1 / 59

STAFF CONFERENCE

SEPTEMBER 19, 2008 MARIA KARLA C. SAN PEDRO, MD. STAFF CONFERENCE. Objectives. Determine the approach to a child with joint swelling Be familiar with Relapsing Polychondritis as a differential diagnosis for joint swelling

sari
Download Presentation

STAFF CONFERENCE

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. SEPTEMBER 19, 2008 MARIA KARLA C. SAN PEDRO, MD STAFF CONFERENCE

  2. Objectives • Determine the approach to a child with joint swelling • Be familiar with Relapsing Polychondritis as a differential diagnosis for joint swelling • Differentiate Relapsing Polychondritis from other arthritides with systemic manifestations • Know the diagnosis and management of Relapsing Polychondritis

  3. Our Patient • D.C., 6 /M • From Meycauayan, Bulacan • First admission on July 23, 2008 • Chief complaint: right ear swelling

  4. History of Present Illness • 9 months PTA • Right ankle pain after tripping on the street, grade 4/10, associated with swelling, warmth, low grade fever; difficulty in ambulation • Given Paracetamol but with no relief • Brought to Philippine OrthopedicCenter; casting done; no relief

  5. History of Present Illness • 8 months PTA • Increase in severity of pain of right ankle, grade 7/10 accompanied with swelling of the nose, tender, warm • Brought to a local health center and given Cefalexin x 5 days with partial relief • 7 months PTA • Fell from his bike; swelling of left ankle with superficial abrasions

  6. History of Present Illness • 6 months 3 weeks PTA • Left knee swelling accompanied by difficulty in ambulation • Brought to a private doctor and given Cloxacillin x 7 days with temporary relief

  7. History of Present Illness • 6 months PTA • Persistence of difficulty in ambulation • Brought to Philippine OrthopedicCenter • A> Septic Arthritis of the left knee • Underwent Incision and Drainage and arthrotomy of the left knee and left ankle; purulent material on drainage of fluid • Given Oxacillin; Biopsy: granulomatous inflammation; started on Anti Koch’s; discharged apparently improved

  8. History of Present Illness • 3 months PTA • Brought to Philippine OrthopedicCenter for swelling of both wrists and elbows accompanied by undocumented fever • Residual purulent material on the left knee and ankle; advised admission but opted to go home

  9. History of Present Illness • 1 month PTA • Increase in severity of symptoms • Brought to Mary Johnston Hospital • A> Juvenile Idiopathic Arthitis • Given Methotrexate

  10. History of Present Illness • 6 days PTA • Enlargement of the right ear with redness and itchiness associated with swelling and tenderness of both wrists, both elbows, left knee, and left ankle • Brought to a private doctor and referred to PGH Rheumatology

  11. Review of Systems (+) Oral ulcers x 6 days (-) Genital ulcers (-) Dyspnea (-) Dysphagia (-) Epistaxis (-) Colds (-) Blurring of vision (-) Vomiting (-) Raynaud’s phenomenon (-) Weight loss (-) Oliguria, Hematuria

  12. Past Medical History • Dengue Hemorrhagic Fever – 3 y/o • Mumps – 5 y/o • No previous Blood transfusions • No previous operations Family Medical History (+) Hypertension – paternal aunt (-) DM, PTB, BA, Kidney disease, Liver disease

  13. Birth and Maternal History • FT via SVD to a 29 year old G3P2 (2002) mother at home c/o traditional birth attendant; mother with regular PNCU c/o LHC • Mother had frequent cough and colds during pregnancy; treated with Paracetamol with relief; no intake of teratogenic drugs, no exposure to radiation • At birth, patient had good cry and activity; no fetomaternal complications

  14. Nutritional History • Breastfed until 2 months old • Shifted to formula feeding with Bonna at 2 months to 1 year old • Complementary feeding at 6 months old • No food preferences

  15. Immunization History • (+) BCG, OPV3, DPT3, Hepa B3, measles Developmental History • At par with age

  16. Personal and Social History • Youngest of 3 children • Stopped schooling in kindergarten due to illness • Mother is a 35 year old housewife • Father is a 53 year old construction worker

  17. Physical Examination • Awake, alert, ambulatory with support, NICRD • BP 110/70, HR 110 bpm, RR 23 cpm, T 38oC • Weight 15 kg, Height 109.5 cm, BMI 13, WFA=71 (moderate PEM), HFA=95 (no stunting), WFH=83 (mild wasting) • Warm, moist skin, no active dermatoses

  18. Physical Examination • Pink palpebral conjunctivae, anictericsclerae • (+) right auricular swelling, (-) discharge, (-) tenderness • (+) saddle nose deformity, (-) discharge, (-) tenderness • (-) oral ulcers, (-) tonsillopharyngeal congestion • trachea at midline, (-) cervical lymphadenopathy

  19. Physical Examination • Equal chest expansion, (-) retractions, clear breath sounds, (-) wheezes, (-) crackles • Adynamicprecordium, distinct heart sounds, tachycardic, regular rhythm, (-) murmur, (-) heaves, (-) thrills • Flat, soft, normoactive bowel sounds, (-) tenderness, (-) organomegaly, LE edge not palpable, (-) masses • Grossly male, descended testes, (-) lesions, (-) discharge

  20. Physical Examination • Full and equal pulses • (+) swelling and tenderness of right elbow, bilateral wrists, left knee, left ankle, (+) superficial abrasion on left ankle, (+) linear scar over left ankle and left knee • (-) cyanosis, (-) jaundice

  21. Physical Examination Left knee: 0°-90°, Left ankle: dorsiflexion: 0° plantar flexion: 0°-20° inversion: 0°-5° eversion: 0° Right ankle: full range of motion

  22. Physical Examination Neuro Exam • General survey: Awake, coherent • Cranial nerves: Pupils 2-3 mm EBRTL, brisk corneals, (-) facial asymmetry, tongue midline, (+) gag reflex, good shoulder shrug • Sensory: 100% on all extremities • Motor: 5/5 on all extremities • DTRs: ++ on all extremities • Cerebellar: Can do FTNT and APST with ease • Meningeals: Supple neck, (-) nuchal rigidity • Other reflexes: (-) Babinski

  23. Initial Assessment • Possible Relapsing Polychondritis

  24. Summary • 6 year old male • Admitted for swelling of the right ear • Right ankle pain and swelling • Swelling of the nose • Swelling of the left ankle and left knee • Progressive joint swelling of both wrists and elbows

  25. Arthritis

  26. Arthritis • Inflammation of a joint space associated with joint swelling, pain, and limitation of motion • Results from infection, trauma, degenerative changes, or metabolic disturbances • Extra articular involvement with arthritis: Wegener’s Granulomatosis and Behcet’s Disease

  27. Wegener’s Granulomatosis nasal/oral inflammation  saddle nose deformity  lung nodules/cavities  microhematuria/red cell casts

  28. Behcet’s Disease  arthritis and arthralgia  recurrent oral ulceration (3x per year)  recurrent genital ulceration  eye lesions  skin lesions like erythemanodosum

  29. Monoarticular Arthritis • TB Arthritis  responsive to Anti Koch’s • Septic Arthritis  responsive to antibiotics  acute onset

  30. Polyarthritis

  31. Gout  excruciating, sudden, unexpected, burning pain  swelling, redness, warmth, and stiffness involving ankle, heel, instep, knee, wrist, elbow, fingers, and spine  tophi affecting the big toe and helix of the ear

  32. Osteochondroma  involvement of cartilage  involvement of bone  enlarging mass  weight loss

  33. Juvenile Idiopathic Arthritis  more than 6 weeks of pain, swelling, and stiffness of joints  involves metacarpophalangeal joints, proximal interphalangeal joints wrists, and metatarsophalangyeal joints  involvement of cartilages such as the ears and nose  nonerosive and asymmetric

  34. Relapsing Polychondritis

  35. Definition • Multisystemic disorder of unknown etiology affecting young adults • Recurrent, progressive episodes of inflammation affecting the cartilaginous structures, resulting in tissue damage • Elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, fibrocartilage of the axial skeleton, and cartilage of the tracheobronchial tree

  36. Diagnostic Criteria (3 or more) recurrent chondritis of both auricles  non erosive inflammatory polyarthritis  chondritis of nose cartilage  inflammation of ocular structures (keratitis, scleritis, episcleritis, uveitis)  chondritis of the respiratory tract (laryngeal and/or tracheal cartilages)  cochlear and/or vestibular damage causing sensorineural hearing loss, tinnitus and/or vertigo

  37. Course in the Hospital

  38. Rheumatologic • Referred to Pediatric Rheumatology and Orthopedics • Referred to Otorhinolaryngology for evaluation and anticipatory care for airway • Xrays: decreased joint space and osteochondral changes on the left knee and left ankle, no joint space, no osteochondral changes on the right ankle

  39. Rheumatologic • Diagnostic aspiration on the right ear • Neck STAPL: intact tracheobronchial airway and no obstruction

  40. Rheumatologic • Started on Prednisone 10 mg/tab (1.5 mkd) 1 tab OD, Naproxen 275 mg/tab (20 mkd) ½ tab BID, Oxacillin (250) 750 mg/IV q6 • 2nd HD, (+) resolution of joint swelling and tenderness, afebrile, with good activity and appetite • P> Oxacillin shifted to Cloxacillin 250 mg/5 ml (120) 9 ml q6 • Prednisone increased to 10 mg/5 ml (2 mkd) 5 ml TID

  41. Cardiac • Referred to Pediatric Cardiology for evaluation of cardiovascular functioning • ECG: sinus tachycardia, no axis deviation, no chamber enlargement • 2D Echo: fair LV systolic function, mild TR, LVE, no vegetation, minimal pericardial effusion • CK MB and Troponin I: positive • A> Possible MyocarditisvsCardiomyopathy

  42. Cardiac • P> Started on Dobutamine (5 mcg/kg/min) at 5 cc/hr, Furosemide (1) 15 mg/IV OD, and Lanoxin (0.003) 0.25 mg/tab, 0.045 mg/pptab, 1 pptab q12 • 2nd HD, comfortable, not in acute distress with HR 90 bpm • Dobutamine discontinued, Lanoxin continued, and Furosemide was shifted to PO 20 mg/tab (0.75) ½ tab OD

  43. Etiology • Remains unknown • Loss of basophilic staining of the cartilage matrix with perichondral inflammation of the cartilage • Perivascular mononuclear and polymorphonuclear cell infiltrates • Chondrocytes become vacuolated, necrotic and replaced by fibrous tissue

  44. Etiology • Release of degradative enzymes • Immune mediated activation of chondrocytes and other inflammatory cells by cytokines including IL-1 and TNF-a • Autoimmunity

  45. Prevalence • 3.5 cases/million in the US (Doros, A.A, October 2004) • 4 cases seen in PGH • Peak age for disease onset is the 5th decade • Female preponderance with ratio of 3:1

  46. Clinical Features • Auricular chondritis • Joint pain with or without arthritis involving metacarpophalangeal, proximal interphalyngeal joints, wrists and knees • “Saddle nose” deformity • Scleritis, episcleritis, keratitis, and conjunctivitis

  47. Clinical Features • Hoarseness, non productive cough, dyspnea, wheezing, and inspiratorystridor • Tenderness over thyroid cartilage and trachea • Aortic regurgitation and mitral regurgitation • Thoracic and abdominal aneurysm, myocarditis, pericarditis, silent myocardial infarction, paroxysmal atrial tachycardia, and 1st degree or even complete heart block

More Related