Rheuma - Lecture 7

Adult Still’s Disease (ADS) is a rare, systemic, inflammatory, autoimmune disorder characterised by

• polyarthritis

• high fevers

• salmon coloured evanescent rash

• auto inflammatory sds (familial mediterranean fever, muckle wells sd → amyloidosis, malarial fever, urticarial rash)

epidemio still’s disease

bimodal peak at 15-25 and 36-46

triggers Still’s disease

rubella, EBV, cytomegalovirus, parvovirus, cocksakievirus b4

fever effects of still’s disease

sudden onset, remittent, mimics septic pattern, >39 degrees, lasts under 4h

rash characteristics Still’s disease

small salmon-pink maculopapular eruption on trunk and extremitis

dissapears at night, coincides with fever spike

features of arthritis and myalgias in still’s disease

may be absent in early stage, mild oligo-articular, transient

myalgia - severe, CK and aldolase elevated

what is the inital symptom of still’s disease 

pharyngitis

general manifestations still’s disease

hepatitis, reactive hemophagocytic sd (pancytopenia), splenomegaly, cervical nodes, pleural effusion, pericarditis

clinical course still’s disease

chronic articular pattern

self-limiting/monophasic pattern

intermittent/polycyclic systemic pattern

rash, polyarthritis and shoulder + hip involvement are predictors of ________ in still’s disease

chronic disease

lab findings still’s disease

high ESR, CRP, Leu, predominance granulocytes

normo anemia, reactive thrombocytosis

intrasv coag

high transaminases, lactate dehydrogenase, Bi

what lab value to monitor still’s disease activity

ferritin (elevated)

imagistic findings still’s disease

joint space narrow, erosions, rapid destruction hip/knee

still’s disease - Yamaguchi classification criteria - 5 or more, 2 major of

major - fever, intermittent >1w, arthralgias >2w, rash, leukocytosis 80% granulocytes

minor - pharyngitis, hepato/splenomegaly, elevated liver enzymes + LDH, onset lymphadenopathy, neg RF + ANA

still’s disease ttt

NSAIDs and aspirin for fever and arthritis

steroids

immunomodulators - MTX, SLZ, CYC

ttt chronic pattern still’s disease

IV Igs

what differentiates still’s disease from lupus, vasculities or malignancies

primary renal involvement

sarcoidosis

systemic inflammatory disorder characterized by presence of noncaseating granulomatous inflammation in organs

epidemio sarcoidosis

black/ northern european women 20-40y

infectious agents as a cause of sarcoidosis

TB, propionibacterium acnes, herpes

histologically what does the granuloma consist of in sarcoidosis

central - epitheloid cells, activated monocyte-macrophages, multinucleated giant cells

periphery - suppressor CD8+ T cells

manifestations pul involvement in sarcoidosis

hilar adenopathy, cough, dyspnea, chest pain

self-limiting, spontaneous remission

cutaneous involvement sarcoidosis

erythema nodosum, subcutaneous nodules, lupus pernio

ocular involvement sarcoidosis

uveitis, optic neuritis, pars planitis

extra-thoracic manifestations sarcoidosis

arthralgia, tenosynovitis myopathies

unilat facial n palsy

pericarditis, arrhythmias

hepatomegaly

DM

vasculitis

Lofgren’s sd (of sarcoidosis) - epidemio

white women

Lofgren’s sd in sarcoidosis - manifestations

hilar adenopathy, acute arthritis in ankles and knees, erythema nodosum

fever, myalgia, weight loss

ttt lofgen’s sd in sarcoidosis + evolution

excellent response to steroids

types of m involvement in sarcoidosis

chronic/acute or nodular myopathy

heefordt’s sd in sarcoidosis - manifestations

ant uveitis, parotid gland enlarged, facial palsy, fever

lab values sarcoidosis

elevated ESR, hypergammaglobulinemia, anemia

leukopenia, lymphooenia, eosinophilia

alkaline phosphate elevated if hepatic involvement

ACE elevation in sarcoidosis

in acute, leads to hypercalciuria and hyperCa+

chest imaging findings sarcoidosis - stages

0. normal

1. bilat hilar adenopathy

2. bilat hilar adenopathy w/ pul infiltrates

3. pul infiltrates w/ lung insufficiency

CT imaging findings in sarcoidosis

hilar and mediastinal lymphadenopathy, nodules, fibrosis, ground glass, cysts, parenchymal masses/bands

findings on bone radio in sarcoidosis

cystic bone lesions, lytic bone lesions on heads, osteoporosis, some scllerotic lesions

panda sign

pul fxn tests in sarcoidosis

restrictive pattern

if endobronchial sarcoidosis - obstructive

bronchoalveolar lavage in sarcoidosis - findings

high CD4:CD8 ratio

dg sarcoidosis

clinical and imaging

biopsy - non caseating granuloma

ttt pul involvement sarcoidosis

steroids, azathioprine, cyclophosphamide, cyclosporine, transplant

ttt extrapul involvement sarcoidosis

steroids, mtx, colchicine, antimalars

ttt cutaneous disease in sarcoidosis

azathioprine

ttt cardia

Behcet’s disease is a systemic inflammation characterized by

oral aphthae, genital ulcers, hypopyon uveitis

epidemio behcet’s disease

onset in 30s, middle and far east

HLA association with behcet’s disease

HLA B51

behcet’s disease skin and mucosal manifesrtations

oral ulcers - first 

genital ulcers

erythema nodosum

superficial thrombophlebitis

Sweet’s sd is a skin manifestation in behcet’s disease characterized by

fever and painful skin lesions on arms neck face and back

eye symptoms behcet’s disease

hypopyon, acute bilat ant uveitis, pan-uveitis, blindness

neuro involvement behcet’s disease

parenchymal (more common) - spinal cord lesions, hemispheric involvement

non-parenchymal - dural sinus thrombosis

vessel involvement behcet’s disease

DVT, arteritis, microaneurysms

pulmonary involvement behcet’s disease

hemoptysis associated w/ pul a aneurysm

pul a occlusion

GI involvement behcet’s disease

severe abd pain, ileocecal ulcers

joint involvement behcet’s disease

usually mono arthritis

oligo/polyarticular - symmetrical (usually knees), intermittent - resolves in a month or chronic

lab findings behcet’s disease

anemia, neutrophilic Leu, esr, crp

CSF protein high and NEU and LEU in behcet’s disease is a _______ prognostic sign

poor

marker of behcet’s disease if GI involvement

Anti-Saccharomyces
Cerevisiae Antibodies (ASCA)

what imaging for which involvement behcet

MRI - neuro

CT - Gi and pul

xray - cardiopul

doppler US - vasc

ttt skin and mucosal involvement behcet

steroids, colchicine in genital ulcers and erythema nodosum

systemic - azathioprine

ttt eye symptoms behcet

azathioprine, cyclosporine A

ttt neuro involvment behcet

cyclosporine A, steroids, MTX

major vessel involvement in behcet - ttt

monthly pulses cyclophosphamide + prednisolone

ttt cardiac involvement behcet

steroids

ttt gi involvement behcet

thalidomide, tnf a blockers

tttjoint involvement behcet

colchicine, prednisolone