Rheumatology Flashcards
Behcets disease sx
oral ulcers- MOST COMMON and recurrent
genital ulcers
polyarthralgia
uveitis
erythema nodosum or acneform lesions
migratory thrombophlebitis
vasculitis
GI sx: abdominal pain and diarrhea
Enteropathic arthralgia
Commonly seen with IBD (UC/ and more commonly with CD)
acute oligoarthritis
mouth ulcers
erythema nodosum
iritis
Reactive Arthritis
Preceding URTI
arthritis
conjunctivitis
uveitis
urethritis
Behcets Disease: RF and patho
epidemiology: mediterranean + east asia: TURKEY, ISRAELI, JAPAN
HLA B51
patho: AI/anti-inflammatory dx due to neutrophil hyperactivity
Behcets diagnosis
Pathery testing: SC prick forms induration in skin within 48 hrs
RF, ANA and ANCA NEGATIVE
HLAB51 POSTIVE
CSF: lymphocytosis or polymorphonuclear cels
MRI: white matter changes
Tx of Behcets dx
oral:
1. topical corticosteroids like triamcinolone
2. colchicine, prednisolone, azt
3. anti-TNF alpha like infliximab
eye: pred + azt or pred + infliximab
gi: pred + infliximab
CNS: pred+ infliximab
vascular: pred + cyclosporine
Retroperitoneal Fibrosis
multifocal fibrosclerotic syndrome including mediastinal fibrosis, sclerosing cholangitis and riedel thydoriditis
males > females
50-60 years old
promontary of sacrum upto L2
sx: back pain, weight loss, fever
dx: intravenous pyelography showing displacement towards ureters
complications: htn, vte
RF: methysergide, BB, methyldopa, hydralazine
Neonatal lupus
Anti-RO antibodies and anti-LA antibodies
usually presents with RASH which lasts 6-8 months
associated with congenital heart block - measure pulse son 18 week scan + give dexamethasone
SLE Pathophysiology
multi-systemic inflammatory connective tissue disorder
auto-immune: type 2 HS
antibodies react with antigen, form complex, attacked by neutrophils and complement system which causes inflammation leading to damage of endothelium causing small vessel vasculitis
women > men
west indian population
Drug induced lupus
men> women
resolves when drugs stopped
drugs causing it: procrainamde, isoniazid, hydralazine - active amino grou p
CNS and renal disease are rare
ANA +
antibodies to dsDNA -
Hughes syndrome
anti-phospholipid antibody syndrome
sx:
- recurrent VTE and Arterial throbosis
- recurrent fetal loss
- thrombocytopenia
- liban sacks endocarditis
- focal neurology
- TIA
Symptoms of SLE
arthralgia or non erosive arthritis
rash: dicoid lupus erythemtousos- scaly red plaques with follicular plugging on sun-exposed sites which heal with scarring, malar rash,
fever
pericarditis, pleurisy,
renal: proteinuria, nephrotic syndrome, glomeruloneprhritis
neuro/psych: psychosis, seizures
heam: leukopenia, thrombocytopenia, haemloysis
alopecia
raynauds
resp: pneumonitiis
cardiac: libman sacks
Dx of SLE
Hx and physical exam showing clinical features
FBC: anemia of chronic dx with normal mcv, low platelets, low neutrophils, low wcc
serology: ANA +, anti-dsDNA antiboodies, low complment levels, anti-Sm antibodies, anti-ro/la and anti RNP antibodies, anti-phosphoslipid antibodies
CRP
low C3 and Low C4- lupus nephritis
Mx of SLE
symptom relief: sunblock, analgesia
supress immune system: DMARDS like hydroxychloroquine, analgesia, mtx, azt, plasma exchange, rituximab
PAN
systemic necrotising vasculities affecting medium artering and cases aneyrysm formation
PAN epidemiology
Middle aged men, associated with HEP B and
PAN aetiology
Idiopathic
Triggered by viral infections like HEP B and HEP C
PAN pathophysiology
Not fully understood
Immune complexes deposit on arteries, cause inflammation, leading to aneurysm formation and thrombosis which leads to ischemia to organs
PAN symx
General: malaise, fever, weight loss
Skin: livedo reticularis, nodules, purpura, necrotic ulcers,
Cardio: MI, pericarditis, HTN
GI: post-prandial ischemia causing abdo pain, bowel perforation
renal: CKD, hematuria, proteinuria- MOST COMMON ORGAN
neuro: stroke, mononeuritis multiplex
DDx of PAN
ESR, CRP, ANCA negative, anemia, leukocytosis, HbsAG +
Biopsy: necrotising inflmmation and fibrinoid necrosis
imaging: angio- anuenyrusms –> ROSARY sign
Tx of PAN
steroids, azt, cyclophosphamide, anti-viral for Hep B, Ace-I or ARB
Granulmatosis with polyangitis
Upper and Lower respiratory tract involcement- sinuses and lungs
Thalassemia
genetic disease causing low hemogobin syntehsis due to decrease or no synthesis of alpha/beta globin chains leading to hemolytic anemia
Epidemiology of thalassemia
mediterranea, middle east or fast east
ALPHA- indian, south east asia, africa
BETA- china, south east asia, middle east, mediterranea
