Rheum Long Flashcards
Scleroderma Hx
- Derm
- Raynauds, skin tightening - Arthritis
- Rheumatoid distribution, Carpel tunnel - GI
- Dysphagia, reflux, diarrhea - Renal
- HTN, CKD, SRC - Resp
- ILD, PHTN, pleurisy - Cardiac
- Pericarditis, arrhthymias, dilated cardiomyopathy -CCF - Other
- Erectile dysfunction, hypothyroid, non melanoma skin cancers
DDX of Scleroderma
-Non associated with Raynauds or ANA
Eosinophilic fascitis
Morphea
Nephrogenic systemic fibrosis
Diabetic cheiroarthopathy
CREST
Calcinosis (deposits in subcut tissue at ends of fingers) Raynaud's Esophageal involvement Sclerodactyly Telangiectasia
Ix for Systemic Sclerosis
- Basic Bloods:
- FBE (Anaemia), ESR, Folate and B12 (malabsorption) - Special Bloods:
- Ig - (Hyper IgG in 50%), ANA, Anti-Scl 70 (diffuse), Anti centromere (limited) - Imaging
- CXR, HRCT - Special tests:
- Gscope and oesophageal manometry
- PFTs
- TTE
- RHC
Tx for Systemic Sclerosis
- Reflux - PPI
- Raynauds - CCB, Prazocin, methyldopa, iloprost
- Dry eyes
- Malabsorption (ensure no SIBO), supplements
- ILD - Cyclophosphamide
- PHTN - Endothelin recepton antagonist, phosphodiesterase inhibitors, Prostanoids
- HTN - ACEi
Extra articular manifestations of RA
- Skin - Raynauds, leg ulcers
- Eyes - Dry eyes, scleritis, episcleritis, scleromalacia perforans, cataracts
- Sore throat, horeseness, neck pain 0 Suggests cricoaryteroid disease
- Recurrent headaches at base of skull and arm tingling - thinck C1/2 sublaxation - Lungs - ILD, pleural effusion, pleuritis
- Cardiac - Pericarditis, valvular disease atherosclerosis
- Renal - Drug use, amyloid
- CNS - PN, mononeuritis multiplex, cord compression, entrapment neuropathy
- Haeme - Anaemia, Felty’s (RA, leukopenia, splenomegaly)
- Systemic - fever, weight loss, fatigue
- Vasculitic - digital arteritis, ulcers, PG
MTX Side effects
Hepatic and pulmonary toxicity
Leukopenia
Thrombocytopenia
SSZ Side effects
Rash Nausea Haematological abnormalities Abnormal LFTs Reversible oligospermia
HCQ Side effects
Nausea
Pigmentation
Bull’s eye retinopathy
LEF Side effects
Diarrhea
Alopecia
Derranged LFTs
Causes of Arthritis and Nodules
RA (Seropositive)
SLE
Rheumatic fever (Jacoud’s arthritis)
Amyloid Arthropathy (usually associated with MM)
DDX for deforming symmetrical chronic polyarthropathy
RA PSoriatic arthritis or seronegative arthropathy Chronic tophaceous gout Primary generalised OA SLE
Investigations to assess activity of RA
ESR or CRP
Hb - severity of normocytic anaemia correlates with activity
Anto CCP and RF titres
PResence of progressive erosions on serial XRs
DDx for raised ESR in RA
Active disease
Sjogrens
Amyloidosis
Infection
Tx of RA
General Principles
- Education
- PT - exercise and splinting of joints to prevent deformity
- OT
- Smoking cessation
- Rest of inflamed joints
- Anti inflammatories
- CVS risk modification
Induce Remission
-DMARDS or biologics
Acute flare Mx
-Steroids, PO or intraarticular
Surgery
- Joint replacements - Hip, shoulder, knee
- Arthroplasty and relief of contractures
Risk factors for destructive disease in RA
High titre RF or positive anti CCP Constitutional Sx Insidious onset Erosions early on XR Rheumatoid nodules early HLA DR4
Rules for the use of biologics for RA
- Failure of at least 6 months of treatment with traditional DMARDs
- Tx must include MTX and combinations of HCQ, SSZ, or LEF
Monitoring for disease activity during review of RA patient
Fatigue Morning stiffness Weight loss Functional limitations Acute phase reactants (ESR and CRP)
OA MX
Non pharm
- Exercise: Stretching and mobility exercises to help maintain ROM; Aquatic exercise; exercise bike; Supervised or group exercise is better for reduction in pain
- Gait aids: SPS, knee braces, foot orthoses
- LoW: most important modifiable RF
- Complimentary meds: Glucosamine, fish oil, chondroitin equal to placebo
Pharm
- NSAIDS/Paracetamol
- Topical NSAID/Capsaicin
- Intraarticular steroids for acute flare
- Opioids topical or oral (increased risk of CVS, fractures, and mortality compared to NSAIDs)
- Duloxetine - superior than placebo
Surgery
Extraarticular manifestations of Ank Spond
Uveitis
AR
Symptoms of cauda equina (late)
Upper lobe interstitial lung disease (Late)
Diagnosis of Axial Spondyloarthritis
- Age < 45
- > 3 months of back pain
- HLA B27 positive + 2 or more of:
-Inflammatory back pain
-Enthesitis
-Uveitis
-Dactylitis
-IBD
-Family Hx of SPA or HLA B27
Elevated CRP
-REsponse to NSAIDs - Sacroilitis on XR or MRI
Mx of axial spondyloarthritis
- Exercise program for flexibility
- NSAIDs - reliev Sx and slow radiographic progression
- Anti TNF alpha if not responsive to NSAIDs
- ?Surgical options
ARA criteria for SLE
Need 4 out of 11
- Malar rash - sparing the nasolabial folds
- Discoid rash
- Photosensitivity rash
- Oral Ulcers
- Arthritis affecting 2 or more peripheral joints
- Serositis - pleurisy, pericarditis
- Renal disorder - Proteinuria > 0.5 g/day or cellular casts
- Neuro disorder - Seizures, psychosis
- Haeme - haemolytic anaemia, leukopenia, lymphopenia, thrombocytopenia
- Immunological disorder - dsDNA, anti-Sm, positive APLS ab
- ANA >1:160
SLE Hx
- General Sx: Nausea, weight loss, malaise, thrombosis
-MSK: arthralgia, arthritis, myalgia, myositis
-Derm: rash, alopecia, oral and nasal ulcers
-Fever
-Neuro psych Sx - delirium, dementia, seizures, chorea, neurpathy, stroke, headache, MS like symptoms, anxiety, depression
-Renal - haematuria, oedema, renal failure
-Resp - pleurisy
-CVS - pericarditis, myocarditis, valvular lesions, CAD
HAeme - anaemia, lymphadenopathy
-GI - diarrhea, pseudo bowel obstruction, perforation
-APLS Symptoms
-Sicca symptoms
KEy Symptoms for SLE
Apthous ulcers Serositis Raynauds Alopecia Photsensitivity rash Dry eyes and mouth Thrombosis miscarriage Nephritis
Features of MCTD
- OVerlapping features of SLE, SS, and polmyositis
- High anti U1RNP
- PErcardial effusion
- Raynaud’s, swollen hands, fatigue and arthritis
- PAH is main cause of death
Major causes of death in SLE
Infection
Renal failure
Lymphoma
AMI
Mx of SLE
General
- Sun protection and avoidance
- Rest and NSAIDs for arthralgia and myalgia
- Cease smoking
Suppressing disease activity
- HCQ (Skin and joint)
- Steroids if serious disease manifestation
- AZA/MMF/Cyclophosphamide -Renal Lupus
- MTX - Joints
- Raynaud’s: CCB
- Ritux/Belimumab - on special access scheme for some patients
Managing Disease complications
- CVS modification
- Thrombosis and hypercoagulability
- Bone health
- Routine Pap smears - risk of cervical dysplasia
General Vasculitis Sx to ask on Hx
Fatigue fever myalgia arthralgia Vasculitic skin rashes - palpable purpura Renal disease HTN GI symptoms
GPA Hx specifics
Ganulomatosis with polyangiitis
- Nasal congestion/Rhinorrhea
- Bloody nasal discharge
- Cough (initially dry, but may progress to hemoptysis)
- Breathlessness
- Renal involvement
GCA Hx specifics
- PMR Sx
- Bitemporal headache
- Visual disturbance - diplopia, visual loss
- Jaw claudication
- Scalp tenderness
PAN Hx specifics
Hep B risk factors
USually multi system involvement
-e.g. foot drop, abdo pain, chest pain
EGPA Hx specifics
- Asthma
- PEripheral eosinophilia
- Allergic rhinitis/nasal polyps
- Eczema
- Cough and breathlessness
- PNS - symmetrical PN or mononeuritis multiplex
Mixed essential cryoglobulinemia Hx specifics
Small vessels due to RF bound to IgG
- Palpable purpura
- Raynauds
- Arthritis
- Neuropathy
- Hep C is common
Levido reticularis DDx
Cholesterol atheroembolism APLS Vasculitis Atrial myxoma Bacterial endocarditis TTP
IX for vasculitis
ESR Renal function - GPA, MPA, PAN LFTs- usually derranged in GPA and PAN Urine MCS, casts, RBC morphology CXR - bilateral diffuse interstial abnormality in GPA, peripheral fluffy patchy infiltrative pattern in EGPA c-ANCA and anti PR3 antibodies- GPA p-ANCA - EGPA and consider sural nerve Bx MPA - p-ANCA and REnal Bx PAN - Angiography/Bx
Tx of GPA
High dose steroids
Cyclophosphamide
Bactrim adjunct
Ritux if above fails
PAN Mx
High dose pred
Cyclophosphamide - 90% go into remission
If associated with Hep B - Interferon alpha or antivrial vidarabine is helpful to lead to remission
EGPA MX
Steroids alone effective
MAy need other immunosuppression if not
