PATHOLOGY, SECOND MBBS, MEDICAL COUNCIL OF INDIA, COMPETENCY BASED UNDERGRADUATE CURRICULUM FOR THE INDIAN MEDICAL GRADUATE, 2018

1. PA9.7
DEFINE & DESCRIBE THE
PATHOGENESIS OF OTHER
AUTOIMMUNE DISEASES
Dr IRA BHARADWAJ
MCI TEACHER ID: PAT 2300569
KUHS FACULTY ID: M21512

2. TEXT BOOK REFRENCES
• ROBBINS BASIC PATHOLOGY
• HARSH MOHAN TEXTBOOK OF PATHOLOGY
• DAVIDSON’S PRINCIPLES AND PRACTISE OF MEDICINE
• OTHER STANDARD REFRENCES

3. SLO
• SJOGREN SYNDROME
• SCLERODERMA
• SOME OTHER AUTOIMMUNE DISEASES
[to be discussed in systemic pathology]
• QUESTIONS ON AUTOIMMUNITY

4. SJOGREN SYNDROME
Sjogren syndrome is defined as Chronic inflammation of salivary &
lacrimal glands due to autoimmune causes
• TYPES
• Secondary – associated with other autoimmune diseases
• Primary due to Autoantibodies – ANA---
• Anti ribonucleoprotein A (ARnA), SS-A, SS-B [Ro – antibody]
• Anti DNA Antibody,
• Anti HISTONE Antibody,

5. SJOGREN SYNDROME
OTHER ETIOLOGICAL FACTORS
• HLA association is weak,
• Viral infections- role of EBV, HCV, HTLV ?
MORPHOLOGY
• Glands show Inflammation, necrosis, fibrosis
CLINICAL FEATURES
• Dry eyes & mouth
• COMPLICATIONS
• Lymphoma in 40%

6. SCLERODERMA
DEFINITION
• Abnormal accumulation of fibrous tissue (sclerosis) in skin &
other organs due to autoimmunity
TYPES
• diffuse – affects multiple systems
• limited – affects only skin of fingers, forearm & face

7. SCLERODERMA
ETIOPATHOGENESIS
Role of cellular immunity: activation against unknown antigen,
causes:
• Injury to endothelial cells lining the BV & activation of
endothelial cells, leading to
• Increase in adhesion molecules &
• Migration of activated CD4+ TL in perivascular areas.

8. SCLERODERMA
• Activated CD4+T (Th2 subset) cells produce cytokines –
(PDGF, TGF & IL-13 ) which activate macrophages & increase
collagen deposition & fibrosis.
• Repeated damage of vascular endothelium leads to
endothelial proliferation & fibrosis, which narrows the
vascular lumen, resulting in ischemia

9. SCLERODERMA
Role of humoral immunity: production of anti nuclear
antibodies [ANA]
• anti-Sci 70 against DNA topoisomerase is seen specifically in
association with pulmonary fibrosis & peripheral vascular
disease
• anticentromere antibody seen in CREST syndrome

10. SCLERODERMA
ETIOPATHOGENESIS

11. SCLERODERMA
MORPHOLOGY
Skin shows the following classical changes:
• Lesion begins in fingers & extends proximally
• Microscopy shows epidermal atrophy, loss of rete pegs,
atrophy of dermal appendages & dermal fibrosis, with
thickened & hyalinized blood vessels surrounded by CD4+T
cells
• Ulceration & auto amputation of terminal phalanges may
occur
Joints, GIT, kidney, lungs, CVS show features of inflammation
& fibrosis

12. SCLERODERMA
CLINICAL FEATURES
Local symptoms
• Tightness & tethering of skin starting in fingers,
• Finger movement is impaired,
• Fingers become tapered & clawed
• Lesions progress to arm, &
• Face has masked appearance &
• Mouth is small
Systemic symptoms depend on the organs affected

13. SCLERODERMA
CREST SYNDROME
• Calcinosis cutis [deposition of calcium nodules in skin]
• Raynaud's phenomena (white-blue -red coloration of terminal digits,
especially of fingers, in response to stress or cold, due to
vasoconstriction & cyanosis, followed by reactive vasodilatation as
the digits rewarm)
• Esophageal dyspepsia / dysmotility,
• Sclerodactyly [scleroderma affecting the skin of fingers only]
• Telangiectasia [dilated capillaries on the skin of face, palmar surface
of hands & mucous membranes]
• DIAGNOSIS – skin biopsy, anti centromere antibodies

14. SOME OTHER AUTOIMMUNE DISEASES
to be discussed in systemic pathology
• RHEUMATOID ARTHRITIS – DISEASE OF JOINTS
• HASHIMOTO THYROIDITIS & GRAVES DISEASE – DISEASE OF
THYROID
• POLYARTERITIS NODOSA – VASCULAR DISEASE
• ATROPHIC GASTRITIS & PERNICIOUS ANEMIA – DISEASES OF GIT
& HEMATOLOGY

15. SHORT ESSAY – CLINICAL CASE
A 26 yr old female complained of malar skin rash, pain in
hands & wrist, and fever off & on for 2 yrs. Lab reports are as
follows : Hb – 9 gm%, MCV – 90 fl, MCH – 30 pg, TLC – 3000
cells / cu mm, platelets – 1 L/cu mm, urine examination
reveals proteinuria & hematuria. Ans the following:
• What is your diagnosis & why?
• Discuss the etiopathogenesis
• Describe the morphological changes in the kidneys
• Name one confirmatory test

16. MCQ
All of the following are autoimmune disease EXCEPT
a) SLE b) Grave’s disease c) Myasthenia gravis
d) Sickle cell anemia
Diffuse proliferative glomerulonephritis, in Lupus nephritis falls under
a) Class II b) Class III c) Class IV
d) Class V

17. MCQ
A 30 year old lady presented to the outpatient department with an
Erythematous butterfly rash on her checks. Which of the following
antibodies should be assayed initially for her suspected condition
a) Anti Ds- DNA b) Anti Ro – Antibody
c) Anti centromere antibody d) Anti mitochondrial antibody

18. MCQ
The following condition is not associated with an anti- phospholipid
syndrome
a) Venous thrombosis b) Recurrent fetal loss
c) Thrombocytosis d) Neurological manifestations

19. MCQ
The following is not part of CREST syndrome:
A) Calcinosis cutis
B) Esophageal cancer
C) Telangiectasia
D) Raynaud's phenomena

20. MCQ
Antibodies most specific for CREST syndrome is
A) anti double stranded DNA
B) anti RNA
C) anti centromere
D) anti histone

21. MCQ
Complications of Sjogren syndrome is
A) Dry mouth
B) Dry eyes & keratitis
C) Lymphoma
D) Infections

22. MCQ
HLA – B27 is strongly associated with
A) Rheumatoid arthritis
B) Sclerosing spondylitis
C) SLE
D) Type 1 DM

23. MCQ
IRF5 gene controls production of
A) Interleukin 1
B) Interferon gamma
C) Interferon receptor
D) Interferon alpha

24. MCQ
IL23R gene is associated with
A) Inflammatory bowel disease [IBD]
B) Irritable bowel syndrome [IBS]
C) SLE
D) Type 1 DM

25. MCQ
A young female has tapering fingers with restricted mobility. She also
has a small mouth & pulmonary hypertension. Which of the following
antibodies should be assayed initially for her suspected condition
a) Anti Ds- DNA b) Anti Ro – Antibody
c) Anti centromere antibody d) Anti Sci-70 antibody