Grading of Sacroiliitis on Imaging

Plain radiographs of the sacroiliac (SI) joints can be semiquantitatively graded based upon the presence of the characteristic radiographic findings :

●Grade 0: Normal .

●Grade 1: Suspicious changes .

●Grade 2: Minimal abnormality – Small localized areas with erosions or sclerosis, without alteration in the joint width . Erosions usually first appear on the iliac side.

●Grade 3: Unequivocal abnormality – Moderate or advanced sacroiliitis with erosions, evidence of sclerosis, widening, narrowing, or partial ankylosis .

●Grade 4: Severe abnormality – Total ankylosis

Bhopalwala. H

Classification Criteria for Axial Spondyloarthritis

Axial SpA — Classification criteria for axial SpA, including criteria for those without plain radiographic changes (nonradiographic axial SpA [nr-axSpA]) and with radiographic (radiographic axial SpA) changes of sacroiliitis, were based upon a large multicenter study [39]. In patients with a history of back pain of unknown origin, which was of at least three months' duration and which began before age 45, the classification criteria for axial SpA exhibited sensitivity of 83 percent and specificity of 84 percent [39]. This algorithm is as follows:

●The entry step is that the patient must have had back pain of any type for at least three months, and the age of onset must be less than 45 years.

●The second step consists of two arms that are evaluated separately based upon the presence either of sacroiliitis on imaging or of human leukocyte antigen (HLA)-B27:

•HLA-B27-positive patients – In patients who test positive for HLA-B27, at least two additional features of SpA from the list below are required for classifying a patient as having axial SpA (see 'SpA features' below)

•Sacroiliitis on imaging – In patients diagnosed with sacroiliitis based upon plain radiographs (structural changes) or magnetic resonance imaging (MRI) (subchondral bone marrow edema [BME]), at least one other feature of SpA from the list below should be present (see 'SpA features' below)

SpA features — The following are SpA features that contribute to the classification criteria for axial SpA (see 'Axial SpA' above):

●Inflammatory back pain – Several definitions for inflammatory back pain have been proposed. For classification purposes, inflammatory back pain can be defined as having at least four of the five following parameters [67]:

•Age of onset <40 years

•Insidious onset

•Improvement with exercise

•No improvement with rest

•Pain at night (with improvement upon arising)

●Other SpA features (each of equal weight) – Other SpA features include the presence of one or more of the non-spinal features noted below. The occurrence can be either before or at the time of evaluation for the following items listed: arthritis, heel enthesitis, uveitis, dactylitis, psoriasis, and inflammatory bowel disease. The features are:

•Arthritis – As diagnosed by a clinician

•Heel enthesitis – Spontaneous pain or tenderness at site of insertion of Achilles tendon or plantar fascia at the calcaneus diagnosed by a clinician

•Uveitis – Confirmed by an ophthalmologist

•Dactylitis – Diagnosed by a clinician

•Psoriasis – Diagnosed by a clinician

•Inflammatory bowel disease – Crohn disease or ulcerative colitis diagnosed by a clinician

•Good response to nonsteroidal antiinflammatory drugs (NSAIDs) – Within 24 to 48 hours

•Family history of SpA – Presence in first- or second-degree relatives of AS or acute anterior uveitis [37]

•Elevated C-reactive protein (CRP) – After exclusion of other causes for elevated CRP

Bhopalwala. H

Classification Criteria for Peripheral Spondyloarthritis

For classification as having peripheral SpA, the patient should NOT have concurrent inflammatory back pain. In that case, the axial SpA criteria should be used. However, inflammatory back pain in the past is considered as a contributing SpA feature. There are two steps in the algorithm for classifying peripheral SpA:

The entry step is that the patient should have, at the time of being seen, at least one of the following three findings:

●Arthritis

●Enthesitis (spontaneous pain or tenderness at any enthesis)

●Dactylitis

If the patient satisfies the entry criteria, the patient should show (or have had in the past) at least one of the features of SpA in Group A (below) or at least two of the features of SpA in group B (below):

●Group A SpA features:

•Uveitis – Confirmed by an ophthalmologist

•Psoriasis – Diagnosed by a clinician

•Crohn disease or ulcerative colitis – Diagnosed by a clinician

•Preceding infection – Urethritis/cervicitis or diarrhea within one month prior to onset of arthritis/enthesitis/dactylitis

•HLA-B27

•Sacroiliitis on imaging

●Group B SpA features:

•Arthritis – Diagnosed by a clinician

•Enthesitis – Diagnosed by a clinician

•Dactylitis – Diagnosed by a clinician

•Inflammatory back pain in the past

•Family history of SpA – Presence in first- or second-degree relatives of AS and acute uveitis

Bhopalwala. H

How to Define Inflammatory Back Pain

●Inflammatory back pain – Several criteria have been proposed for defining IBP ; we use the "ASAS [Assessment of SpondyloArthritis International Society] expert criteria" to identify patients with IBP . The criteria for IBP are met if at least four of the following five features are present and IBP is described as "suggested" in the presence of three of five features:

•Onset of back discomfort before the age of 40 years

•Insidious onset

•Improvement with exercise

•No improvement with rest

•Pain at night (with improvement upon arising)

The presence of four of the five features listed above has a sensitivity and specificity of 80 and 74 percent, respectively, for an inflammatory cause of the back pain among patients with chronic back pain of unclear origin and onset of the back pain at <45 years of age who were evaluated by local rheumatologists . However, when IBP is present, and considered independently of other factors, the probability of AS among patients with chronic back pain increases only from 5 percent to 14 to 16 percent .

Bhopalwala. H

Rhupus

Rhupus – The term rhupus has been used to describe patients with overlapping features of both SLE and RA. Whether rhupus is clinically and immunologically a distinct entity, a true overlap of SLE and RA, or a subset of patients with SLE remains a matter of debate. In addition to having serologies consistent with both SLE and RA, some patients classified as rhupus may have an erosive arthropathy that is atypical for SLE

Bhopalwala. H

Kikuchi's disease

●Kikuchi's disease – Kikuchi's disease is a benign and usually self-limited form of histiocytic-necrotizing lymphadenitis. Clinical features at presentation include lymphadenopathy as well as fever, myalgias, arthralgias, and, less commonly, hepatosplenomegaly. Associations with SLE have been reported, but the clinical course is usually favorable with spontaneous remission often occurring within four months. The diagnosis of Kikuchi's disease is based on a lymph node biopsy, which reveals a histiocytic cellular infiltrate.

Bhopalwala. H

Psoriatic Arthritis

Diagnostic criteria for Psoriatic Arthritis :

●CASPAR criteria – The CASPAR study concluded that a patient with an inflammatory musculoskeletal disease (peripheral arthritis, spondylitis, or enthesitis) can be classified as having PsA if a total of at least three points is accumulated from the presence of the following list of features (each of which is assigned a certain number of points):

•Skin psoriasis that is:

-Present – two points, OR

-Previously present by history – one point, OR

-A family history of psoriasis, if the patient is not affected – one point

•Nail lesions (onycholysis, pitting) – one point

•Dactylitis (present or past, documented by a rheumatologist) – one point

•Negative rheumatoid factor (RF) – one point

•Juxtaarticular bone formation on radiographs (distinct from osteophytes) – one point

These classification criteria should facilitate studies in PsA and may function well in diagnosing PsA, given sensitivity and specificity in four studies, including two of early arthritis patients, ranging from 91 to 100 percent and 97 to 99 percent, respectively [90-94]. However, these criteria can only be applied to individuals who demonstrate evidence for inflammatory musculoskeletal disease (peripheral arthritis, axial disease, or enthesitis).

Rheumatology has a lot of Rheum (Room) for Internal Medicine.

Bhopalwala. H

Anti-Ro/SSA antibodies and neonatal lupus

Hello everyone!

Did you know? Anti-Ro/SSA antibodies are associated with neonatal lupus (congenital heart block (CHB), neonatal transient skin rash, hematological and hepatic abnormalities).

How do I remember this? 

Mixed Connective Tissue Disease : An Overview

Hi everyone ! Just a short post reviewing MCTD! References are Harrison's and Medscape!

Mixed Connective Tissue Disease (MCTD)

1. What is it ?
- It's a somewhat ambiguously used term for disease characterised by a collection of few symptoms from different autoimmune connective tissue disorders.

- Namely , features of Systemic Sclerosis (SSc) , Lupus , Myositis and sometimes RA are present in some proportion in the same patient.
________________________________________
2. What are its chief presenting features ?

A. Features of SSc :
- Raynaud's is often the presenting feature. May also get edematous fingers.
- Dactylitis and digital gangrenes may be + due to Raynaud's
- Sclerodactyly
- Other Limited Cutaneous SSc features like CREST.

B. Features of Lupus :
- Arthritis
- Photosensitivity and Malar rash
- Evidence of Anti phospholipid Antibody Syndrome - associated with SLE. 

C. Features of Myositis :
- Proximal myopathy features
- Muscle tenderness
- May get Cutaneous features of Dermatomyositis.

Also may have Pulmonary Hypertension, Pulmonary Fibrosis.
________________________________________
3. What are the criteria for diagnosis ?

• Immunologically = Anti U1 RNP +

• Clinically = ( any 3 )

Mnemonic = A REM Sleep

Acrosclerosis
Raynaud's
Edematous Hands
Myositis (proven)
Synovitis-Arthritis

________________________________________
4. When to suspect MCTD?
- When a Patient comes with features of Limited Scleroderma (Raynaud's) , but not enough to fulfill its criteria ;

- SSc specific antibodies are not generally positive. (Anti Histone or Topoisomerase) ;

- Suspected SSc patients with  unusually prominent features of Arthritis, Muscle Pain or Rash

________________________________________
5.What tests would one order if suspecting MCTD ?

A. CBC with ESR -
May see Leucopenia with fairly elevated ESR

B. Serology -

• Confirm absence of SSc - Anti Centromere and Anti Topoisomerase.

• For MCTD -
U1 RNP Ab's are fairly specific

• For Myositis -
Anti Jo Ab's (Especially polymyositis)
Anti Mi Ab's (Especially Dermatomyositis)

• For Lupus -
ANA
Complement levels.

• For RA -
Anti CCP Ab's

C. Blood profile -
• Creatine Kinase - Elevated in Myositis
• Urine Routine + Microscopy for Lupus Nephritis type changes
• Electrolytes

D. For Complications -
• Chest X Ray for any ILD or fibrosis
• HRCT if needed.
• Pulmonary Function Tests
• MRI Brain for multi infarct lesions if APLA is + and if neurological changes are +
• ECG - For myocarditis
________________________________________
6. How is the treatment like ?

- NSAIDs - Symptomatic Relief.
- Steroids confer some Relief unlike in SSc. So it's important to differentiate the two!
- Hydroxy chloroquine and Methotrexate may be used to keep disease activity in check.
- Treatment of complications.

Hope this was helpful!
Happy Studying!
Stay Awesome!

~ A.P.Burkholderia

USMLE Step 3 CCS: Kawasaki disease

Hello, these are my hypothetical orders for KD.
Let me know if I missed something out!

Physical examination (PE)

CBC
BMP
ESR
CRP
Blood culture (to rule out infection)
Urinanlysis (to rule out infection)
Urine culture (to rule out infection)
CXR (to rule out infection)

LFT
ASO
Strep pharyngitis culture
EKG
Echocardiogram

IVIG
Aspirin

That's all!
-IkaN

Fact of the day: Sleep Apnea linked with Acute Gout attacks

Hey Awesomites

Those who suffer from sleep apnea are usually overweight, and so may be those with acute gout exacerbations.

In addition to lower body temperature and nighttime dehydration, hypoxic patients of sleep apnea are at upto 50% higher risk of having acute attacks of gout at night. This is due to excess tissue damage and cell breakdown, both of which increase uric acid levels that may accumulate in joints to cause exacerbations !!

- Jaskunwar Singh

Vasculitis Classification : A way out

Here's my way out of the necrotising vasculitis. Pan intended ;;).

So depending on the size of the vessel involved you can classify it as -

1. Large Vessel Vasculitis
2. Medium Vessel Vasculitis
3. Small Vessel Vasculitis

For the first two Remember :

TT PK

So Large vessel = TT
Takayasu Arteritis
Temporal Arteritis (Giant cell Arteritis)

And Medium Vessel = PK
Polyarteritis Nodosa
Kawasaki disease

Now Small Vessel Vasculitis can be classified further. It can be positive for a particular antibody called ANCA or it can be negative. This antibody is purely an association and not a causative one.

ANCA Negative : HCC

H - Henoch Schonlein Purpura
C - Cryoglobulinemia related
C - Cutaneous Leukocyto-angiitis

(Think of crying babies.
Cry = Cryoglobulinemia related Vasculitis
And another baby related Vasculitis​ is HSP).

Now ANCA positive Vasculitis can be either c-ANCA or p-ANCA depending on what part it stains.

Remember - WC
(Like Western commode)
Wegner's is c-ANCA.

Remember - PCM
p-ANCA in Churg Strauss and Microscopic Poly-angiitis.

Please note - The names of Wegner's and Churg Strauss have been changed to Granulomatosis with Polyangiitis , and Eosniophilic GPA respectively. This is said  to be because the scientist Wegener had been discovered to be possibly related to some Nazi war time activities and this opportunity was made the most of to also take a step towards  a more pathology-specific name-calling by altering the honorific name given to the disease.

That's all!
Happy studying.
And Stay Awesome !

~ A.P.Burkholderia.

Fact of the day: Ferritin is an inflammatory disease marker

Ferritin is an acute phase reactant.

Serum ferritin arises from damaged cells, and is thus a marker of cellular damage.

Rheumatoid Arthritis deformities : Mnemonic

Hi everyone. This is just a mnemonic post on RA deformities.

So there are 2 important deformities that are definitely seen clinically but are hard to remember.
The Boutonniere and the Swan neck deformities both occur as some defect at 3 joints :
The Proximal Interphalangeal Joint (PIP)  and the Distal Interphalangeal Joint (DIP) and Metacarpophalangeal Joint (MCP).

So Remembering one of them is sufficient as the other one would be the exact inverse.

Remember :

Bout.on.ni.e.re

So Out one Ex .
Which means there's extension at DIP (the outer joint). Now after extension , alternate the next joint with flexion ( so Flexion at PIP ) followed by extension again ( so extension at MCP )

So , Boutonniere is -
DIP - Extension (out one is ex)
PIP - Flexion
MCP - Extension.

And Swan neck would be the opposite :
DIP - Flexion
PIP - Extension
MCP - Flexion

Hope this helped !
Stay awesome.

~ A.P.Burkholderia

Step 2 CK: Differentials of proximal muscle weakness

Hello!

Here's a short post about proximal muscle weakness!

Psoriatic arthritis mnemonic

A simple one: PSORIATIC

P- Pencil-in-cup deformity

Pencil-in-cup deformity

S- Sausage-like digits

O- Onycholysis and Onychodystrophy

R- Rheumatoid factor negative

I- Ivory phalanx (increased bone density)

A- Arthritis multilans

T- Telescopic fingers

I- Itchy skin

C- Cold weather (more severe)

Thats all

- Jaskunwar Singh

Churg Strauss Syndrome

Hello awesomites, I am kind of obsessed with fancy syndromes. So here is one of them.

Churg Strauss Syndrome (CSS) also known as Eosinophilic granulomatosis with polyangitis or allergic granulomatosis.
It is a rare  autoimmune condition, that causes inflammation of small and medium sized blood vessels.

Manifests in 3 stages-
Early stage (Prodromal stage) : Present as
Asthma or
Allergic Rhinitis
Sometimes with nasal polyps and sinusitis
(Remember 'A'  is the first letter, so it should always come first)

Second stage : Abnormally increased eosinophils= Hypereosinophilia
Which causes tissue damage mostly lung and digestive tract.
Manifestations are Night sweats, weight loss, cough, abdominal pain, GI bleeding, fever, purpura

Third stage: vasculitis- which leads to infarction which further leads to atrophy
Further progression leads to complications.
But not all patients develops all three stages, or progress in the same order.

Pathophysiology - Its a Autoimmune disorder where different cell types are responsible for immune response especially Eosinophils, T&B cells, endothelial and epithelial cells. Mainly it is Th2 mediated reaction.

Complications can be life threatening -
(Most Grievous)
M- Myocardial involvement is the most common complication and most common cause of death in CSS patients
G- GI bleeding, GI perforation, Glomerulonephritis, Glomerulosclerosis,
Granulomatous appendicitis

Treatment - Conventional treatment includes glucocorticoids like Prednisolone and immuno suppressive drugs like Azathioprine, cyclophosphamide.
Newer drugs direct against specific cytokines like mepolizumab have additional steroid sparing property angood tolerability. Use of  Rituximab is under investigation and limited to few cases.

That's all.  :)

Rheumatoid arthritis mnemonic

Hello!

Here's an old mnemonic on some of the clinical features of Rheumatoid arthritis.
Well, the name itself tells it.. RHEUMATOID ;p

R- Respiratory distress (Interstitial lung disease, bronchiolitis, pleural effusion)
H- Haematological manifestations (anemias, thrombocytosis, neutropenia)
E- Extra- articular RA (ExRA)
U- Urinary tract infections (mainly drug- induced)
M- Median nerve compression/ Morning stiffness
A- Amyloidosis
T- Tenosynovitis and bursitis
O- Ocular manifestations (Keratoconjunctivitis sicca, scleritis, episcleritis)
I- Immunologic manifestations (Sjogren's, Felty's and Caplan's syndrome)
D- Deformities (boutonniere, swan- neck, button- hole)

Thats all :)

- Jaskunwar Singh

Polyarteritis nodosa mnemonic

Heyy!

Polyarteritis nodosa is a necrotizing vaculitis of small and medium sized muscular arteries.

Behcet's syndrome mnemonic

Hey guys! 

So I wanted to write something about Behcets and created a lame mnemonic for it.

Let's start :D