ZVORALIVE ZVORALIVE

34-year-old male patient presents with long standing pain abdomen with
associated significant weight loss. On examination, his temperature is 98.2 F,
HR 74/min, RR 23/min. Rheumatoid factor is positive. There are diffuse
swellings in the bilateral cheek and neck. CECT abdomen is shown. What is the
diagnosis?

● a) Rheumatoid arthritis
● b) SLE
● c) IgG4 related disease
● d) Chronic pancreatitis
 IgG4-RD

System Manifestations

Orbit

Salivary gland

Thyroid

CNS

Retroperitoneum

Pancreas

Kidney

Biliary system
 Pathology

Lympho-
plasmacytic Storiform Obliterative
fibrosis phlebitis
infiltrates
 AIP- Pathology

● Type 1- ● Type2

○ Lymphoplasmacytic Sclerosing Pancreatitis (LPSP) ○ Idiopathic Duct Centric Pancreatitis (IDCP)

○ Elevated IGg4

○ Extra pancreatic involvement ○ Normal IGg4

○ MC in Eastern world ○ No extrapancreatic involvement except IBD

○ MC in Western world

www.pancreapedia.org/reviews/histology-of-autoimmune-pancreatitis
-Young + Morning stiffness
-SPARING:
-MC:
-HLA DR4, smoking
Extra-articular MC:
NOT seen-
R/o malignancy-
MCC death-
1st line Rx -
Elderly
-Young + INFLAMMATORY LBA
-IOC/ Most sn-
VALVULAR lesion-
1st line Rx -
-Young
-Skin lesions
-Dactylitis
-DIP mc
-Young
-Skin lesions
Hook like metacarpals
A 55-yr old male presents to you with acute
right knee pain. Xray is shown to you. What
is the likely diagnosis?

● a) Gout
● b) CPPD deposition disease
● c) Septic arthritis
● d) Psoriatic arthritis
 Gout Pseudogout

• Mono-arthritis
• Acute
• Synovial aspiration-Inflammatory

Joint affected

Synovial
aspiration

Imaging
GIANT CELL ARTERITIS

LARGE
GRANULOMA

F
1) >50ys
2) New onset headache
3) Temporal artery tenderness
4) ESR >50mm/h
5) Abnormal biopsy
Small vessel vasculitis-LUNG
Churg-Strauss
EFPA

SMALL
P-ANCA +
GRANULOMA
F
ANY 4:
1)Asthma (MC)
2) Eosinophilia
3) MM
4) Fleeting pulmonary opacities
5) Sinusitis
6) Bx-Extravascular granuloma,
eosinophils
 GIANT CELL TAKAYASU PAN WEGENER’s MPA Churg-Strauss
ARTERITIS ARTERITIS GPA EGPA
TYPE LARGE LARGE MEDIUM SMALL SMALL SMALL
PAT GRANULOMA GRANULOMA IC MEDIATED C-ANCA + P-ANCA P-ANCA +
H GRANULOMA GRANULOMA
MC F F F M=F M F
1) >50ys 1) <40yrs 1) HBV 1. AbN U/A 1) RPGN ANY 4:
2) New onset 2) Claudication 2) Mononeuritis 2. Sinusitis 2) DPH 1)Asthma (MC)
headache 3) Decreased multiplex 3. Airway stenosis 3) Bx- 2) Eosinophilia
3) Temporal brachial 3) Testicular pain 4. C-ANCA Necrotising 3) MM
artery pulse 4) DBP > 90 5. Abnormal vasculitis 4) Fleeting pulmonary
tenderness 4) BP >10mm 5) Raised U/Cr CXR/CT 4) p-ANCA + opacities
4) ESR difference 6) Livedo 6. Bx- 5) Purpura, GI 5) Sinusitis
>50mm/h 5) Bruit in reticularis Granulomatous bleed 6) Bx-Extravascular
5) Abnormal SCA/Ao 7) Wt Loss>4kg inflammation granuloma, eosinophils
biopsy 6) Abnormal 8) Myalgia
angiogram 9) Angiographic
Anb
10) Bx
Q. Diagnosis?

● a) SLE
● b) Behcet’s disease
● c) Crohn’s disease
● d) IgG4-RD
Behcet’s disease

● HLA B*51
● Neutrophilic vasculitis
● Criteria:
Recurrent oral ulceration –MC : painful, shallow +
1. Recurrent genital ulcers
2. Skin lesions
3. Ocular – Panuveitis, scarring
4. Positive Pathergy

o venous thrombosis
o PA aneurysm