تفريغ محاضرة
Abnormal ECG & Arrhythmia
للدكتورة أمة الرحيم

ملف دورة ECG بصورة واضحة ل الذي لم يتضح له في الملخص

تفريغ لكلام الدكتوره بس ما ادري ان كان كافي او لا 🤷🤷

تفريغ محاضرة الدكتورة أمه الرحيم القهالي

nephritic syndrome.doc'

mechanical valves VS  bioprosthetic valves

محاضرة الدكتورة امة الرحيم congenital heart disease👆👆

من قناة الدفعه 2 البكالوريوس

كل الشكر للزميل عادل القيسي

الملف الذي كتب الدكتور نشوان الحالات منه في الاختبارات السابقة 👆

_____بوست 3️⃣____
Unusual features in SLE & Associated conditions
-----------------------------------------------------------------------------

دليلك الكامل لكل ما هو صعب وغير نمطى فى مريض
الذئبه

#Nonspecific cutaneous SLE
~~||~|~~~

✅Nonspecific cutaneous SLE refers to features relating to underlying illness rather than an autoimmune attack.

🌀These features may occur in other connective tissue and autoimmune diseases.

1) Nail fold capillary telangiectasia

2) Raynaud phenomenon (white fingers and toes on exposure to the cold)

3) Vasculopathy of tips of digits (occlusion of small blood vessels by thrombus)

4- Diffuse hair thinning without brittle hair

5- Urticaria, which may be neutrophilic on biopsy
Angioedema also can occur

6- Cutaneous vasculitis: palpable purpura or
urticarial vasculitis

7- Livedo reticularis (a network pattern of blood vessels) in 20–30% patients with SLE

N.B : patient with SLE can present by Angioedema

🔷Uncommon GIT symptoms
•••••••••••••••••••••••••••••••••••••••

✏️Anorexia , nausea and vomting

✏️Abdominal pain due to peritoneal inflammation ( peritonitis as one of polyserositis)

✏️mesentric vasculitis , bowel infarction , perforation & peritonitis

✏️Liver affection
Associated elevated liver enzymes

✏️SLE can be associated with Autoimmune hepatitis at the same time

🔷Uncommon chest Features
••••••••••••••••••••••••••••••••••••••

✏️Acute pneumonitis :
Presents é fever , cough , dyspnea and pleurisy along with late inspiratory crackles on the chest
🫁CT chest : ground glass apperance

✏️Alveolar hemorrhage ; acute onset of dyspnea , fever , hemoptysis + low O2 saturation
🫁CT chest : bilateral alveolar shadows with airbronchograms

✏️interstitial lung fibrosis related to SLE as any connective tissue disease ( gradual dyspnea , dry cough along with inspiratory crackles on the chest

✏️Pulmonary Hypertension ( high JVP, pitting LL edema , accentuated P2 ± left parasternal pulsations/heave

✏️Shrinking Lung Syndrome
It is a a rare complication characterized by dyspnea , episodic pleuritic chest pain , and progressive reduction in lung volume in abscence of interstitial fibrosis
🫁CT chest : unexplained lobar collapses


✏️Pulomnary embolism can occur due to Anti-phospholipid syndrome secondary to SLE مهمه جداااا

Unusual Cardiac manifestations of SLE
=================================

✏️Valvular lesions called Libman-Sacks endocarditis that develop in 6 - 10 % of all SLE patients
#there are non-bacterial verrucous lesions near the edge of mitral , aortic and tricuspid valves { mostly affecting the mitral valve leading to Mitral Regurge

Some of cases are Asymptomatic while
the others are Symptomatic ,
Some cases lead to systemic emboli
Also Non-bacterial endicarditis may occur

N.B : Mitral Valve proplapse is frequently seen in SLE patients

✏️Myocarditis : is an uncommon comolication of SLE

C/P
resting tachycardia out of proportion to temperature with non specific ECG changes + Cardiomegaly
-> Global hypokinesia is seen on ECHO

✏️Coronary heart disease can occur because of autoimmune coronary vasculitis + accelerated atherosclerosis

✏️Associated Anti-phospholipid Syndrome may lead to Myocardial infarction

✏️Pediatric complications
In mothers of SLE with positive Anti-Ro and/or Anti-La , there is 3% risk of developing NEONATAL LUPUS with Congenital Heart Block

Unusual neuropsychiatric features in SLE
••••••••••••••••••••••••••••••••••••••••••••••••••••••

Rule 1 : the range of neuropsychiatric symptoms of SLE is broad , it ranges from mild symptoms such as poor concentration and emotional disturbances to SEVERE SYMPTOMS such as Seizures, stroke and psychosis



Rule 2 :
التصنيف من الاكثر حدوثا فالاقل و هكذا

From the most to the least common, symptoms range from DEPRESSION, COGNITIVE DISTURBANCES , HEADACHE , MOOD DISORDERS , STROKE/TIA, SEIZURES , POLYNEUROPATHY , ANXIETY and PSYCHOSIS

Rule 3 : any neuro-psychiatric features should be assessed carefully ( there are many causes of each symptoms
لابد من استبعاد اى سبب اخر لاى عرض عصبى او نفسى ،، وفى حالة استبعاد الاسباب الاخرى تصنف الاعراض العصبيه او النفسيه بانها عرض للذئبه الحمراء

الكليه الامريكيه للروماتيزم صنفت كل الاعراض العصبيه
⤵️ للذئبه الحمراء كالاتى

Central ( brain & Spinal cord affection )

- Psychosis
- Mood disorder نفسى
- Cognitive dysfunction
مشاكل فى الذاكره والتركيز
- Anxiety disorder
اضطراب القلق
- Acute Confusional state
خلل حاد فى مستوى الوعى مع اضطراب الكلام والتصرفات

- Seizure تشنجات
- Stroke جلطة بالمخ
- Headache الصداع النصفى والعصبى
- Myelopathy
التهاب حاد فى الحبل الشوكى وغالبا ما يسبب شلل الاطراف وفقدان الاحساس تحت مستوى ال lesion مع عدم التحكم فى البول

- Movement disorders {Chorea & Ataxia}

- Demyelinating diseases

- Aseptic meningitis

Peripheral
••••••••••••••
- Guillain Barré Syndrome ( Association )
- Autonomic Neuropathy
- Mononeuropathy
- Polyneuropathy
- Plexopathy
- Cranial Neuropathy
- Myasthenia Gravis ( Association)

Uncommon manifestations include ⤵️

🚩Tranverse Myelitis
Triad ( Paraplegia + sensory level + Sphincteric features )
ودى حاله طارئه

🚩 CNS vasculitis
Rare manifestion in SLE ( fever, severe headache , confusion , progressing rapidly to seizures , psychosis then coma

🚩Reversible posterior leucoencephalopathy syndrome ( clinically , Seizure , disturbed conscious level , visual changes , and headache
MRI brain in PRES 🧠 --> vasogenic cerebral edema in posterior cerebral hemispheres

🚩Movement disorders ( chorea & Ataxia )

🚩Cranial neuropathies ( 5th, 7th and bulbar )

🚩Aseptic Meningitis

Unusual hematological features of SLE
===================================

#Evans Syndrome

Prescence of Autoimmune hemolytic anemia ( Coomb's positive ) + Thrombocytopenia concurrently at the same time

#TTP ( Thrombotic Thrombocytopenic Purpura )

Hemolytic anemia + thrombocytopenia

بس بشروط وهى ⤵️⤵️⤵️⤵️

1) evidence of intravascular hemolysis ( high retics , high LDH , low haptoglobin , positive hemosiderin ± hemoglobin in urine

2) Coomb's test must be negative to exclude autoimmune type of hemolytic anemia

3) blood film must have evidence of Fragmented RBC's ( Schistocytes)

4) associated Renal impairement
ممكن اه وممكن لا

5) Neurological features at the same time of this hemotological problem
فى صورة خلل فى الوعى او ضعف مؤقت فى جزء من الجسم وليس جلطه مكتمله بالمخ وده بردو ممكن اه او لا

6)associated fever at the same time of hematological problem

والنوع ده من ال ----------⤵️⤵️⤵️
TTP is secondary to SLE

#Pancytopenia

#Macrophage Activating Syndrome ( MAS)
•••••••••••••••••••••••••••••••••••••••••••••••••••••••••

It is a rare condition that occurs secondary to SLE

It is a form of HLH ( hemophagocytic lymphohisteocytosis or hemophagocytic syndrome )

ودى اعراضها كالاتى ⤵️

- Bi or pancytopenia
- high PT , PTT
- marked elevated FERRITIN بالالاف
- high Triglycerides

Cause : low or abscent Natural killer cell activity --> ↑ soluble CD25 ( IL2 receptors)

Clinical features
••••••••••••••••••••••

Fever , weight loss , arthritis and rash

فى مثل هذه الحالات لابد ايضا من عمل بذل نخاع لاثبات التشخيص
BM examination will reveal hemophagocytosis ( infiltration of the BM by phagoctyes or histeocytes )

This condition responds well to
immunosuppression

🪩 Eyes Signs & symptoms in SLE
````````````````````````````````````````````````

المفروض مريضة الذئبه مش بتشتكى من عنيها ،، طب لو اشتكت ايه الاحتمالات الممكنه اللى ممكن تصيب العين ؟

1) Keratoconjunctivitis sicca

ودى هتخلى المريضه تشتكى من جفاف بالعين محتاج قطره واحمرار متكرر واحساس بوجود جسم غريب بالعين يسبب الحكه gritty sensation

Keratoconjunctivitis Sicca ? Why ??
⤵️
Associated Sjogren's syndrome

ملحوظه :
Sjogren's syndrome might come as a primary condition ,or secondary to other collagen diseases like SLE and Rheumatoid Arthritis
RA > SLE

2) Anterior Uveitis , keratitis and episcleritis
ودول بيحمروا العين ودكتور الرمد بيميزهم

3)retinal vasculopathy

General features of SLE
••••••••••••••••••••••••••••••••

As SLE is a systemic autoimune inflammatory disease , so Active inflammation with Active immune system will lead to the following 🔰

#weight loss

#Generalized Lymphadenopathy

#Organomegaly particularly,Splenomegaly!!

Very important item
°°°°°°°°°°°°°°°°°°°°°°°°°
#Conditions which might come in Association with SLE

🚩مهم جدااا جدااااا🚩

1️⃣ Anti-phospholipid Syndrome ( APS)

SLE & APS = high Association

2️⃣ Sjogren's syndrome
⤵️⤵️⤵️⤵️
Dry mouth & Dry eyes + red eyes

3️⃣ Rheumatoid Arthritis

SLE + RA at the same time

Rheumatoid + Lupus is called ”RUPUS”
ودى تسمية خبراء الروماتيزم الاجانب

4️⃣Polymyositis + Systemic sclerosis ( scleroderma) + SLE in the same patient
”Mixed connective tissue disease ”

Specific autoantibody
Anti-U1 RNP

5️⃣If the 3 diseases affect the same persons with coexisting Sjogren's ± RA , it is called Overlap syndrome.

6️⃣Fibromyalgia

طبعا هتلاقى المريضه بتشتكى من الم فى عضلاتها

NB ; diffuse muscle pain can't be explained by SLE

7️⃣other autoimmune non rheumatological conditions e g Thyroid disease , AIH , Myasthenia and etc !!

____بوست 2️⃣ 💻______
How to take focused history ?
To do focused examination 🩺 ‼️
To order suitable workup in a case 🧪
Of SLE and diagnostic criteria
🎯 والمعايير التشخيصية العالميه 🎯
🔷 لمرض الذئبه الحمراء 🔷 _________

👥Focused history/Focused Examination in SLE 🩺
====================================

🕵️Ask about the following 🔰

1️⃣ joint pain ( using SOCRATES rule )

✓Comment on distribution & Morning stiffness

✓Examine for affected jounts ( hands, UL & LL)

🩺 Look , feel , move protocol , give a particular attention to prescence of ACTIVE SYNOVITIS and DEFORMITIES { JACCOUD'S ARTHROPATHY}

2️⃣Skin features
~~

✅Assess the prescence of acute , subacute and chronic cutaneous lupus lesions

✔️Look for such lesions and describe them

3️⃣Hair/scalp lesions
~~~~~~

✔️Ask about hair loss , Alopecia , rash on the scalp

✅Look fo such rashes and describe them

4️⃣Mucosal affection
~~~~~

✔️Ask about Oral & nasopharyngeal ulcers

✔️Look for ulcers

5️⃣Serosal affection
~~~

✔️Ask about
Pleurisy ( pleuritic chest pain )

✔️Pleural effusion ( dyspnea )
Confirm your diagnosis by auscultation & percussion

✔️Pericarditis ( ask about pleuritic chest pain that ↓ with leaning forwards

✔️Confirm your Dx by auscultation of Pericardial Rub

✔️pericardial effusion
Screen for Beck's Triad ( high JVP , hypotension and distant heart sounds)

5️⃣Blood Affection
~~

✔️Ask about pallor & fatigue ( anemia )

✔️Ask about jaundice ( hemolytic anemia )
Confirm by exam

✔️Ask about bleeding tendency bleeding spots( purpuric eruption and bleeding orifices )

6️⃣Kidney Affection
~~~~

✔️Ask about Frothy urine , red urine and reduced urine output

✔️Ask about periorbital edema ( especially at the morning )

✔️Check BP for high BP

7️⃣CNS /PNS affection
~~~~~

✔️Ask about confusion , delerium , seizures and psychotic features

✔️Ask about foot drop and hand drop ( confirm by exam )

🕵️Lab abnormality 🧪
~~~~~

✔️Ask about any previous labs like
Anemia
Or
✔️Autoimmune hemolytic Anemia ( AIHA)
Or
✔️Thrombocytopenia
Or
✔️Leucpenia
Or
✔️Pancytopenia

#Any labs that prove high ESR

#Any lab test that proves Positive ANA and Anti-ds DNA

✏️Workup✏️
°°°°°°°°°°°°°

Basic Labs
~~~~~~~

✓CBC
✓LFTs
✓RFTs
✓Coagulation profile
✓Random blood glucose

Specific Labs
----------------------

*ANA ( titre must be ≥ 1/80)

*Anti-ds DNA ± Anti Smith Ab

* Lupus Anticoagulant , Anti-cardiolipin Ab and Anti-glycoprotein Ab

* C3 & C4

* ESR

🔷Screen for organ affection ⤵️

🟤Kidney ;
✔️Urine analysis & microscopy , ACR
✔️Biospy should be done if the proteinuria is significant or RPGN is suspected

🟤Blood ; Coomb's test , Retics

🟤Polyserositis ( CXR & ECHO if clinically indicated ) Diagnostic aspiration is indicated also

Important Quiz ‼️❓

Does ANA negative SLE occur ?

The answer
==========

If you suspect SLE but ANA is negative , please don't rule out SLE untill doing HEp-2 assay !!

American College of Rheumatology ACR recently recommend the new specific technique for detecting ANA which is called HEp-2 assay

The HEp-2 cell, a native protein array with hundreds of antigens, provides an ideal substrate for the detection of ANA1.

The detection of ANA in human serum is an important screening tool for connective tissue diseases, and IIF ( indirect immunofluorescne )is the reference method for ANA testing

. Recently, IIF on HEp-2 cells has been replaced in some laboratories with antigen-specific immunoassays and multiplex methods.

Due to concerns over false negative results and the lack of transparency to clinicians,

the American College of Rheumatology formed a Task Force that concluded that IIF using HEp-2 cells should be the “gold standard” for ANA screening.

🔷Diagnostic criteria 🔷 المعايير التشخيصية
``````|``````|``````|

``|``

|

ACR 1982 criteria ( updated in 1997 ) for the diagnosis of SLE

🟢 11 criteria 🕵️


✏️Mnemonics for simplification
اختار اى واحد يعجبك ويسهل الحفظ

💻SOAP BRAIN MD

S: Serositis ( pleurisy or pericarditis )

O: Oral/nasal ulcers

A: Arthralgia/arthritis ( non erosive)

P: Photosensitivity

B: Blood disorder : hemolytic anemia , Leucopenia , lymphopenia or thrombocytopenia

R: Renal involvement : persistent proteinuria or cellular casts ( dysmorphic RBC's)

A: Anti-nuclear Antibody (ANA)

I: immunologic marker ( positive Antids DNA , Anti-smith Ab , and/or Anti-phospholipid Ab

N: Neurological disorder ( seizures or psychosis)

M: Malar Rash

D: Discoid Rash

-------------------------------
Another Mnemonic !!

💻I'M DAMN SHARP
~~~~~

I: immunoglobulins : antidsDNA , AntiSm Ab , Anti-phospholipid

M: Malar Rash

D: Discoid Rash

A: Anti-nuclear Antibody (ANA)

M: Mucositis ( Oropharyngeal ulcers)

N: Neurological disorders

S: Serositis { pleurisy & pericarditis }

H: Hematologic ( Cytopenias)

A: Arthritis

R: Renal disease(Nephritis)

P: Photosensitivity
----------------------------------------------
Another Mnemonic!!

💻RASH ON MAIDS ( young woman with rash) ⤵️

R: Renal ; proteinuria , active urine sediments)

A: Arthritis

S: Serositis

H: hematologic

O : Oral or Nasopharyngeal ulcers

N: Neurologic

M: Malar Rash

A: ANA positive

I: Immunologic like AntidsDNA

D: Discoid lupus

S : Sun sensitive rash

To diagnose SLE , the patient must fulfill at least 4/11 criteria ( ≥ 4/11)

📈 SLICC criteria for the diagnosis of SLE ( 2012)

🅰️] CLINICAL CRITERIA :
---------------------------------------
1️⃣) Acute cutaneous lupus ( malar rash & maculopapular rash & photosensitive rash )

#Subacute cutaneous lupus : ( non indurated psoriasiform lesions and/or annular polycyclic lesions that resolve without scarring

2️⃣) Chronic cutaneous lupus ( Discoid rash above the neck )

3️⃣) oral ulcers ( oral, palate nasal)

4️⃣) Non-Scarring alpoecia : diffuse thinning or hair fragility with visible broken hair

5️⃣) Synovitis involving 2 or more joints characterized by swelling or effusion or tenderness on two or more joints and 30 minutes or more morning stiffness

6️⃣) serositis
( pericarditis or pericardial effusion)
(Pleurisy or pleural effusion )

7️⃣) Renal affection
( proteinuria > 0.5 gm and or RBC's casts

8️⃣) Neurologic ( seizures , psychosis , mononeurutis multiplex )

9️⃣) hemolytic anemia
1️⃣0️⃣) leucopenia ( < 4000)
1️⃣1️⃣) thrombocytopenia ( < 100,000)

🅱️] Immunological Criteria
-----------------------------------------
1️⃣) ANA above lab reference range
2️⃣) anti-ds DNA above lab ref. range
3️⃣) Anti-Sm Ab
4️⃣) Antiphospholipid Abs ( lupus anticoagulant , Anticardiolipin Ab)
5️⃣) Low Complement
6️⃣) Direct coomb's test

💻To diagnose SLE , the patient must fulfill
≥ 4/ 18 with at least one clinical and one lab criteria

N .B
SLICC : systemic lupus international collaborative clinic

Diagnosis needs at least 4/18 , (at least one clinical and one lab)

__________
New ACR/EULAR 2019 classification Criteria for systemic lupus erythromatosis (SLE) diagnosis
___________

❇ Entry Criterion 👇👇

ANA positive( titre ≥ 1/80)

➡ if abscent don't classify as SLE

➡If present , Apply ⬇⬇⬇

Additive Criteria 👇👇👇

▶ Constitutional
Fever -----> 2️⃣

▶Hematologic
Leucopenia ---> 3️⃣
Thrombocytopenia --> 4️⃣
Autoimmune hemolysis ---> 4️⃣

▶ Neuropsychiatric
Psychosis ---> 3️⃣
Delirium ---> 2️⃣
Seizures ----> 5️⃣

▶Mucocutaneous
Non-scarring alopecia ---> 2️⃣
Oral ulcers --->2️⃣
Subacute cutaneous lupus or discoid lupus ---> 4️⃣
Acute cutaneous lupus ---> 6️⃣

▶ Serosal
Pleural effusion /Pericardial effusion ->5️⃣
Pericarditis --> 6️⃣

▶ Musculoskeletal
Joint involvement ---> 6️⃣

▶Renal
Proteinuria > 0.5 gm --> 4️⃣
Biopsy class II or V lupus nephritis -> 8️⃣
Biopsy class III or IV lupus nephritis
-> 1️⃣0️⃣

▶Anti phospholipid antibodies
Lupus anticoagulant or
Anticardiolipin Ab or Anti beta2 glycoprotein --> 2️⃣

▶ Complement proteins
Low C3 or Low C4 --> 3️⃣
Low C3 + Low C4 --> 4️⃣

▶ SLE specific antibodies
Anti double stranded DNA Ab
Or
Anti- Smith Ab ----> 6️⃣

To diagnose SLE , we need at least one clinical criteria + ≥ 1️⃣0️⃣ points Criteria ( not need to occur simultaneously )

N.B

ACR : American College of Rheumatology

EULAR : European League Against Rheumatism

•••••••••••••••••••••••••••••••••••••••••••••••

احدث واحده هى الاخيره
ACR/EULAR 2019

🚩ملحوظه🚩
فى حالة عدم القدره على حفظ ⤵️⤵️
New ACR/EULAR

من الممكن استخدام ال SLICC criteria لانها اقرب للقديمه واسهل وفى نفس الوقت درجة دقتها عاليه

••••••••••••••••••••••••••••••••••••••••••••••
✏️ SLE from A to Z 🎯 بوست 1️⃣ 💻
••••••••••••••••••••••••••••••••••••••••••••••

🔷Definition

💻Lupus is a chronic autoimmune disease characterized by production of Autoantibodies which deposit within the tissues and fix complement leading to TISSUE
INFLAMMATION

✅Manifestations can vary from Skin and joint disease to organ dysfunction such as Renal affection , cerebral affection , hematological affection ETC

💥 Prognosis can vary depending on the degree of organ involvement and damage

🔎 Gender affection !!
كما هو معروف ،، فإن هذا المرض هو مرض يصيب الإناث
غالبا ونادرا ما يصيب الذكور

✓Female ♀️to Male♂️ ratio ; 9 : 1 or 10 : 1

✓The typical patient is female of age range from 15 - 45 years in childbearing period

✓This suggests a strong relationship between Female Sex hormones and development of SLE

السؤال المهم ؟ 🌀

هل من الممكن أن يحدث مرض الذئبه الحمراء فى سن أقل من ١٥-١٦ ؟! وهل هناك إمكانية لحدوثه فى
الخمسينات ؟

الاجابه ؛ تقريبا 20% من الحالات بتحدث فى سن أقل من ١٦ سنه ،، مع بقاء النسبه الأكبر أيضا للإناث

ولكن الغريب أن مرض الذئبه الحمراء اكتشف مؤخرا كنتيجة للاحصائيات الاخيره حدوث نسبة ١٥ ٪ من الحالات بعد ال 55 سنه

ملحوظه : عندنا قاعده فى البراكتس وهيا استبعاد مرض الذئبه الحمراء بعد سن الخمسين ،، ولكن هذا غير دقيق ،، لابد من وضعها أيضا فى الاحتمالات ولكن ليست هيا الاحتمال الأول‼️

✏️ If you use a mindmap for mutlisystem Connective Tissue diseases ‼️

📈What are the organs affection in SLE ?!!
•••••••••••••••••••••••••••••••••••••||••••••••••••••••

🟢SKIN ✔️

🟢MUCUS MEMBRANES ✔️

🟢JOINTS ✔️

🟢SEROSAL MEMBRANES
( pleura & pericardium ) ✔️

🟢BLOOD ✔️

🟢KIDNEYS ✔️

🟢CNS/PNS ✔️

🟢BLOOD VESSELS ( occasional )

🟢LUNGS ( rare)

🟢HEART ( rare)

🟢MUSCLES ( it is an association,but not a feature)

🟢EXOCRINE GLANDS ( it is an association but not a feature)

🟢EYES (it is an association but not a feature )

🟢CARTILAGES ( it is an association but not a feature)

🕵️Every affected tissue has immune deposits ( complexes ICs) which lead to certain Symptom , sign or even end organ failure

✏️Note 1️⃣

SLE is an autoantibody mediated disease
So , ANA must be positive , and in some cases Anti-dsDNA and Anti-smith Antibody might be positive

✏️Note 2️⃣
SLE has strong association with Anti-phospholipid syndrome

🚩 features of each organ بشكل مبسط !!

🟤Skin :

✏️Malar rash ( butterfly shaped erythematous rash on the cheeks sparing the nasolabial folds)

✏️Photosensitivity in sun- exposed areas

احمرار الوجه أو أى جزء من الجلد تم تعرضه للشمس

✏️Discoid lupus
```````````````````````

(DLE) is a chronic scarring skin condition.

➡️DLE is a sub-type of chronic cutaneous lupus erythematosus.

➡️It is characterised by persistent scaly plaques on the scalp, face, and ears which subsequently can progress to scarring, atrophy,dyspigmentation, and permanent hair loss in affected hair-bearing areas.

👥Other skin lesions 👥

Specific cutaneous SLE

Cutaneous lupus (CLE) has specific acute, subacute and chronic manifestations.

Acute cutaneous lupus
~~~

- Central face malar or "butterfly" violaceous erythema with a sharp cutoff at lateral margins, resolves without scarring (may result in persistent telangiectasia)

- Bullous systemic lupus erythematosus: a blistering rash,less common

✏️maculopapular rash resembling morbilliform drug eruption; less common

✏️Mucosal erosions and ulcerations (lips, nose, mouth, genitals); less common

✏️Photosensitivity: lupus rashes are mainly on sun-exposed sites. Photosensitivity can be mild to very severe with the rash appearing after minimal light exposure.

✏️Diffuse hair loss (nonscarring alopecia) with brittle hair shafts

🟤Subacute cutaneous lupus
•••••||•••••|••••••|•••••||•••••

✏️Flat, scaly patches resembling psoriasis, often in a network pattern

✏️Annular (ring-shaped) polycyclic (overlapping circular) lesions
Lesions resolve with minimal scarring
Affects trunk and arms
Flares on exposure to the sun, but usually spares face and hands

🟤Chronic cutaneous lupus
``||````|````|````||````|```|````|``

Classic discoid lupus is most common: indurated hyperpigmented plaques
Localised (above the neck in 80%) or generalised (above and below the neck in 20%)

🟤Joint pain
===========

✅Arthralgia is more common than Arthritis

Often, there is pain in joints ( small hands joints , large joints of arms like Elbows, shouders and large joints of legs ( knees)

✏️Note 👥

🔷The pain is of Inflammatory nature !!
(↓with movemens of the joints ➕ morning stiffness)

🔷If arthritis occurs ( true synovitis ) with joints swellings , hotness , redness , tenderness and limitations in movements --> it is non erosive Arthritis , unlike Rheumatoid arthritis!!

🕵️Quiz !!

Are there any deformities that could occur due to Arthritis of SLE ?!

✏️Answer
Arthritis related to SLE often doesn't lead to deformities except in special form called Jaccoud's arthropathy

🕵️Jaccoud's Arthropathy is a deforming, non-erosive arthritis, occurring in 10 % of SLE patients. It is correctable with physical manipulation

🚩While Jaccoud's Arthropathy can involve all joints, it most commonly manifests as severe deformations of the hands, including ulnar deviation, swan neck and boutonniere deformities, and Z-deformity of the thumb, with multiple non-erosive subluxations.

🔎Ulnar drift with subluxation of the MCP joint is often the first sign, whereas swan neck, boutonniere, and Z-deformities may occur at later stages.

🟤Oral ulcers
===========
They are often painless and commonly affecting the palate !!

🟤Serosal membrane affection { Serositis }
°°°°°|°°°°°°°°°°°°°°°°°°°|°°°°°°°°°°°°°°°°°°°|°°°°°°

👥Pleural affection ;

✅Pleurisy = pleuritic localized stitching chest pain ↑ é inspiration
Or
✅Pleural effusion ; dyspnea preceded by chest pain

👥Pericardial affection

✅Pericarditis ; pleuritic type chest pain which ↑ with inspiration and ↓ on leaning forwards { rub may be heard on the heart
Or
✅Pericardial effusion ; often manifested by Tamponade { Beck's triad : hypotension + high JVP and distant Heart sounds )

N .B : both Pleural effusion and pericardial effusion are exudative ( high protein & ↑LDH )

🟤Blood affection
==|====|=====|==

🕵️Hematological affection might be in the form of ⤵️

✅ Thrombocytopenia
~~~~~

( either isolated or in association with anemia )

Features : purpura ( petechiae & echymosis & orificial bleeding like vaginal bleeding , epistaxis , etc )

🟤Anemia
~~~

Either caused by autoimmune destruction ( warm type of Autoimmune hemolytic anemia ; AIHA)
Or
Anemia of chronic disease ( active collagen disease .

- Leucopenia , mainly due to lymphopenia ( note that isolated lymphopenia is a common lab sign in SLE , also it indicates activity

- Pancytopenia ( the least common feature )

🟤Kidney Affection
==============

SLE causes Glomerulonephritis ( immune complexes mediated ; immune deposits )

Renal affection can occur in some patients but not all patients

🟤Features of renal affection
°°°°°|°°°°°°°°°°°|°°°°°°°°°°|°°°°°°°

-Isolated Proteinuria > 0.5 gm
It might be heavy ≥ 3.5 gm causing Nephrotic Syndrome

- proteinuria with hematuria with RBC's casts ( Nephritic syndrome

- isolated hematuria ( with dysmorphic RBC's )

🔷 Many cases of lupus nephritis needs biopsy to assess the staging !;

International Society Of Nephrology ( ISN staging of lupus Nephritis )

Class I – Minimal mesangial lupus nephritis

Class II – Mesangial proliferative lupus nephritis

Class III – Focal lupus nephritis (active and chronic; proliferative and sclerosing)

Class IV – Diffuse lupus nephritis (active and chronic; proliferative and sclerosing; segmental and global)

Class V – Membranous lupus nephritis

Class VI – Advanced sclerosis lupus nephritis

✏️Note ✏️

Class II & III & IV --> nephritic syndrome with active urinary sediments(cellular & RBC's casts )


🚩Class III & IV are more serious , why ?!

✅Because , they have the potential to cause Crescentic GN with Rapidly progressive GN and AKI

✅Class V ( membranous ) often causes Nephrotic syndrome with heavy proteinuria

✅Class VI likely to cause CKD

🟤CNS/PNS affection
=============||===

➡️ Cerebral affection
``|````|````|```|````|````|``

-It is also called " Lupus Cerebritis "

🟢The major features of lupus Cerebritis are ⤵️

- Seizures ( not explained by another disease)

- Psychosis ( abnormal behaviour , severe depression and/or delerium)

PNS : Mononeuritis Multiplex ⤵️
( affection of a nerve in different group in different anatomical locations )

🎯إزاى تشخص مرض ال Mixed Connective tissue disease طبقا للمعايير التشخيصية العالميه؟ 🎯

🔷 Alarcón-Segovia diagnostic criteria
~|~~||||~~~

The Alarcón-Segovia diagnostic criteria consist of a positive anti–U1 RNP titer (>1:1600) and at least three of the following five clinical findings ⤵️

1️⃣Hand edema

2️⃣Synovitis

3️⃣Biologically or histologically proven myositis

4️⃣Raynaud phenomenon

5️⃣Acrosclerosis with or without proximal systemic sclerosis

🔷Kasukawa Criteria ( Japanese 🇯🇵)
``|````|````|````|````|```|```|```|```|```|```|````

1️⃣ Common manifestations: Raynaud phenomenon, puffy fingers and/or swollen hands

2️⃣ Immunologic manifestation: Anti–U1-RNP antibody positivity

3️⃣ Characteristic organ involvement: Pulmonary arterial hypertension, aseptic meningitis, trigeminal neuropathy

4️⃣ Overlapping manifestations: (A) Systemic lupus erythematosus (SLE)–like, (B) systemic sclerosis–like, (C) polymyositis/dermatomyositis-like

🅰️: SLE-like manifestations include the following ⤵️

✓Polyarthritis

✓Lymphadenopathy

✓Malar rash

✓Pericarditis or pleuritis

✓Leukopenia (4,000/μL or less) or thrombocytopenia (100,000/μL or less)

🅱️Systemic sclerosis–like manifestations include the following ⤵️

✓Sclerodactyly

✓Interstitial lung disease

✓Esophageal dysmotility or dilatation

©️Polymyositis/dermatomyositis-like manifestations include the following ⤵️

✓Muscle weakness

✓Elevated levels of myogenic enzymes

✓Myogenic abnormalities on electromyogram

🕵️For diagnosis of MCTD, the patient must have the following ⤵️

✓At least one common manifestation
➕
✓Anti–U1-RNP antibodies
➕
✓At least one characteristic organ involvement, or at least one feature of at least two of the three disorders in the overlapping manifestations category (A, B, and C)

🎯ازاى تقدر تشخص مرض بهجت المناعى باستخدام المعايير التشخيصية العالميه المختلفه 🎯

___________
🌎International Criteria for the diagnosis of Behcet's disease 🌏. 🧑‍🏫🧑‍🏫🧑‍🏫🧑‍🏫🧑‍🏫🧑‍🏫🧑‍🏫
-------------------------------------------------------------------------

🅰️ The International study group criteria
» The most widely recognized criteria «

🧑‍🏫 To diagnose Behcet's disease , the patient must have the following features
⤵️⤵️⤵️

🕵️ Recurrent painful oral ulcerations
( aphthous or herpetiform) at least 3 times in one year
🕵️ القُرَح المُؤلمه فى الفم دى عرض أساسى لابد يكون موجود طبعا ( نوبات شديده من القُرح على الاقل ٣ مرات فى السنه ...

➕

≥ 2️⃣ of the following ⤵️

🕵️ مطلوب اتنين على الاقل من الاربعه دول 🤷

🔶1️⃣ Recurrent genital aphthous ulceration
or scarring observed by a physician or reported reliably by patient

قُرَح مُؤلمه فى الاعضاء التناسليه ↙️↙️↙️
Scrotal ulcers in ♂️ & labial ulcers in ♀️

🔶2️⃣ Skin lesions (including erythema nodosum-like lesions, pseudofolliculitis, papulopustular lesions, or acneiform nodules) observed by a physician in a post-adolescent patient not receiving corticosteroids.

🕵️انواع مختلفه من ال skin lesions كما هو موضح،، ممكن تستعين بطبيب الجلديه لمعرفتها والتأكد من تشخيصها ❗

🔶3️⃣ Eye involvement with anterior/posterior uveitis or retinitis ( retinal vasculitis )observed by an ophthalmologist.

🕵️ التهاب مناعى داخل العين ولازم تستعين بطبيب رمد ضرورى عشان يأكد وجود الحاجات دى أو لا ‼️

🔶4️⃣ Positive pathergy test performed with oblique insertion of a ≤21-gauge needle under sterile conditions and interpreted by a physician at 48 hours.

🕵️ اختبار سهل ممكن تعمله بواسطه ابره تخترق الجلد بعد تطهيرها ومتابعة حدوث Papule اكبر من ٢ مم او Pustule فى خلال ٢٤ - ٤٨ ساعه

🕵️The positivity of Skin Pathergy Test
is defined as the presence of an erythematous papule ≥2 mm or a pustule after 24–48 h.

🅱️International criteria for Behcet's disease
``````|``````````|``````````|```````````|``````````|`````````

🧑‍🏫 معايير تشخيصه معتمده على نظام سكور ‼️

💁Oral Aphthosis ( ulcers )--»2️⃣

💁Genital Aphthosis( ulcers ) --» 2️⃣

💁Ocular Lesion --» 2️⃣

💁Skin Lesion --» 1️⃣

💁Neurological Manifestation --» 1️⃣

💁Vascular Manifestations --» 1️⃣

💁Positive Pathery Test -» 1️⃣ ( optional ❗)

🧑‍🏫 Scoring at least 4️⃣ ( total score ≥ 4️⃣) indicates Behcet's disease !!

🕵️ Note ✍️

🔶Neurological involvement 🧠
-------------------------------------------------

✓Neurologic involvement is seen in 5% of patients with Behcet's Diseaee .

✓There are two main forms:
vascular and parenchymal.

✓Parenchymal neuro-Behcet's Disrase leads to inflammatory lesions in the brain stem, diencephalon, basal ganglia and, less frequently, the spinal cord and cerebellum.

✓ It usually presents with bilateral pyramidal signs, unilateral hemiparesis, behavioral changes, sphincter disturbances, and headache.

✓The vascular neuro-Behcet's Disease
is dural sinus thrombosis, mainly characterized by headache and papilledema. It usually associates with venous thrombosis in lower extremities, and has better prognosis compared to parenchymal neuro-Behcet's disease

🔶Vascular involvement
--------------------------------------

✓Vascular Neuro Behcet's
✓DVT ± Pulmonary embolism
✓pulmonary vascular affection
✓possible arteritis

©️Japanese criteria
توجد معايير تشخيصه قام بوضعها خبراء يابانيون ومش هنذكرها عشان الناس ما تتلخبطش . اردت فقط ان اذكر ان هناك معايير يابانيه خاصه بمرض بهجت المناعى..

🎯ازاى تقدر تشخص متلازمة ال Anti-phospholipid syndrome طبقا للمعايير التشخيصية العالميه 🎯

----------------------------------------------------------------------
SYDNEY CLASSIFICATION CRITERIA FOR
ANTIPHOSPHOLIPID SYNDROME ( APS)
----------------------------------------------------------------------

A] Clinical Criteria
-----------------------------
1) Vascular thrombosis :
➡ either arterial or venous thrombosis
جلطات شريانيه مثل ال stroke مثلا وجلطات وريديه مثل ال DVT ‼️

2) Pregnancy Morbidity :
مشاكل متعلقه بالحمل ‼️

a) death of normal fetus at ≥ 10 weeks
b) premature birth at ≤ 34 weeks.
c) ≥ 3 consecutive abortions at ≤ 10 weeks
d) placental insufficiency at < 34 weeks .

B] LAB CRITERIA :
----------------------------

1) Anti- cardiolipin IgG/IgM
2) lupus anticoagulant
3) anti beta 2 glycoprotein

The labs should be proved positive at medium to high titre at least 2 times 12 weeks apart .

To diagnose APS , the patient must have at least one clinical and at least one lab test .

➡ Diagnosis of Catastrophic Antiphospholpid syndrome (CAPS) 👇👇

1) history of APS or persistence aPL Abs
2) 3 or more organs involved simultaneously or within 1 week ( blood, kidney , liver etc )
قصور حاد فى وظائف الكبد أو الكلى مع مشاكل فى الدم (انيميا ونقص صفائح )
3) microvscular thrombosis on histopathology
4) Exclusion of other causes of multi-organ thrombosis

N.B ✍️ : CAPS is a fatal condition

___بوست 1️⃣__
Rheumatology Made Easy
-------------------------------------------

🎯 Smart simplified proposed Guide for classification of rheumatological diseases
( Rheumatology Mindmap) 🧠

عشان ترتب كل الأمراض الروماتيزميه فى دماغك بطريقه ذكيه بحيث تلم كل الأمراض ... فعليك أن تقرأ البوست ده
البوستات القادمه هتكون بوستات تجميعيه لكل الاعراض الشهيره لكل امراض الروماتيزم ببساطه .. لكل من يعانى من صعوبه فى التعامل مع ال Rheumatology ‼️

✍️ الأمراض الروماتيزميه قد تكون ⤵️
Either
✔️Musculoskeletal disease ‼️
Or
Multi-system Collagen/connective tissue
disease ‼️

ـــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــ

🅰️ Musculoskeletal diseases include
----⤵️---------⤵️---------⤵️----------⤵️----

1️⃣Articular diseases
(mainly joint problem )

🔴 Inflammatory arthritis
✓ Rheumatoid Arthritis
✓Seronegative Spondyloarthritides


🔴 Mechanical arthropathies
✓Degenerative joint diseases
Osteoarthritis & Spondylosis

🔴 Metabolic joint diseases
Crystal arthropathies
✓ Gout
✓ Pseuogout

🔴 Infectious arthritis
( viral & bacterial; septic arthritis )
-----------------------

2️⃣ Bone diseases

🔴 Osteomalacia
🔴 Osteoporosis
🔴 Paget's disease
------------------------------

3️⃣ Muscle disease
( inflammation of the muscles : myositis)

🔴 Polymyositis
🔴 Dermatomyositis

--------- Note ✍️ -------
They might be Multi-system along with muscle affection , with prescence of Autoantibodies
-------------------

4️⃣Pain Syndromes

🔴 Diffuse pain syndromes

✓ Fibromyalgia
✓ Polymyalgia Rheumatica

🔴Localized pain syndromes

✓Complex regional pain syndrome
Severe pain with allodynia

✓Specific syndromes

- Shoulder
Adhesive capsulitis ( frozen shoulder)
Rotator calf tendinitis

- Elbow ( e.g tennis elbow )

_________

🅱️Multi-system Collagen/connective tissue diseases that include
⤵️ ⤵️⤵️⤵️⤵️⤵️

1️⃣ Group of systemic autoimmune diseases associated with the Prescence of Antibodies

ℹ️ Group of systemic autoimmune
Collagen diseases associated with
positive ANA and other specific antibodies

✔️ SLE
✔️ Scleroderma
✔️ Sjogren's syndrome
✔️ Polymyositis & dermatomyositis
✔️ Mixed connective tissue disease
✔️ Overlap syndromes
-------------

ℹ️ℹ️ Anti-phospholipid Syndrome
-------------

ℹ️ℹ️ℹ️ ANCA associated vasculitides

✔️ GPA ( Wegener's granulomatosis)
✔️ EGP ( Churg strauss syndrome )
✔️ Microscopic Polyangiitis( MPA)

-------------------------

2️⃣ Group of diseases that are not associated with the prescence of Autoantibodies

⚡Vasculitides except ANCA-associated type
⚡Behcet's disease
⚡Relapsing Polychondritis
⚡Sarcoidosis
⚡Amyloidosis
⚡Febrile Syndromes including
⤵️⤵️⤵️
✓ Rheumatic fever
✓Still's disease
✓Autoinflammatory Periodic fever
Syndromes e g FMF etc