findings EXPOSURE SYNDROMES e.g. Gulf War Illnesses, silicone breast implants, sick building syndrome

  25. Inclusion criteria To be eligible veterans had have been deployed to the Gulf War between August 1990 and August 1991, and to endorse > 2 of the following symptoms: fatigue limiting usual activity pain in > 2 body regions neurocognitive symptoms These symptoms had to begin after August 1990, last for more than six months, and be present at the time of screening.

  26. Differential Diagnosis Prof.Mohamed Elwy AinShams U., 2015

  27. You Should Exclude D.D.

  28. Hypothesis of FM pathogenesis Prof.Mohamed Elwy AinShams U., 2015

  29. Many Theories: 1- Deficiency of serotonin: (or its precursor, tryptophan). 2- High substance P: increase production of substance P in the afferent neurons  increase pain awareness and sensitivity. 3- HPA dysfunction: Hypothalamic-pituitary adrenal axis (HPA) dysfunction, and other abnormalities of the “NeuroEndocrinal Axis”. 4- Muscle mitochondrial dysfunction & oxidative stress. 5- Viral etiology: Was reported in patients with chronic fatigue $, but in FM is lacking. 6- Life style and chronic stress. 7- Sympathetic hyperactivity. 8- Dopamine dysfunction: Some fibromyalgia patients responded in controlled trials to pramipexole, a dopamine agonist that selectively stimulates dopamine D2/D3 receptors and is used to treat both Parkinson's disease and restless leg syndrome. Theories of FM cause

  30. Many Theories (Cont.): 10- Sleep disturbance: This explain both the sleep disorder and pain associated with fibromyalgia. (due to poor relaxation at night) involving alpha-wave intrusion into Stage IV (non-REM) deep sleep. Theories of FM cause

  31. Local nodule Exam Prof.Mohamed Elwy AinShams U., 2015

  32. Local Gross Exam.  “Fibrositic” nodules: - Are small, tender lumps which may appear at or near sites of pain. - These ('trigger areas') often correspond to ‘acupuncture points‘. - Sometimes, but not always, lies on the 'classical Chinese meridians'. ******************* Local Histological Exam.   (1) Herniation of oedematous fat through fascia.  (2) Small round-cell infiltration.  (3) Type II Muscle atrophy+ fibre necrosis+ lipid accumulations. Nodules (Gross & Histology)

  33. Diagnostic Criteria (a) ACR (1990) (b) ACR (May,2010)

  34. (a) ACR (1990)

  35. (a) ACR (1990) Diagnostic Criteria for FM: 1) Widespread pain - Rt side. - +Lt side. - +Above waist. - +Below waist. 2) Tenderness in >11 point of 18. For >3months + Axial skeletal pain (C-spine, ant.chest, thoracic spine and LBP). Prof.Mohamed Elwy AinShams U., 2015

  36. Common tender points in fibrositic syndrome (n=9, total score = 18), if >11 (simultaneously, or sequentially), it is diagnostic - - - - - - - - - - - - - - - - - - - - - - - - - - X2 1- Occipital. 2- Mid-trapezius = Supraspinatus (above spine of scapula). 3- 2nd costochondral junctions. 4- Medial border of scapula. 5- Lateral epicondyles (maximum tenderness 1-2cm distal to epicondyles). 6- Gluteus (upper outer quadrants of buttocks). 7- Greater trochanter. 8- Medial fat pads of knees 9- Low cervical (C4-6 interspinous lig.). & Lower lumber spine (L4-S1 interspinous lig.). - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -

  37. ACR-Recommended Manual Tender Point Survey* for the Diagnosis of FM TRAPEZIUS – Upper border of trapezius, midportion LOW CERVICAL –Anterior aspects of C5, C7 intertransverse spaces OCCIPUT – At nuchal muscle insertion FOREHEAD SUPRASPINATUS – At attachment to medial border of scapula SECOND RIB SPACE–about 3 cm lateral to sternal border RIGHT FOREARM ELBOW – Muscle attachments to Lateral Epicondyle GLUTEAL – Upper outer quadrant of gluteal muscles KNEE – Medial fat pad of knee proximal to joint line GREATER TROCHANTER – Muscle attachments just posterior to GT LEFT THUMB Control Points Tender Points • Manual Tender Points Survey: • Presence of 11 tender points on palpation to a maximum of 4 kg of pressure (just enough to blanch examiners thumbnail) *Based on 1990 ACR FM Criteria 1. Adapted from Chakrabarty S and Zoorob R. Am Fam Physician. 2007;76(2);247-254.

  38. Fibromyalgia Syndrome (FMS) “ACR” Diagnostic Criteria Widespread pain lasting ≥ 3 months 11 positive tender points out of possible 18 using 4 kg of palpation Occiput Low cervical Trapezius Supraspinatus Second rib Lateral epicondyle Gluteal Greater trochanter Knee Wolfe F, Smythe HA, Yunus MB, Bennett RM, Bombardier C, Goldenberg DL, et al. (1990) The American College of Rheumatology 1990 criteria for the classification of fibromyalgia: report of the multicenter criteria committee. Arthritis Rheum 33:160–72.

  39. (b) ACR (May, 2010) NewCriteria

  40. (b) New Criteria: (1) WPI (Widespread Pain Index) Scale: • Lists 19 areas of the body and you say where you've had pain in the last week. • You get 1 point for each area, so the score is 0-19.

  41. (2) SS Scale: a b The numbers assigned to each are added up, for a total of 0-12.

  42. For a diagnosis you need I+II+III (I) OR WPI of 3-6 And SS score of at least 9 WPI of at least 7 And SS score of at least 5, (II) Symptoms have persisted at this level for the past 3 months. Patient does not have any other disorder or cause to explain the pain. (III)

  43. Determining What’s Wrong

  44. How is FM diagnosed? Symptoms of FM are typically very non-specific, common to many other conditions. Many Symptoms cannot be objectively evaluated.

  45. Value of the New Criteria: 1- Eliminate the use of tender point exam. 2- It allows people with fewer painful areas but more severe symptoms to be diagnosed. 3- It includes cognitive symptoms. 4- It recognizes the difference between "fatigue" and "waking un-refreshed”. 5- Some flexibility is built in, which recognizes the fact that fibromyalgia impacts patients differently, and that symptoms can fluctuate. 6- Better sensitivity and accuracy than the 1990 criteria. Prof.Mohamed Elwy AinShams U., 2015

  46. Investigations FM is a diagnosis of Exclusion

  47. RF; ANA ALT & AST ESR CBC T3, T4 & TSH Blood Sugar CPK HCV Done : Either done to: Prove 1ry FMS ( being all normal ), OR: To diagnose 2ry FM.

  48. Treatment Who treat Fibromyalgia? Since the 1990s, fibromyalgia primarily has been treated by rheumatologists. Even so, it can be difficult to find a doctor who's willing and able to effectively diagnose and treat fibromyalgia. Even so, it can be difficult to find a doctor who's willing and able to effectively diagnose and treat fibromyalgia.

  49. Controversies ? It’s not a real illness, it’s in the “patient’s head” A real condition with severe physical effects in some, although psychologic factors including depression may be the major determinant of pain in others

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