HLA-DR4 (mnemonic: DRive 4 Rheuma 🚗 💨 )

1. Auto-Ab (RF, ACPA[Anti-CCP] )

‣

💬

2. Complex formation + Environmental triggers

1. INFLAMMATORY INFILTRATE → fluid in the joint + bone marrow edema

2. SYNOVIAL HYPERTROPHY + PANNUS 📷

⇒ bone erosions + cartilage dmg (→narrow joint space)

‣

What are the environmental triggers?

1. 🚬
2. viruses + bacteria

female 35-50y

6weeks

(otherwise it could be an infection)

small joints (hand + feet) + later large joints (elbow, spine, knees, ankle , foot)

active joint = swollen + tender

+ systemic signs (fever + other B-sympt)

Gaenslen Sign: 📷

INFLAMMATORY PAIN

↓pain with activity

↑pain with rest (worse during night+morning)

(opposite to mechanical pain in OA)

>60min

• Ulnar deviation
• Boutonniere deformity
• Swan neck deformity
• Z shaped thumb
• MCP subluxation

Nodules 📷→ filled with cholesterol when you biopsy them

RF/ACPA + HLA DR4 +

1. General B-symptoms
2. Derma → nodules
3. CV → various heart pathologies + vasculitis
4. Lungs
5. Eye
6. Osteoporosis
7. Hema: anemia, ↑plts
8. Neuromuscular

if location: C1, C2 → probably RA

→ Xray

Felty = RA + Neutropenia + Splenomegaly + ↓Plts 📷

Still’s = RA + ↑Np + evanescent (”salmon”) rash + fever (39°>1w)

‣

💬

⇒ Both ANA +

⇒ Felty’s RF +

⇒ Stills’s RF -

1. handicapped
1. after 2 years → mod. handicaped
2. some after 10 years → sev. handicaped
3. some during first 10y → incapable of profess. life
2. psychological disorders

• polycyclic (70%): intermittent or continuous, incomplete remission or progression
• monocyclic (20%): Significant remission in 1st year
• progressive(10%) = malignant: Continuous, generalized; poor prognosis

1. ESR + CRP
2. CBC → Anemia, ↑plts (n/↑ leukos)
3. Rheumatoid factor (RF) → If RF neg.→ its probably Ø RA
4. Anti-CCP ( ACPA)→ if CCP is pos. → its probably RA

1. erosions?
2. panus?
3. deformities?
4. periarticular osteopenia/osteoporosis?
5. narrow joint space

US

1. Arthrocentesis → inflammatory joint: Np >2k but < 50k
2. MRI → bone marrow edema (earliest sign of imflammation aka early detection of joint inflammation in RA)

Xray + US

(rarely MRI)

XRAY (+MRI)

1. Lab → ESR + CRP
2. US
3. MRI

predni + NSAID(symptomatic)

#1 line MTX → everbody

‣

💬

#2 line add-on other cDMARDS

#3 line bDMARDS (biologics) → inflixi-, rituximab 📷

#4 line tsDMARDS → Jak inhibitors

T

CV → CV drugs

osteoporosis → anti-osteoporotic drugs

Hep B +C

1. adequately vaccinated?

2. TB test?

‣

💬

3. Fungi?

→ if you downregulate the immunesysteme these things will fuck the patient up

• infections
• herpes zoster

• thromboembolism (if CV-RF)

‣

💬

can be assoc. with lupus

→ thrombosis

→ fetal loss

→ thrombocytopenia

→ anti-phospholipid Ab (anti-cadiolipin, lupus anticoagulant)

→ (livedo reticularis)

“SOAPBRAIN MD” 📷

‣

Besides serositis of lung + heart: what can be the cardiac + pulmo. manifestations? (2+2)

‣

Which joints are affected in lupus? How are the joints affected?

💡

Jaccoud's arthropathy 📷 is a chronic, non erosive, rheumatoid-like deformity of the hands associated with rheumatic fever and systemic lupus erythematosus (SLE). This deforming arthropathy may present difficulties in differentiating SLE from rheumatoid arthritis (RA)!

‣

Neurological symptoms (esp. seizures) are associated with what antibody?

anti-phospholipid

‣

What is NPSLE?

👉🏽

🧠😵‍💫 neuropsychiatric systemic lupus erythematosus

1. CNS: Aseptic meningitis, Cerebrovascular disease, Demyelinating syndrome, Headache (including migraine and benign intracranial hypertension), Movement disorder (chorea), Myelopathy, Seizure disorder, Acute confusional state, Anxiety disorder, Cognitive dysfunction, Mood disorder, Psychosis
2. PNS: Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome), Autonomic disorder, Mononeuropathy (single/multiplex), Myasthenia gravis, Neuropathy (cranial), Plexopathy, Polyneuropathy

‣

What do you have to do when smb has neurological symptoms?

check anti-phoslipid Ab + Neuro-workup

‣

With what skin test can you DDx SLE vs. cutaneous lupus?

👉🏽

lupus band test 📷

‣

💬

• The lupus band test is performed on a skin biopsy using direct immunofluorescence staining.
• It helps to distinguish between systemic lupus erythematosus (SLE) and cutaneous lupus.
• In SLE, the lupus band test is positive in both involved and uninvolved skin.
• In cutaneous lupus, only the involved skin shows a positive result.
• The test detects the deposition of IgG and complement at the dermoepidermal junction.

1. Infections
2. CNS vasculitis
3. Artherosclerosis → CV-events
1. Kidney failure

yes

(but classic for scleroderma (systemic sclerosis) + mixed connective tissue disorders)

Follow up: Complement + dsDNA

Renal activity: Protein-/Hematuria

kidney biopsy:

≥ 4 criteria of MD SOAP BRAIN 📷 → at least 1 clinical + 1 Antibody (immunological)

(+lifestyle measures in general + stress coping mechanism)!

(double click)

nothing 🤡

Penicillamin

PPI

1. Eating behavior + diet
2. Prokinetics
3. Recurrent diarrhea or pseudo obstruction → AB + pro-biotics

non-pharma:

• avoid cold+stress exposure
• avoid digital trauma
• Øsmoking
• stop vasoconstrictive drugs

pharma:

• #1 CCB
• PG-analogs (i.e Ilprost)

ACEi

→ refract.?→ Dialysis / kidney-transplant

• like in lupus nephritis
• induction: Cyclophosphamide
• Maintenance: AZA
• Alternative: Myocophenolate mofetil
• bDMARDS: Tocilizumab (Anti-IL6)
• Anti-fibrotics (TK-inhibitor: Nintedanib)

• Bosetarn + viagra
• Iloprost

(like digital ulcers)

• 📜 rapid progressive cutaneous
• 🫁 Interstitial lung diseases
• 💪🏽 joint/muscle inflamm.
• 🫀heart inflamm.

• cDMARDS = classic

= same as #2line lupus/lupusnephritis

1. Cyclophosphamide, AZA, Mycophenolate
2. MTX + Cyclosporin A
• bDMARDS
• Tocilizumab (interstital lung D)
• Rituximab (others)

occular muscles

PM/DM + 🫁 Interstitial lung diease

1. MSA (Myositis-specific-Ab):
1. Antisynthetase Ab → Anti-Jo + Anti-Mi
2. Anti-SRP
2. MAA (Myositis-assoc.-Ab):
1. ANA (i.e. anti-PM-Scl)

• clinic (ILD + PM/DM)
‣

What specific skin manifestation is assoc. with antisynthetase syndrome?

👨‍🔧 mechanic’s hands: 📷 

• check anti-Jo
• high resolution CT
• ground glass 📷

• Past radiation + HepC → dry eyes + mouth
• Diabetes → dry mouth + parotid

1. red eye
2. ulceration
3. perforation → dry keratoconjunctivitis 📷

• oral candidiasis
• dental: caries + peridontitis

degenerative disease → focal cartilage loss

→ bone erosions

→ bone repair → osteophytes

T

1. hand → CMC1, PIP + DIP (MCP might also be involved esp MCP2+3)
2. spine
3. hip
4. knee

1. swollen + tender joint
2. mechanical pain (worse with activity, better w/ rest)
3. restricted movement
1. morning stiffness <30min
2. deformities (i.e. Heberden + Bouchard nodules)
4. Øextra-articular manifestations + Øsystemic inflamm. signs ‼️

1. radiation towards medial thigh above the knee
2. ↓int. rot + adduction (cant bring it in)

1. episodic inflammation
2. Baker cyst
3. muscle hypotrophy (quadrizeps)
4. deformation + instability

Direct Dgx of OA =

pain + morn. stiffness <30m + funct. limitation + 1 more typical

Xray

‣

What are Xray findings in OA? (4)

1. Joint space narrowing
2. Subchondral sclerosis
3. subchondral cysts
4. Osteophytes

• >65y?
• Comorbidities?
• Steroids?
• History ulcer/bleeding?

KIDNEY!

T

(difference to the others!)

1. Suspicion of cancer / inflamm / trauma?
1. age: <20 or >50y
2. B-symptoms (cancer, inflammatory)
1. noctural (inflammatory + cancer)

→ activity → ↓pain in inflammatory but not in cancer

3. PMH
1. cancer, osteoporsis, steroids
2. trauma/recent infection
2. Periph neuro def. (i.e paresthesia, motor deficit, radiation)

<6w

(also Ø RF + ANA check)

2-4 joints

asymetrical

1. Common variable back pain
2. OA
3. discprolapse
4. SpA

PAIR

1. Psoriatric arthritis
2. Ankylosing spondylitis

3. IBD associated ankylosing spondylitis/SpA

‣

💬

4. Reactive arthritis
5. Juvenile SpA

only ankylosing spondylitis + IBD assoc. = symmetrical

rest = assymetrical

mainly reactive + psoriatic (+juvenile)

You can copy and paste this into a word processor to maintain the table structure. If you need the table to be formatted in a specific way or to include additional data, please provide the details.

all of them can have peripheral features

• Peripheral arthritis
• Enthesitis 📷 (tendon inflammation)
• Dactylitis 📷 (fingers / toes)

T

(inflammation of the whole digit)

psoriatric + reactive

(can involve hands or feet → “sausage-like digits”)

all of them 🤡

inflammatory joint → pain+swelling & stiffness>60min

assymetrical → esp in lower limbs: knee, ankles, digits 📷

⇒ deposition + IBD-relation

None 🤡 (no RF, no ANA)

HLA-B27 esp. + in ankylosing spondylitis

→ familiar!

→ there are also other markers: IL23R + ERAP1

• pressure (ant-post + lateral)

• Schober test 📷

‣

💬

+Ddx workup (non rheumatologic disease → common varibale back pain, OA, disc proloapse etc ⇒ look for clinic, red flags + MRI if necc.)

+Lab: Inflammatory markers (CRP/ESR)

• sacroillitis 📷→ assymetric vs. symmetrical

can be negative → non-radiographic axial SpA

• osteoitis → shiny corners + squaring of vertebra 📷
• syndesmophytes → bamboo spine 📷 📷

MRT → early detection +active → bone marrow edema in osteitis

CT → chronic erosions + sclerosis

Definite ankylosing spondylitis: If the radiological criterion is associated with at least 1 clinical criterion

peripheral skeletal arthritis (not in axial skeleton aka spine)

• Treatment for SpA must be personalized based on subtype, joint affectation (peripheral or axial), presence of extra-articular disease, and comorbidities.
• AxSpA: NSAIDs and biologic agents (TNFi and IL-17i).
• Peripheral SpA: NSAIDs, corticosteroids, cDMARDs (MTX, LEF, SSZ), biologics (TNFi, IL-17i, IL-12/23i), JAKi.

• Urogenital: Chlamydia trachomatis, Ureaplasma urealyticum
• Enterogenic: Salmonella, Shigella, Yersinia, Campylobacter, Clostridium

ØNSAIDS! + Tx IBD → with sulfasalazin → infliximab

early inflammatory → pseudoinfective signs+symptoms (non-specific) + self-limited

stenosis + aneurysm 📷 📷

US + PET

Hep B

complex mediated → focal segmental necrotizing vasculitis → Aneurysm (+thrombosis)

Alternating areas of necrotic vasculitis + fibrotic lesion (healed) ⇒ typical imaging appearance(see below)

all organs can be affects → NEVER LUNGS!!

most common:

🥐 renal artery

🍤 mesenterial artery

🌕 nerves

1. Renal artery → HT (hematuria, oliguria, RF)
2. mesenteric → GI pain + melena
3. nerves → periph. neuropathy ⇒ MONONEURITIS MULTIPLEX 📷
‣

💬

Mononeuritis multiplex: painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy

4. +SKIN LESIONS
‣
What are the skin leasion in PAN?
1. 🕸️ Livedo reticularis (recamosa) 📷
2. 🟥 Purpura + Echymosis 📷
3. 🧈 Paniculitis (erythema nodosum) 📷
4. 😙 Kussmaul nodules 📷
5. infarction fingertips + ulcerations 📷
5. coronary arteries → MI
6. Genitals → orchitis
7. Arthralgia/Myalgia

→ serology for surface Ag + (HBsAg)

ANCA/ANA negative

angigraphy (like takayasu)

‣

What is the most important angiographic sign of PN?

string + pearls of renal artery

‣

💬

BIOPSY

EMG / ENG → for neuro-muscular evaluation

• Induction: cyclophosphamide + steroids
• Maintenence: AZA/ Myophenolate (+steroids?)
• Anti-HepB → Entecavir
• Organ specific Tx

• Uncontrolled BP
• Anuria (RF)

Asian <5y

CRASH and BURN on a KAWASAKI

1. Conjunvtivits (bilat)
2. Rash: Polymorphous non-vesicular
3. Adenopathy (cervical)
4. Strawberry tongue + cracked red lips
5. Hand+foot: red palms + soles
6. Burn: Fever >5d

⇒ 📷

CORONARIES!!

→ thrombosis → MI

→ Aneurysm → rupture

→ other 🫀-pathos: Myocarditis, Pericarditis, valvulopathy, arrythmias

ASS + IVIg

‣

💬

ASS:

⇒ if i.v.Ig fails → Inflixmab, i.v. steroids, plasmapheresis

⇒ if multiple/large coronary aneurysm → anticoagulation

“C-disease”*

1. Granulomatosis with polyangitis (GPA=Wegeners granulomatosis)
2. Microscopic polyangitis (MPA)
3. Eosinophilic granulomatosis with polyangitis (EGPA=Churg-Strauss syndrome)

Asthma! (+ periph. eosinophilia)→ adult on-set