-Case presentation

-How to publish case reports

ABUAJELA SREH – ST5

GASTROENTEROLOGY – SHEFFIELD
TEACHING HOSPITALS
14/01/2022
 Case report timeline

Case presented June – Sept 2016

Diagnosis made sept 2016
Case followed up over 7 months
Case submitted April 2017 to BMJ , rejected !
Resubmitted April 2017 to EJCRIM
Editing and reviewing 1 month
Published May 2017
 Case presentation

A 34 year-old Afro-Caribbean presented at the emergency

department with a 2 month history of fever and mild lower
abdominal pain.
She was initially treated in the community for recurrent
urinary tract infections but her symptoms persisted so she self-
presented to the emergency department. Patient reported
gradual onset of fever and intermittent lower abdominal pain.
Patient also recently developed a sore throat for which she was
treated in the community for upper respiratory tract infection.
Patient denied any genitourinary tract symptoms. Patient
neither had any changes in her bowel habit throughout her
illness period nor did she experience any per rectum bleeding.
Patient had no respiratory or neurological symptom and
denied any rash, weight loss, night sweats, arthralgia, or
lumps or bumps anywhere in her body.
Patient is married and has one child. Last menstrual period
was one week prior to her admission. Patient had been to
France with her family 3 weeks prior to her presentation. She
declined any illness or exposure to ill people while abroad.
There was no other recent travel history.
She was otherwise medically fit and takes no regular
medications.
She denied taking or injecting any illicit drugs. There was no
family history of recent illness, connective tissue,
haematological diseases, or malignancies.
 O/E

Patient was alert on clinical examination with no

pallor, jaundice, cyanosis, or clubbing.
Vital signs on admission showed temperature of 39.4
degrees and tachycardia at 126 beats per minute,
otherwise normal respiratory rate, blood pressure,
and oxygen saturation of 99% on air.
 O/E

Patient was noted to have enlarged cervical lymph

nodes but normal palpation of other lymph node
groups.
Cardio-respiratory examinations were unremarkable
and abdomen was soft with mild suprapubic
tenderness in the absence of any organ enlargement,
guarding, or rebound tenderness with normal bowel
sounds on auscultation.
No focal neurology or any signs of meningism.
 Differentials

Pelvic inflammatory disease

Urinary tract infection/ pyelonephritis
? HIV
Infection ? Other source ? Viral
Observations
 Investigations

Gynaecology opinion for PID and positive BHCG

(urine) and normal serum HCG.
TV US – Normal, no products of conception.
Treated with IV Tazocin/Gentamycin and PO
clarithromycin
Remained pyrexial
Urine c/s: no growth
Bloods: CRP 159. WCC 11.7. HB%127.
Normal UE’s. TSH normal. BMs normal.
LFT’s – Albumin 31, ALP 140, ALT 240.
Amylase normal
VBG normal
ECG normal
Urine dip negative
Chest x-ray
Differentials ?
 Investigations

Repeated Blood c/s no growth

Gynae swabs, throat, stool and urinary MCS negative
HIV negative
Hep A/B/C/E and syphilis negative
Serology – CMV, EBV, rubella, toxoplasma, Infectious mononucleosis negative
Respiratory PCR – negative to all virus
Urine legionella and pneumococcal antigen negative
Malaria screen negative
Rheumatoid screen negative
ANA weakly +ve
ENA, ANCA and Lupus screen- negative
Sickle cell and haemo-globinopathy screen negative
 Investigations

 TTE Echo – NAD

 CT T/A/P – Nil acute , no collection, no malignancy, no LN
 Differentials ??

Pyrexia of unknown origin

 Further tests

 ESR 120 ( 5 – 10 )
 LDH 2000 ( 120 – 240 )
 ACE 158 ( 12 – 71 )
 Ferritin 55000 ( 13 – 150 )
 Differentials

Infections now less likely

Autoimmune ? Sarcoidosis ? SLE
Metabolic ? Hemochromatosis
Malignancy ? Lymphoma
 Management

 Seen by infectious disease specialist at Sandwell Hospital

 Seen by Rheumatologist:
 Patient symptoms, signs, and investigations scored only
one out of eleven (for positive ANA) on the American
college of rheumatology criteria for systemic lupus
erythromatosis (SLE), excluding a diagnosis of SLE
 Bone marrow biopsy normal !
 Management

 Represented a week later with no improvement

 Diagnosis ?

Whole septic screen repeated, all negative !

 Diagnosis !

A diagnosis of exclusion of Adult Onset Still’s

Disease (AOSD) was reached and confirmed by a
rheumatologist using Yamaguchi diagnostic criteria
alongside the significantly raised serum ferritin as well
ESR and LDH.
Patient satisfied two major criteria (fever of more than
39° Celsius lasting more than a week alongside
leucocytosis with more than 80% polymorphoneuclear
cells) in addition to 3 minor criteria (sore throat,
lymphadenopathy, and deranged liver function).
 Adult Onset stills disease

Diagnostic criteria for adult onset Still’s disease (AOSD) Yamaguchi’s criteria
(Yamaguchi M. et al., . J Rheumatol. 19:424-30, 1992)

Major criteria
▪ Fever >39 °C, lasting 1 week or longer
▪ Arthralgia or arthritis, lasting 2 weeks or longer
▪ Typical rash
▪ Leukocytosis >10,000/mm3 with >80% polymorphonuclear cells

Minor criteria
▪ Sore throat
▪ Recent development of significant lymphadenopathy
▪ Hepatomegaly or splenomegaly
▪ Abnormal liver function tests
▪ Negative tests for antinuclear antibody (IF) and rheumatoid factor (IgM)

5 criteria and out of them 2 or more has to be major

 Management

All the antibiotics were stopped

patient was started on high-dose prednisolone,
to which patient responded remarkably well and so
steroids were tapered over four weeks.
Patient improved significantly when she was
reviewed in the rheumatology outpatient clinic.
Patient was explained the possible need for disease
modifying anti-rheumatic drugs (DMARDs) if her
symptoms relapse again in the future.
Management
 Adult Onset Still’s Disease

Rare, multi-Systemic inflammatory disorder of unknown

etiology and pathogenesis

Characterized by
High spiking fever
Arthralgia or arthritis
Maculopapular rash

Diagnosis is based on exclusion of infections, autoimmune

disease, hematological disorders and malignancies.
Adult onset still’s disease
 Treatment

No randomized controlled trials have been performed to

evaluate treatments for AOSD

 NSAID’s
 Steroids
 DMARDs
 Monoclonal antibodies
 LEARNING POINTS

The four giant causes of fever of unknown origin are:

infections, connective tissue diseases,
haematological disorders, and malignancies.
AOSD is a diagnosis of exclusion using Yamaguchi
criteria alongside raised ferritin levels (the most
sensitive biomarker).
Most AOSD patients will respond to steroid
treatment. However, approximately one third of the
patients require further immunosuppression to
achieve disease remission
How to publish case reports
 Tip 1

Think about publishing your case if:

Rare in literature e.g. 1 in 100,000

Atypical presentation of a common disease
Management challenges
 Tip 2

Once you encounter an interesting case, make sure

you get a written consent ASAP ! Wont lose anything
 Tip 3

Work smart AND hard

Follow up you cases when admitted and if needed as
outpatient e.g. ambulatory , specialty clinic, over the
phone , chase clinic letters, etc
 Tip 4

Ask around for known journals for your case

depends on the specialty
 Tip 5

Then start gathering your information on that journals

case report proforma :
Title
Keywords
Abstract
Learning points
Case descriptions
methods and procedures
Discussion
References, image captions and acknowledgment
 Tip 6

Work in a group ! Will make life very easy and saves

lots of time e.g. case report data, follow up, literature
review etc.
Speak to the librarian ! Will help a lot with your
literature review.
Most journals will allow 4-5 authors
Consultant to go through the final draft , ideally you
need a consultant who is a specialist in that area.
 Tip 7

Don’t be put off !

If one journal rejects, another journal will accept.
Keep trying and never give up !

Plenty of advice available on Google and YouTube.

 Tip 8

A recommended start is BMJ Minerva OR

Endgames:

Minerva pictures (spot diagnosis)

These are pictures which offer an educational message
and which will publish clearly and depict the
abnormality obviously.
Endgames:
Short case repots. Endgames is designed to help
doctors across all levels and specialties test their
knowledge and reflect on their practice for continuing
medical education
 What happens after submission?

Editing team : for proofreading, assuring adherence

to the journal’s proforma and correct referencing
method followed.

Reviewing process: a decision re accepting the paper,

usually specialist in the area, lots of questions and
criticism !!

Publication process: confirming names, final draft

etc
 References

Bywaters EG. Still's disease in the adult. Ann Rheum Dis

1971; 30: 121–33.
Magadur-Joly G, Billaud E, Barrier JH, et al. Epidemiology
of adult Still's disease: estimate of the incidence by a
retrospective study in west France. Ann Rheum Dis 1995;
54: 587–90.
Crispin JC, Martinez-Banos D, Alcocer-Varela J. Adult-
onset Still disease as the cause of fever of unknown origin.
Medicine (Baltimore) 2005; 84: 331–7.
https://www.bmj.com/company/bmj-resource-centre/bmj-
case-reports-resources/bmj-case-reports-how-to-get-
published/