Rheumatology Flashcards
What is Rheumatoid Arthritis ??
Chr., systemic autoimmune disorder characterised by Inflammatory SYNOVITIS => joint damage along with extra-articular manisfestations
- FEMALE predominance
- 30 to 50 yrs old
Pathophysiology of RA ??
- Autoimmune: immune system targets SYNOVIUM => Chr. inflam.
- Key Cytokines: TNF, IL-1, IL-6
- Pannus formation: invades Cartilage, bone => Joint destruction
- Genetics: a/w HLA- DR4, DR1
C/F of RA
RA is a Clinical Dx. supported by lab. tests & Imaging
Joint c/f + Systemic c/f + Extra-articular manifestations
- SYMMETRICAL Polyarthritis involving Small joints of hand (MCP, PIP, MTP)
- Morning stiffness > 30 min
- Tenderness, swelling & reduced range of movt.
- Deformities
What is RA Classification Criteria ?? (Very imp. for exam)
2010 ACR/EULAR Criteria for RA [score >= 6/ 10 confirms RA]
- Joint involvement (0- 5): Small joint scores»Large joint scores
- Serology (0- 3): RF & ACPA
- Acute phase reactants (0- 1): ESR or CRP (indicate disease activity)
- Symptom duration (0- 1): <6 wks or >= 6 wks
X-ray features of RA ??
Early: Periosteal Osteopenia, Joint space narrowing
Late: Erosion & Subluxations
What is the cornerstone of RA Rx. ??
- METHOTREXATE
- Severe/ Rapid progression: MTX + Biologic DMARD
- Regular assessment every 1 to 3 months during dose adjustments
How is RA managed in Pregnancy ??
- Safe drugs: Sulfasalazine, HCQ, Low-dose Corticosteroids
- Avoid: MTX (stop 6 months before conception), Leflunomide (requires washout protocol)
- Flares are common POST-PARTUM
What are the indicators of poor prognosis of RA ??
- High RF or ACPA titres.
- Early erosions on imaging.
- Extra-articular manifestations.
What is Felty’s syndrome ??
RA + Splenomegaly + Neutropenia
Rx. strategy of RA ??
Symptom relief: Naproxen, Ibuprofen
Acute Flares/ Bridging Therapy: PREDNOSOLONE
Conventional DMARDs:
- 1st line: MTX + 5mg Folic acid weekly
- Other DMARDs: Sulfasazine, HCQ, Leflunomide
Biologic DMARDs (Target Therapy
- Indication: Active disease despite DMARDs Monotherapy/ combination therapy
What are the Biological DMARDs (Targeted Therapy) used in the treatment of RA ??
TNF inhibitors: Etanercept, Infliximab, Adalimumab
- Risk: TB Reactivation (screen before use), DEMYELINATION
IL-6 inhibitors: Tocilizumab
B-cell depleters: RITUXIMAB (used after TNF failure)
JAK inhibitors: Baricitinib, Tofacitinib
Hallmark features of Biologic DMARDs therapy in RA ??
TNF inhibitors:
- screen for TB before starting
- Demyelination (specially with ETANERCEPT) can occur
Rituximab is used ONLY after TNF failure
Common complications of RA ??
Pulm. Fibrosis, pleural effusion
KCS
Osteoporosis, depression, infections
What should be monitored in a pt. with RA on HCQ ??
Regular EYE examinations
- Bull’s eye RETINOPATHY
- Corneal deposits
Ix. of Choice:- Colour Retinal Photography & Spectral Domain Optical Coherence Tomography of MACULA
Which axial skeleton pathology is seen in RA ??
Cervical Spine Instability (due to Atlanto-axial subluxation)
What is Psoriatic Arthropathy ??
INFLAMMATORY Arthritis a/w psoriasis & is Seronegative
- Poor prognosis with Cutaneous Psoriasis & often PRECEDES skin lesions
- Males & females are equally affected
Hallmark features of Psoriatic Arthropathy ??
- Symmetrical Polyarthritis (similar to RA) - 30 to 40% cases
- Asymmetrical Oligoarthritis (typically- Hands & Feet) - 20 to 30%
- Sacroiliitis
- DIP joint disease
- Arthritis Mutilans (Telescoping fingers)
- Nail changes: Pitting, Onycholysis
- Periarticular disease (Enthesitis, Tenosynovitis, Dactylitis)
X- ray features of Psoriatic Arthritis ??
Erosive change + New bone formation
- Periostitis
- ‘Pencil-in-cup’ appearance
Rx. of Psoriatic Arthropathy ??
Rx. similar to that of RA; managed by Rheumatologist
- Mild peripheral arthritis/ Mild axial disease: NSAIDs
- Use of monoclonal antibodies such as USTEKINUMAB (targets IL-12 & 23) & SECUKINUMAB (targets IL-17)
has better prognosis than RA
Hallmark features of Ank. Spon. ??
HLA-B27 associated Spondylo-arthropathy
- 3x MC in Males; 20 to 30 yrs
- Low back pain + Stiffness (Insidious onset)
- PAIN at night which improves on getting up
- STIFFNESS worse in morning & improves with exercise
O/E features of Ank. Spon. ??
Reduced LATERAL Flexion
Reduced FORWARD Flexion -
- Schober’s test: a line is drawn 10cm above & 5cm below [the line joining Venus dimples] => the distance b/w the 2 points should increase by > 5cm on forward flexion
Cauda equina synd. can occur
What is Axial Spondyloarthritis ??
Clinically Heterogenous, Chr. inflammatory rheumatologic condition that may cause musculoskeletal & extra-musculoskeletal manifestations
What are the A’s of Ank. Spon. ??
Apical Fibrosis
AV Block
Aortic Regurgitation
Amyloidosis
Anterior Uveitis
Achilles tendonitis
Features of Axial Spondyloarthritis ??
Radiologic Axial SA
- signs of Sacroiliitis & str. changes on x-ray (aka Ank. Spon.)
Non- radiological Axial SA
- No x-ray changes but possible Sacroiliitis on MRI
Extra-musculoskeletal manifestations
- Acute anterior uveitis
- IBD
- &/or Psoriasis
Pathophysiology of Axial Spondyloarthritis ??
HLA-B27
Mechanical Stress
Infection
Gut Microbiome disruption
Rx.
- Initial: trail of NSAIDs (at max. tolerated dose for 2-4 wks)
- Switch to another NSAID (if does’t provide adequate pain relief)
What are the Anti-Neutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis ??
Small vessel Vasculitides
- Churg-Strauss Disease (E-GPA) pANCA (+)ve
- Wegener’s Disease (GPA) cANCA (+)ve
- Microscopic Polyangiitis- pANCA (75%) & cANCA (40%)
More common with Increasing age
Features of ANCA associated Vasculitis ??
Renal impairment
- Caused by Immune Complex GN: raised Cr., Haematuria & Proteinuria
Respiratory symptoms
- Dyspnoea
- Haemoptysis
Systemic symptoms
- Fatigue, Wt. loss, Fever
Vasculitic Rash (minority pts.)
Ear, Nose & Throat c/f
Sinusitis
Which other conditions are a/w pANCA ??
Ulcerative Colitis (70%)
PSC (70%)
Anti-GBM disease (25%)
Crohn’s disease (20%)
What are the targets of
- cANCA ??
- pANCA ??
- Serine Proteinase 3 (PR3)
- Myeloperoxidase (MPO)
cANCA, to some extent can be used to monitor disease activity but NOT pANCA
What is Polyarteritis Nodosa ??
Vasculitis affecting the Meduim-sized arteries with NECROTIC Inflammation leading to Aneurysm formation
- Middle aged MEN
- a/w Hep. B infection
Features of PAN ??
- Fever, malaise, Wt. loss
- Arthralgia
- HTN
- Mononeuritis multiplex & Sensorimotor Polyneuropathy
- Livedo reticularis
- Haematuria, Renal failure
- pANCA - 20% of ‘Classic’ PAN pts.
- HBV serology (+)ve in 30%
What is Behcet’s Syndrome ??
Complex multi-system disorder a/w presumed Autoimmune -mediated inflammation of arteries & vein
Features of Behcet’s disease ??
Oral ulcer–Genital ulcer–Ante. Uveitis
- Thrombophlebitis & DVT
- Arthritis
- CNS (eg. Aseptic Meningitis)
- GI (Abd. pain, Diarrhoea, Colitis)
- Erythema nodosum
Hallmarks of Behcet’s synd. ??
- HLA-B51 (a split antigen of B-5)
- Eastern Mediterranean (eg. Turkey)
- More common & severe in MEN
- Young adults: 20 to 40 yrs old
- (+)ve FHx. in 30%
Test used to Dx. Behcet’s synd. ??
NO definitive test
- Dx. based on C/F
(+)ve Pathergy test is suggestive
- puncture site following needle prick becomes red & inflamed with Small pustule formation
What is Familial Mediterranean Fever (FMF) ??
aka Recurrent POLYSEROSITIS
- A R, typically seen in 2nd decade
- MC in Turkish, Armenian & Arabics
Note: Behcet’s is also MC among Eastern Mediterraneans)
Features of FMF ??
Attack typically lasts 1 to 3 days
- Abd. pain (due to Peritonitis)
- Pleurisy
- Pericarditis
- Arthritis
- ERYSIPELOID rash on LLs
Rx.- Colchicine
What is Langerhans Cell Histiocytosis ??
a/w Abnormal proliferation Histiocytes
- Typically presents in Childhood with Bony lesions
- Bone pain of SKULL or Proximal FEMUR
- Cutaneous nodules
-Recurrent otitis media/ Mastoiditis
Ix. hallmarks of Langerhans Cell Histiocytosis ??
X-ray skull: Multiple, well defined ‘punched out’ osteolytic lesions with Scalloped edges (Geographic skull); lesions have bevelled edges
Electro-microscopy: BIRBECK Nodules (Tennis racket shaped)
What is Chronic Fatigue Synd. ??
Disabling Fatigue for >= 4 months affecting Mental & Physical function > 50% of times & is a Dx. of exclusion
- FEMALE predominance
- Past Psychiatric history is NOT a risk
Investigation of CFS ??
Large no. of Screening blood test to exclude other pathology eg.- FBC, U&E, LFT, Glucose, TFT, ESR, CRP, Ca2+, CK,
Ferritin (Children & Young people only), Coeliac screening, Urinalysis
Rx. of CFS ??
- CBT (very effective) [NNT is 2]
- Graded Exercise Therapy- a formal supervised program & [NOT the Gym]
- Pacing- organize activities to avoid tiring
- Poor Sleep: LOW dose Amitriptyline
- Pain predominant: Pain mgt. clinic
Better prognosis in CHILDREN
What is Fibromyalgia ??
Synd. characterised by widespread PAIN throughout the body with TENDER Points at specific anatomical sites
- 5x more in WOMAN 30- 50 yrs old
Features of Fibromyalgia ??
- Chr. PAIN: at multiple sites
- Lethargy + Sleep disturbed + Headache + Dizziness
- Cognitive slowdown ‘Fibro fog’
Dx. & Rx. of Fibromyalgia ??
American College of Rheumatology Classification (lists 9 pairs of tender points - 11/ 18 confirms Dx.)
- AEROBIC Exercise (strongest evidence base)
- CBT
- Pregabalin, Duloxetine, Amitriptyline
Epidemiological features of SLE ??
- F : M = 9:1 ; MC in Afro-Caribbean origin & Asian communities
- Onset 20 to 40 yrs
Incidence: Black Americans»Black Africans
Pathophysiology of SLE ??
- Autoimmune: Type 3 HS reaction
- a/w HLA- B8, DR2, DR3
- Immune system dysregulation => Immune Complex formation => can affect any organ (Skin, Joints, Kidney, Brain)
What is the MC Cardiovascular & Renal pathology seen in SLE ??
Pericarditis-MC cardiac manifestation
Renal
- Proteinuria
- Diffuse Proliferative GN [the MC type]
Which are most Sensitive & Specific antibodies of SLE ??
- ANA (99% sensitivity, low specificity)
- Anti-dsDNA: Specificity > 99% & Sensitivity 70%
- Anti-Smith: Specificity > 99% & Sensitivity 30%
How is SLE disease activity monitored ??
- ESR is generally used
- C3, C4 are low during active disease (immune complex formed=> consumed)
- Anti- dsDNA can be used (but not present all the time)
- raised CRP can indicate underlying infection
Rx. of SLE ??
- ToC is HCQ
- If Internal organs involved: Prednisolone, Cyclophosphamide
- NSAIDs & Sun-blocks
What is Neonatal Lupus Erythematosus ??
SLE maternal antibodies can cross the placenta & cause this disease
- Congenital Heart Block
- Strongly a/w Anti-Ro (SSA) antibody
Rx. of Discoid Lupus Erythematous ??
- 1st line: Topical Steroid cream
- 2nd line: HCQ
- Avoid Sun exposure
What is Discoid Lupus Erythematous ??
Characterised by Follicular Keratin Plugs & has autoimmune cause
- MC in Females & is ANA (-)ve
- Erythematous, raised rash, sometimes scaly & may be photosensitive
- MC Sites: Face, Neck, Ear, Scalp
Lesions heal with atrophy, scarring (causes scarring alopecia) & pigmentation
Hallmarks of Drug induced Lupus ??
Renal & CNS involvement is unusual & Skin & Resp. features are common
- Arthralgia, Myalgia,
- Malar Rash & Pleurisy are COMMON
Anti-Histone antibody (80-90%)
ANA (+) in 100%, dsDNA is (-)ve
Name the drugs causing Drug-induced Lupus
MCC: Procainamide & Hydralazine
Less Common: Isoniazid, Minocycline, Phenytoin
What is Antiphospholipid Syndrome ??
Acquired disorder & Can occur as
- Primary disorder or
- Secondary disorder (MC being SLE)
Features of APL Synd. ??
- Venous/Arterial Thromboses
- Recurrent Fetal loss
- Livedo Reticularis
Thrombocytopenia + Prolonged APTT
Rx. of APL Synd. ??
Primary Thromboprophylaxis
- Low dose Aspirin
Secondary Thromboprophylaxis
- VTE (1st time): Warfarin (lifelong) with a INR target of 2-3
- VTE (Recurrent): Warfarin (lifelong); if occurred while on Warfarin, consider adding Low dose Aspirin & INR target at 3-4
Arterial Thrombosis
- Warfarin (lifelong); INR target 2-3
Why is there a paradoxical rise in APTT in APL synd. ??
An Ex-Vivo reaction to Lupus anticoagulant autoantibodies with Phospholipids involved in Coagulation Cascade
What is Gout ?
Form of Microcrystal synovitis caused by MSU crystal deposition in the synovium
- A form of Inflammatory Arthritis
- Chr. hyperuricaemia (UA > 0.45 mmol/l)
Ix. done in Gout ??
Synovial Fluid Analysis
- Needle shaped (-)vely birefringent MSU crystal under Polarised light
Uric Acid
- Checked after acute episode subsides (2 wks later)
Features of Gout ??
PAIN + Swelling + Erythema
- 1st MTP joint (involved in 70% cases)
Other commonly affected joints are
- Ankle, Wrist, Knee
If untreated can lead to Chr. joint problem
Radiologic features of Gout ??
- Joint Effusion is a Early sign
- Well defined punched out erosions + Sclerotic margins in juxta-articular distribution often with overhanging edges
- Joint space preserved until Late disease
- Eccentric erosions
- NO Periarticular Osteopenia
Rx. of Acute Gout ??
- 1st line: NSAIDs»>& Colchicine
(Max. dose NSAIDs prescribed until 1- 2 days + PPIs) - 2nd line: Corticosteroids- 15 mg/day (if 1st line is CI)
- Intra-articular Steroid injection
- Allopurinol should be continued if already on it
What are the indications for Urate Lowering Therapy ??
- > = 2 attacks in 12 months
- Tophi
- as Prophylaxis if on Cytotoxics/ Diuretics
- Renal disease
- UA Renal stones
Rx. strategy of Urate Lowering Therapy (ULT) ??
1st line: ALLOPURINOL (starting of ULT is delayed until acute inflam. has settled as it is better discussed when pt. is NOT in pain)
- Colchicine cover should be considered
- NSAIDs used if Colchicine is CI
2nd line: FEBUXOSTAT (xanthine oxidase inhibitor)
What drugs are tried in Refractory cases of Gout ??
- Uricase (Urate oxidase)- catalyzes conversion of Urate into Allantoin
- Persistently symptomatic + Severe Gout despite adequate ULT => PEGLOTICASE (polyethylene glycol modified mammalian Uricase), achieves rapid control of Hyperuricemia
Which Anti-hypertensive has a Uricosuric action ??
LOSARTAN
Drug induced causes of Gout ??
Diuretics (Thiazides, Furosemide)
Ciclosporin
Alcohol
Cytotoxics
Pyrazinamide
Aspirin (Both Low & High dose)
MoA of Colchicine ??
(-) Microtubule Polymerization by binding to Tubulin => interferes with Mitosis
Also (-) Neutrophil motility & activity
- Slow onset of action than NSAIDs
- Diarrhoea is the main S/E
What is Lesch-Nyhan Synd. ??
Hypoxanthine-guanine phospho-ribosyl transferase (HGPRTase) deficiency
- X- linked Recessive
- Gout, Renal failure, Neurological deficits, Learning difficulties, Self-mutilation
What is Pseudogout or Acute Ca Pyrophosphate crystal deposition disease ??
Form of Microcrystal Synovitis
- Ca Pyrophosphate dihydrate crystal deposition in the synovium
Risk Factors of Pseudogout ??
strongly a/w Increasing age
If pts. develop at younger age < 60yrs, they usually have some RFs
- Haemochromatosis
- Hyperparathyroidism
- Low Mg2+, Low PO4
- Acromegaly
- Wilson’s disease
Features & Rx. of Pseudogout ??
-Knee, Wrist & Shoulders MC affected
- Joint aspiration: Weakly (+)ve bi-refringent Rhomboid-shaped crystals
X-ray: CHONDROCALCINOSIS
- In Knee: Linear calcifications of meniscus & articular cartilage
Rx. of Pseudogout ??
Joint aspiration, exclude Septic Arthritis
NSAIDs or Intra-articular/ IM/ Oral Steroids
What is Marfan’s syndrome ??
A D connective tissue disorder
- FBN1 gene defect on Chr. 15 which codes for Fibrillin 1
Features
- Tall stature; Arm span > 1.05
- High arched palate
- Arachnodactyly, Pes planus
- Pectus excavatum
Mention the following about Marfan’s
- Heart pathology ??
- Eye pathology ??
- Spinal pathology ??
- Dilated Aortic Sinus (90%) which can lead to Aortic aneurysm, Aortic dissection, AR, MVP (75%)
- Superotemporal Ectopia Lentis, Blue Sclera, Myopia
- Ductal Ectasia (Ballooning of Dural sac at Lumbosacral level)
Rx. of Marfan’s ??
2D Echo monitoring
Beta blockers/ ACEi therapy
- Aortic dissection & other CVS problems remain the leading cause of death
What is Ehler-Danlor Synd. ??
A D connective tissue disorder
MC affects Type 3 collagen
Features
- Elastic, Fragile skin
- Joint Hypermobility: Recurrent Joint dislocation
- Easy bruising
- AR, MVP & Aortic dissection
- SAH
- Angiod Retinal Streaks
What is Sharp’s Synd. ??
Mixed Connective Tissue Disease
Has features of SLE + SSc + Myositis
- Females 3x more common
- Presents by 30- 40 yrs; may present in children
Presents with Raynaud’s phenomenon in 90% cases
Sausage Fingers (Dactylitis)
Features of MCTD ??
- Polyarthralgia/arthritis ; Myalgia
- Dermatology: Photosensitive rash, Scleroderma-like changes, Alopecia
- Oesophageal dysfunction
- Resp.: Pleuritis, Pulm. HTN, ILD
- Hamatological: Anaemia, Lymphadenopathy, Splenomegaly
- Cardiac: Pericarditis, Pericardial effusion, Accelerated CAD
- Renal: Glomerulonephritis
- Neuropsychiatric: Seizures, Mood changes
Ix. done in MCTD ??
- FBC: Anaemia, Leucopenia, Thrombocytopenia
- U&E: Renal impairment
- CRP & ESR raised
- Anti-U1 RNP (extractable nuclear antigen, ENA) must be (+)ve
- ANA usually (+)ve
Rx. of MCTD ??
- Raynaud’s: CCBs
- Reflux disease: PPIs
- Pulm. HTN: Endothelin receptor antagonist/ Prostacyclin analogues
- Smoking cessasion
- Moderate exercise
1/3- Long term remission; 1/3- Chr. symptoms; 1/3- Severe systemic involvement & Premature death
What is Myopathy ??
Symmetrical muscle weakness (Proximal»Distal)
- Difficult to get up from chair/ getting out of bath
- Sensation is normal, Reflex normal, No fasciculations
Causes-
- Polymyositis
- Duchenne/ Becker M D, Myotonic D
- Cushing’s, Thyrotoxicosis
- Alcohol
Ix. & Rx. of PMR ??
Raised ESR > 40 mm/hr
CK & EMG are NORMAL
Prednisolone 15mg (OD)
- Pts. dramatically responds & Failure to respond should prompt consideration of an alternative Dx.
What is Polymyalgia Rheumatica ??
> 60 yrs old + Muscle stiffness + Raised Inflammatory marker
- Closely related to Temporal arteritis
- RAPID Onset
- Aching, Morning stiffness in Proximal limbs (Weakness is NOT considered a c/f in PMR)
- Mild Polymyalgia, Lethargy, Depression, Low-grade fever, Anorexia, Night sweats
What is Temporal Vasculitis ??
Large vessel Vasculitis that overlaps with PMR
- > 60 yrs + Rapid Onset (< 1 month)
- HEADACHE
- Jaw Claudication
- Tender palpable Temporal artery
Ix. of Temporal arteritis ??
- ESR > 50 mm/hr (In 10% cases, it is <30 mm/hr)
- CRP can also be elevated
Temporal Artery Biopsy - SKIP Lesions
CK & EMG are Normal
Most imp. Ix. that should be done in pts. with Temporal arteritis ??
Vision testing
- AION is the major ocular complication
- Due to POSTERIOR Ciliary Artery (a branch of Ophthalmic artery) => Ischaemia of O N head
- Swollen pale disc with Blurred margins on Fundoscopy
- Amaurosis fugax
Rx. of Temporal Arteritis ??
Urgent High dose Glucocorticoids as soon as Dx. & before T Biopsy
- High dose Prednisolone (if NO visual loss or Jaw/ tongue claudication)
- IV Methylprednisolone (if evolving Visual loss or Jaw/tongue claudication present) followed by High dose Pred.
There should be dramatic response to Rx or else consider alternative Dx.
Bisphosphonates for bone protection
What is Polymyositis ??
Inflammatory disorder causing Symmetrical, Proximal muscle weakness
- T-cell mediated cytotoxic process directed against Muscle fibres
- Idiopathic or a/w Connective T D
- a/w Malignancy
Middle aged FEMALES
Features of Polymyositis ??
- Proximal muscle weakness +/- Tenderness
- Raynaud’s phenomenon
- Respiratory muscle weakness
- ILD: Fibrosing alveolitis or Organising pneumonia
- Dysphagia, Dysphonia
Ix. of Polymyositis ??
- CK is elevated
- Other muscle enzymes are also elevated: LDH, Aldolase, AST & ALT
- EMG
- Anti- SYNTHETASE antibody
- Polymyositis + Lung involvement, Raynaud’s & Fever is a/w which antibody ??
- Polymyositis
- Dermatomyositis
- Anti-Jo-1 antibodies
- Anti-Jo-1 antibodies
- Anti-Mi-2 antibody
What is Dermatomyositis ??
Polymyositis (same C/F) + Skin lesions
- Idiopathic or a/w Connective tissue disorders or Malignancy
- Polymyositis is a variant of the disease where Skin manifestations are not prominent
What are the Skin features of Dermatomyositis ??
- Photosensitive
- Macular rash over Back & shoulder
- Heliotrophe rash in periorbital area
- Gottron’s papules: Roughened Red papules over Extensors of Fingers
- Mechanic’s Hand: Extremely dry & Scaly hands with linear cracks on Palmer & Lateral aspects of fingers
- Nail Capillary fold dilatation
Which malignancies are a/w Dermatomyositis ??
Ovarian, Breast & Lung Cancers
- Screening for the same is done following the Dx. of Dermatomyositis
Markers & Antibodies a/w Dermatomyositis ??
ANA (+)ve in 80% cases
Aminoacyl T-RNA Synthetase (Anti- Synthetase antibodies) in 30% cases
- Antibodies against Histidine- tRNA ligase (called Jo-1)
- Antibody to Signal Recognition Particle (SRP)
- Anti-Mi-2 antibodies (Highly Specific but only seen in 25% cases)
Ix. in Dermatomyositis ??
- Elevated CK
- Muscle Biopsy & EMG
- ANA (+)ve in 60% cases
- Anti-Mi-2 antibodies (25% cases)
Rx of Polymyositis & Dermatomyositis ??
Prednisolone
What is Relapsing Polychondritis ??
Multi-system disorder characterised by Inflammation & Deterioration of Cartilage
- MC affects EARS
- Also affects Nose & Joints
Features of Relapsing Polychondritis ??
- Ears: Auricular chondritis, Hearing loss, Vertigo
- Nose: Nasal chondritis => Saddle- nose deformity
- Resp. Tract: Hoarseness, Aphonia, Wheezing, Inspiratory Stridors
- Ocular: Episcleritis, Scleritis, Iritis, KCS
- Joints: Arthralgia
- Cardiac Valvular Regurgitation, CN palsies, Peripheral neuropathy, Renal dysfunction
Rx. of Relapsing Polychondritis ??
Induce Remission: STEROIDS
Maintenance: Axathioprine, MTX, Cyclosporin, Cyclophosphamide.
What are the 2 forms of Reactive Arthritis ??
Post-STI (MC in Men)
Post-Dysentric (Equal Sex incidence)
Causes-
Post-STI form
- Chlamydia trachomatis
Post-dysenteric:
- S flexineri
- S Typhimurium
- S Enteritidis
- Yersinia Enterocolitica
- Campylobacter
Features of Reactive Arthritis ??
Develops within 4 wks of initial infection & C/F last for around 4- 6 months
- ASYMMETRIC Oligoarthritis of LLs
- Dactyltis
- Urethritis
- Eye: Conjunctivitis & Anterior Uveitis
- Skin:
—- Circinate balanitis: Oainless vesicles on the Coronal margin of Prepuce
—- Keratoderma blenorrhagica: Waxy yellow/ brown papules on palms & soles
What is Sjogren’s disease ??
Autoimmune disorders affecting Exocrine glands => Dry surface
- Primary
- Secondary to RA or other Connective Tissue disorders
- 9x more common in FEMALES
- Increased risk of LYMPHOID Malignancy (40 to 60 folds)
Features of Sjogren’s disease ??
Dry eyes: KCS
Dry mouth, Vaginal dryness
Arthralgia
Raynaud’s, Myalgia
Sensory Polyneuropathy
Recurrent episodes of Parotitis
Subclinical RTA
Ix. of Sjogren’s disease ??
- RF (+)ve in 50% pts.
- ANA (+)ve in 70% cases
- Anti-Ro (SSA) antibodies (70%)
- Anti-La (SSB) antibodies (30%)
- Schirmer’s teat
- Histology: Focal Lymphocytic Infiltration
- Hypergammaglobulinaemia, Low C4
Features of Still’s disease in Adults ??
Bimodal age distribution- 15 to 25 yrs & 35 to 46 yrs
- Arthralgia
- Serum FERRITIN elevated
- Rash: Salmon-pink, Maculopapular
- Pyrexia: Late afternoon/ Early evening in a daily pattern + worsening Joint symptoms & Rash
- Lymphadenopathy
- RF & ANA are (-)ve
Dx. & Rx. of Still’s disease ??
Yamaguchi Criteria most widely used
NSAIDs
- 1st line for Fever, Joint pain & Serositis
- Trialled for at least a week before Steroids are added
STEROIDS
- Control symptom but do NOT improve prognosis
- If symptoms persists, use MTX, IL-1 or anti-TNF therapy.
What is Systemic Sclerosis (SSc) ??
Hardened, sclerotic skin & other connective tissue
4x more common in Females
3 patterns of disease
- Limited Cutaneous SSc
- Diffuse Cutaneous SSc
- Scleroderma (Without Internal Organ Involvement): Tightening + Fibrosis of Skin; manifests as Plaques (Morphea) or Linear
Features of Limited Cutaneous SSc ??
- Raynaud’s may be 1st sign
- FACE & DISTAL Limb predominantly
- a/w Anti-Centromere antibodies & ANA (+)ve
- CREST syndrome is a subtype (Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia)
Features of Diffuse Cutaneous SSc ??
- TRUNK & PROXIMAL Limbs Predominantly
- a/w Scl-70 antibodies & ANA (+)ve
- MCC of death is due to Resp. Complication- ILD & PAH
- Renal disease & HTN
- Poor prognosis
