Discoid Lupus Erythematosus

Discoid lupus erythematosus — It is estimated that 15 to 30 percent of patients with SLE develop DLE . Patients with localized or generalized DLE are estimated to have cross-sectional prevalences of concurrent SLE between 5 and 28 percent.

The presence of DLE lesions among patients with SLE may modify the risk of specific SLE features. Compared with SLE patients without DLE, those with DLE have increased risk for photosensitivity and leukopenia but decreased risk for serositis and arthritis. There is no obvious change in risk of nephritis despite variable reports of a "renal-protective effect" of the presence of discoid lesions among SLE patients.

Data on the risk for progression of DLE to SLE are limited to retrospective cohort studies, studies lacking power to detect statistical significance of potential markers of progression, and studies that do not address DLE specifically as a CLE subset. In these studies, progression to SLE has occurred in 0 to 28 percent of patients initially presenting with DLE . Progression to SLE often is delayed; in a Swedish-based population cohort study, 10 percent of patients with DLE who subsequently developed SLE did so within the first year and 17 percent developed SLE within the first three years . Two retrospective studies have suggested SLE develops within five years in 50 percent of the DLE patients who indeed go on to develop SLE . Risk factors for progression include an increasing number of clinical and serologic features of SLE: more widespread DLE lesions, arthralgias and arthritis, high antinuclear antibody (ANA) titers, leukopenia, and high erythrocyte sedimentation rates .

It is worth noting that patients with DLE and other mucocutaneous manifestations of LE may meet ACR classification criteria for SLE without having other end-organ disease . Revised classification criteria (the SLICC criteria) have been proposed to address some limitations of the ACR criteria.

●Clinical manifestations – The classic findings of DLE are discrete, erythematous, somewhat indurated plaques covered by a well-formed adherent scale that extends into dilated hair follicles (follicular plugging). The plaques tend to expand slowly with active inflammation at the periphery and then heal, leaving depressed central scars, atrophy, telangiectasias, and hyperpigmentation and/or hypopigmentation . DLE most often involves the face, neck, and scalp but may also occur on the ears (particularly conchal bowls) and, less frequently, on the upper torso . Localized DLE is limited to sites above the neck. Generalized DLE refers to DLE occurring both above and below the neck.

Hypertrophic DLE is an uncommon clinical variant of DLE characterized by the development of hyperkeratotic, verrucous plaques

Fun Fact : SEAL, the famous singer suffers from DLE.

https://en.m.wikipedia.org/wiki/Kiss_from_a_Rose

Bhopalwala. H

Study group discussion: Systemic Lupus Erythematosus

*Review question session on SLE*

Which is the most sensitive antibody?
ANA

Most specific?
Ds DNA

Drug induced lupus?
Anti histone

I have mnemonics on these!

Please share!

http://immense-immunology-insight.blogspot.ae/2013/12/its-never-lupus-mnemonics.html

Most common type of lung involvement in SLE?
Pleurisy

Skin changes in SLE?
Malar rash
Discoid rash

Butterfly rash, discoid lesions

And?

Photosenstivity

Good.

How do you differentiate between discoid lupus and SLE?
Discoid lupus is a milder form of SLE.

I will approach the question in a different way.. Do we do skin biopsy in SLE?
Yes.

And what test we do?
Band test.
Correct!

Where?? Which level of the skin?

Between dermis and epidermis.

Dermo-epidermal junction. Correct!

So what do you think will be the difference in DLE and SLE?

Skin biopsy shows a green band under fluorescence.
In DLE..you will have a positive band test only in regional areas.
Whereas in SLE..the test is common all over the body, and not only the affected areas.

Ok so this differentiates DLE vs SLE.

Never heard about this thing. Thanks all!

This crazy skin test.

I didn't know this either. Amazing.

Also, nephritis is much more common in SLE.
Wire loop deposit.
Great!!

Which drugs cause drug induced SLE?

There is a very big list for sure.

The most common causes to remember are
1) Procainamide
2) Hydralazine
3) Isoniazid

Easy question would be..Which drugs don't cause SLE.

Yes. Because they are related to acetylators. The slow and fast acetylators.

Can you explain I mean how does it effect? The slow and fast acetylators?

I'm not sure.. But the slow acetylators are more prone to DILE. I'll cross check and let you know

Slow acetylators metabolize the drug slowly.. Hence a higher chance of toxicity.

Presumably, this is because acetylation of the aromatic amine or hydrazine functional group leads to a non-toxic product. Several other drugs which have been implicated in drug-induced lupus also contain an aromatic amine or hydrazine group. The clinical and laboratory characteristics of drug-induced and idiopathic lupus are similar but the degree to which the pathophysiological mechanisms are related, if at all, is unknown.
Source: http://www.ncbi.nlm.nih.gov/pubmed/7011656

Complex.

Ok so which symptoms you won't see in drug induced lupus?

Donno.. I know they'll disappear on discontinuation of the medication.

You won't see
CNS involvement and renal involvement in drug induced.

One last.

What happens to complement levels in lupus flare up?

Decreases.

Brilliant.

And what happens to dsDNA in flare up?

And what about levels of complement and anti ds Dna in drug induced lupus?

Anti dsDNA levels decrease in the lupus flare up.

Lol hope I am not bugging you guys!! Haha so I will answer the last one!!

Oh you're not. Medicine is addicting.
If we knew the answers we'd be jumping and answering :P

Haha yeah medicine is addicting once you get to know some of it.
You just can't back off! If when you have learnt there is much more that you don't know!

Complement levels and anti dsDNA levels are normal in drug induced lupus.

They do have positive ANA.

Ah. Makes sense.

Alright guys! It was wonderful! Keep learning medicine.
And keep rocking!

Labs in SLE

Laboratory testing — We obtain the following routine laboratory tests, which may provide diagnostically useful information:

●Complete blood count and differential may reveal leukopenia, mild anemia, and/or thrombocytopenia

●Elevated serum creatinine may be suggestive of renal dysfunction

●Urinalysis with urine sediment may reveal hematuria, pyuria, proteinuria, and/or cellular casts

In addition to the routine laboratories described above, we perform the following laboratory tests which support the diagnosis of SLE if abnormal:

●ANA

●Antiphospholipid antibodies (lupus anticoagulant [LA], IgG and IgM anticardiolipin [aCL] antibodies; and IgG and IgM anti-beta2-glycoprotein [GP] I)

●C3 and C4 or CH50 complement levels

●Erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) levels

●Urine protein-to-creatinine ratio

The ANA test is positive in virtually all patients with SLE at some time in the course of their disease . If the ANA is positive, one should test for other specific antibodies such as dsDNA, anti-Sm, Ro/SSA, La/SSB, and U1 ribonucleoprotein (RNP). In some labs, a positive ANA test by indirect immunofluorescence will automatically result in testing for such additional antinuclear antibodies that are often present in patients SLE.

●Anti-dsDNA and anti-Sm antibodies are highly specific for SLE, but anti-Sm antibodies lack sensitivity . Anti-dsDNA and anti-Sm antibodies are seen in approximately 70 and 30 percent of patients with SLE, respectively.

●Anti-Ro/SSA and anti-La/SSB antibodies are present in approximately 30 and 20 percent of patients with SLE, respectively; however, both antibodies are more commonly associated with Sjögren's syndrome.

●Anti-U1 RNP antibodies are observed in approximately 25 percent of patients with SLE, but they also occur in patients with other conditions and high levels are almost always present in patients with mixed connective tissue disease (MCTD).

●Antiribosomal P protein antibodies have a high specificity for SLE, but have low sensitivity for SLE. They also lack specificity for involvement of a particular organ system or disease manifestation.

If the initial ANA test is negative, but the clinical suspicion of SLE is high, then additional antibody testing may still be appropriate. This is partly related to the differences in the sensitivity and specificity among the methods used to detect ANA. A more detailed discussion on the techniques used to detect ANA is presented separately.

Fun fact : Anti-Sm antibody was actually named after a patient with Lupus, Mr. Smith.

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

Skin Findings in Dermatomyositis

Skin findings — Several distinct cutaneous eruptions, which are generally evident at the time of clinical presentation, occur in DM but not in PM . Other skin changes may occur in patients with PM and in patients with DM and are not specific to either disorder. Dermatologic manifestations may be prominent but can be quite subtle in some patients.

Characteristic dermatomyositis findings — Gottron's papules and the heliotrope eruption are the hallmark and likely pathognomonic features of DM. Gottron's sign, photodistributed erythema, poikiloderma, nailfold changes, scalp involvement, and calcinosis cutis are also characteristic and useful in distinguishing DM from PM.

●Gottron's papules – Gottron's papules are erythematous to violaceous papules that occur symmetrically over the extensor (dorsal) aspects of the metacarpophalangeal (MCP) and interphalangeal (IP) joints (picture 1A-C). In addition, these lesions may involve the skin between the MCP and IP joints, particularly when the eruption is prominent. Gottron's papules often have associated scale and may ulcerate. When scaling is present, the lesions may mimic psoriasis or lichen planus.

●Gottron's sign – Definitions used for Gottron's sign have varied in the literature. We define Gottron's sign as the presence of erythematous to violaceous macules, patches, or papules on the extensor surfaces of joints in sites other than the hands, particularly the elbows, knees, or ankles. By contrast, some authors have used the term Gottron's papules to refer to papules in these areas, reserving Gottron's sign for macular or patch-like lesions (picture 2) .

●Heliotrope eruption – The heliotrope eruption is an erythematous to violaceous eruption on the upper eyelids, sometimes accompanied by eyelid edema, which, at times, may be quite marked .

●Facial erythema – Patients may have midfacial erythema that can mimic the malar erythema seen in SLE . In contrast to those with SLE, patients with DM will often have involvement of the nasolabial fold, which can be helpful in distinguishing these two photosensitive midfacial eruptions.

●Photodistributed poikiloderma (including the shawl and V signs) – Poikiloderma refers to skin that demonstrates both hyperpigmentation and hypopigmentation, as well as telangiectasias and epidermal atrophy. In DM, patients may demonstrate poikiloderma in any photo-exposed site; however, classic areas of involvement are the upper back (shawl sign) and the V of the neck and upper chest. The poikiloderma in DM often presents with a violaceous hue. Early in the course of cutaneous disease, these areas may demonstrate only erythema rather than well-developed poikiloderma . The erythema may be macular (nonpalpable) or papular. In rare patients, these lesions become thickened and resemble papular mucinosis. The cutaneous eruption of DM is often associated with significant pruritus, which may assist in distinguishing its photo-exacerbated eruption from that of lupus erythematosus (LE).

●Holster sign – Patients with DM may also have poikiloderma on the lateral aspects of the thighs, referred to as the "Holster sign" . It is unclear why this cutaneous manifestation occurs on this classically photo-protected site.

●Generalized erythroderma – In rare patients, erythroderma may occur, which involves extensive cutaneous surface area, including areas that are less exposed to ultraviolet light.

●Periungual abnormalities – The capillary nail beds in DM may be erythematous and may show vascular changes similar to those observed in other systemic rheumatic diseases (eg, scleroderma and SLE). Abnormal capillary nail bed loops may be evident, with alternating areas of dilatation and dropout and with periungual erythema . In addition, cuticular overgrowth, sometimes termed "ragged cuticles," is characteristic and may be associated with hemorrhagic infarcts within the hypertrophic area . The degree of cuticular involvement is thought to reflect ongoing cutaneous disease activity, representing active vasculopathy .

●Psoriasiform changes in scalp – Changes in the scalp resembling seborrheic dermatitis or psoriasis occur in a high percentage of patients with DM . The scalp involvement in DM is diffuse, often associated with poikilodermatous changes and with prominent scaling. Scalp involvement may result in severe burning, pruritus, and/or sleep disturbance. In addition, severe pruritus may occur in patients without visible disease.

●Calcinosis cutis – The deposition of calcium within the skin, a finding known as calcinosis cutis, occurs commonly in juvenile DM. It is infrequent in adult DM. In children, calcinosis has been associated with a delay in treatment with glucocorticoids and/or immunosuppressive therapy. Calcinosis cutis, which is known to be very challenging to treat, may be seen in a variety of conditions, including SSc, particularly limited cutaneous SSc; SLE (rarely); and overlap connective tissue disorders. It may be more common in patients with DM with the anti-p140/anti-MJ autoantibody

Bhopalwala. H

Belimumab mnemonic

What is belimumab?

Belimumab is a  monoclonal antibody directed against soluble B lymphocyte stimulator (BLyS).

Belimumab is used in the treatment of? 

Systemic Lupus Erythematosus (SLE)

Mnemonic: Belly Selly SLE (rhymes! sing it enough times and you will never forget)

At present, belimumab is indicated as add-on therapy in adults with active, antinuclear antibody or anti-dsDNA-positive SLE with a high degree of disease activity in the skin and/or musculoskeletal systems that remain moderately to severely active despite optimized standard immunosuppression. 

Patients with severe lupus nephritis or active CNS lupus are not the candidates for belimumab.

That's all!

-IkaN

HLA subtype associated diseases mnemonics

Hi everyone!
Here's a complete guide on how to remember the HLA associations. Let's catch em all!

Doctors (DR) will turn into MD's someday.. The thought makes you go, "Aah"
Starting with 2 and ending with 5, your mnemonic for DR associations is "MD.. AAH!"
HLA DR2 - Multiple sclerosis
HLA DR3  - Diabetes mellitus type 1
HLA DR4  - Arthritis (Rheumatoid, also the one associated with Lyme's disease)
HLA DR5  - Anemia (Pernicious, causing B12 deficiency)
HLA DR5  - Hashimoto's thyroiditis

SLE vs RA

The arthritis in SLE may look very similar to RA.

The main difference is that it is non erosive, unlike RA.

Pearls of wisdom :

When treating chronic conditions like Rheumatoid Arthritis, Osteoarthritis, SLE, Fibromyalgia, Psoriasis, and Psoriatic Arthritis, you've got to involve the patient in the care. You've got to explain to them that these are  chronic conditions with no cure. Goals should be damage control and remission.

A good strong patient doctor relationship when dealing with these conditions, works better than any pill on planet Earth.

Credits : Dr.G

Bhopalwala. H

Mnemonic for Antibodies in SLE & it's Clinical Importance

Hi everyone!

Here is a hack.

ANA = All Negative Absent = All Positive identified = Highest Sensitivity ( So best Screening Test ) 

Anti dsDNA = Disease Severity 

Anti Sm = Specific Most

Anti RNP = Raynaud & Polymyositis ( MCTD )

Study group discussion: Ferritin

Hello everyone!


Why do ferritin levels increase in Rheumatoid arthritis?
It is an acute phase protein.
The levels increase in serum as well as in synovial fluid. The rise is more in synovial fluid because of local production of ferritin in the inflamed joint.
Ferritin levels show a positive correlation with ESR, CRP, platelet count, and DAS score.
And a negative correlation with hematocrit levels.

Is there a difference of levels in inactive and active disease?
Yes, the levels are lower in inactive RA patients because of iron deficiency.
Synovial ferritin production in active RA leads to increase of ferritin in active disease.

Gold standards for diagnosis of iron deficiency in RA?
1. Bone marrow iron stain
2. Serum transferrin receptor analysis

What leads to high ferritin levels in SLE?
Ferritin synthesis is induced by interleukins IL-1, IL-6 and Tumor Necrosis Factor (TNF) alpha in hepatocytes.  In SLE, there is defect in IL-1 production while IL-6 and TNF-alpha levels are increased. So, the high levels of ferritin are due to IL-6 and TNF-alpha. (IL-1 does not play a major role in the synthesis of ferritin)
The ferritin levels are correlated with ANA titre, anti-dsDNA titre, and SLEDAI score.
(No significant correlation with acute phase parameters and negative correlation with complement levels)

Mnemonico diagnostico : SLE revised 2012 diagnostic criteria

Hey Awesomites

SLICC classification criteria for Systemic Lupus Eryhtematosus mnemonic :

COVID-19: Hydroxychloroquine mechanism and role in management of SARS-CoV-2 infection

Hello everyone, this post aims to highlight all the important aspects of the recently famous drug hydroxychloroquine in the management of COVID-19.

Mechanism of action: In a study by Aartjan et al, zinc ions (Zn2+) in high intracellular concentrations have been shown to inhibit viral RNA polymerase. However, zinc being an ion cannot enter the cell through the plasma membrane, so it needs ionophores such as pyrithione (PT) to enter the cell, where, in high concentrations, it can efficiently impair the replication of a variety of RNA viruses. Chloroquine can also act as an ionophore that can increase zinc ions transport into the cell.
According to Harrison’s principles of internal medicine, “Infection of tissue culture cells by viruses such as Semliki Forest virus, vesicular stomatitis virus, and certain strains of influenza virus can be prevented by chloroquine, an agent that blocks the function of lysosomes. Chloroquine is a weak base that diffuses into lysosomes and becomes protonated, raiding the pH and ionic strength of the lysosome. When the pH rises, the lysosomal enzymes fail to function. Viruses that require acid pH to fuse with cell membranes can no longer do so in the presence of chloroquine, and the cells are protected from infection.”

Studies revealed that it also has potential broad-spectrum antiviral activities by increasing endosomal pH required for virus/cell fusion, as well as interfering with the glycosylation of cellular receptors of SARS-CoV. The anti-viral and anti-inflammatory activities of chloroquine may account for its potent efficacy in treating patients with COVID-19 pneumonia.

Chloroquine can also prevent orf1ab, ORF3a, and ORF10 from attacking the heme to form the porphyrin and inhibit the binding of ORF8 and surface glycoproteins to porphyrins to a certain extent, effectively relieving the symptoms of respiratory distress. The infectivity of the nCoV pneumonia was not completely prevented by the drugs, because the binding of E2 glycoprotein and porphyrin was not inhibited. You can read more about this on our previous post on: Coronavirus and hemoglobin https://www.medicowesome.com/2020/04/covid-19-coronavirus-and-hemoglobin.html

Current place in the management of COVID-19

1. In India, ICMR has recommended this drug for prophylaxis to healthcare workers dealing with infected patients and asymptomatic contacts of infected people at a dose of 400 mg per week. Besides AIIMS(New Delhi) has recommended this drug for the treatment of moderate to severe cases who are admitted in the hospital at a dose of 400 mg BD for 1 day which is followed by 200 mg BD for 5 days.

2. Chen et al in an unpublished RCT of 30 patients did not find HCQ provided benefit. The study suggests that if it has an impact, it is likely small. 

3. Gautret et al in a non-RCT of 36 patients suggested that HCQ reduced the duration of viral shedding in infected patients. 6 patients in a post-hoc analysis who received HCQ in combination with azithromycin showed further reduction in the viral carriage. However, this was not statistically significant and groups were not well balanced at baseline. 

4.  Chen et al in a double-blind RCT of 62 patients showed that HCQ can significantly shorten the time to clinical recovery and promote the absorption of pneumonia among patients with COVID-19. However, this study has not yet been certified by peer review. 

5. The Marseille study, an unblinded, non-randomized study of 26 infected patients showed a significant reduction in viral load with HCQ. And the number of positive cases was spectacularly reduced by the combination of HCQ with azithromycin. However, this study was full of flaws, there wasn’t adequate matching between the two groups, there were 6 dropouts who weren’t accounted in the study, patients in the control group didn’t have uniform testing, and the patients in the HCQ group had more severe symptoms and were further along in their clinical course. Apparently, this was the study, based on which President Trump promoted the use of HCQ!

6. The patients taking HCQ should be closely monitored for toxicity, in particular, QT prolongation; especially if it is used with azithromycin. Combining lopinavir/ritonavir with HCQ or chloroquine can cause serious arrhythmias and drug interactions due to the increased QT interval. 

Effect of the pandemic on drug supplies for Rheumatology patients

Hydroxychloroquine has been in use since the 1940s for the treatment of rheumatological conditions such as RA, SLE, and Sjögren’s syndrome. The sudden interest in this drug has led to shortages for patients who rely on it for the treatment of their autoimmune conditions. The Lupus Foundation of America has called on drug manufacturers to increase the production of HCQ, in order to ensure that patients with SLE are still able to access it without much difficulty.

Overall, no agent has proven efficacy for COVID-19. A number of approaches are being investigated based on in vitro or extrapolated evidence, including remdesivir, hydroxychloroquine, chloroquine, interleukin-6 pathway inhibitors, and convalescent plasma. When treatment of COVID-19 is being considered, patients should be referred to a clinical trial whenever possible. A registry of international clinical trials can be found at clinicaltrials.gov. 

Thank you! :) 

-Vinayak

References:

1. CHEN J. ,LIU D. et al. A pilot study of hydroxychloroquine in treatment of patients with common coronavirus disease-19 (COVID-19). J Zhejiang Univ (Med Sci), 2020, 49(1): 0-0.
2. Gautret P, Lagier JC, Parola P, et al. Hydroxychloroquine and azithromycin as a treatment of COVID-19: results of an open-label non-randomized clinical trial. Int J Antimicrob Agents. 2020. [PMID:32205204]
3. Wang M, Cao R, Zhang L, et al. Remdesivir and chloroquine effectively inhibit the recently emerged novel coronavirus (2019-nCoV) in vitro. Cell Res. 2020. [PMID:32020029]
4. Efficacy of hydroxychloroquine in patients with COVID-19: results of a randomized clinical trial. Zhaowei Chen, Jijia Hu, et al. medRxiv 2020.03.22.20040758; doi: https://doi.org/10.1101/2020.03.22.20040758
5.te Velthuis AJ, et al. Zn(2+) inhibits coronavirus and arterivirus RNA polymerase activity in vitro and zinc ionophores block the replication of these viruses in cell culture. PLoS
Pathog. 2010 Nov 4;6(11):e1001176. doi: 10.1371/journal.ppat.1001176. PubMed
PMID: 21079686; PubMed Central PMCID: PMC2973827.

Causes of Acute Pancreatitis mnemonic

Hi!

Causes of Acute Pancreatitis mnemonic :

ABCDEFGHIjK

Classification of Cryoglobulinemia

●The Brouet classification criteria is the most commonly used system that classifies cryoglobulinemia into three different subgroups based on their Ig composition. These classification criteria are also useful in that the subgroups partly correlate with pathogenicity and clinical manifestations.

•In type I cryoglobulinemia, the cryoglobulins are monoclonal Ig, typically IgG or IgM, and less commonly IgA or free Ig light chains. Type I cryoglobulinemia develops in the setting of protein-secreting monoclonal gammopathies such as a monoclonal gammopathy of undetermined significance (MGUS) or a B-cell lineage malignancy (eg, multiple myeloma, Waldenström macroglobulinemia, or chronic lymphocytic leukemia).

•In type II cryoglobulinemia, the cryoglobulins are composed of a mixture of a monoclonal IgM (or IgG or IgA) with rheumatoid factor (RF) activity and polyclonal Ig. Type II cryoglobulins are often associated with persistent viral infections, particularly hepatitis C virus (HCV) infection, and are associated with the mixed cryoglobulinemia syndrome. Other clinical associations with type II cryoglobulinemia include other infections such as hepatitis B virus (HBV), HIV, autoimmune diseases (mainly systemic lupus erythematosus [SLE] and Sjögren's syndrome), and lymphoproliferative disorders.

•In type III cryoglobulinemia, the cryoglobulins are composed of a mixture of polyclonal IgG (all isotypes) and polyclonal IgM. These cases are often secondary to autoimmune disorders, but can also be associated with infections (mainly HCV).

Bhopalwala. H

Type 1 RTA pathophysiology, notes and mnemonic

Hello! This post is on type 1 renal tubular acidosis.

What causes Type 1 RTA?
Defective H+ ion secretion in the distal tubule.
Impairment in H+ ions secretion result in an inability to acidify the pH beyond 5.5 (Used in the diagnosis of type 1 RTA)


The plasma bicarbonate is significantly reduced and may fall below 10 meq/L.
These patients tend to have urinary K+ wasting and hypokalemia (thought to be due to increased potassium secretion by distal tubular cells in the setting of diminished H+ ion secretion.)

What type of RTA is associated with an enhanced chance if nephrolithiasis?
Distal or type 1 RTA can cause nephrocalcinosis / calcium oxalate kidney stones.
Mnemonic: ONE predisposes to stONEs

Pathophysiology: Hypercalciuria, hyperphosphatemia, nephrolithiasis (calcium phosphate stones) and nephrocalcinosis are frequently associated with untreated type 1 RTA. The hypercalciuria is thought to be due to:
1) increased calcium phosphate release from bone as a result of bone buffering of excess acid and
2) reduction in tubular calcium reabsorption secondary to chronic acidosis.
The hypercalciuria, alkaline urine, and reduced excretion of citrate in the urine (which normally prevents calcium crystallization) promote the precipitation of calcium phosphate and stone formation.

Which conditions are associated with type 1 RTA?
diStal RTA is associated with the 3 S's:
Sjogren's
SLE

Sickle cell anemia

Treatment: Bicarbonate administration

That's all!
-IkaN

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

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

Celiac Disease (Spectrum of Manifestations)

 Hello friends! I hope all of you are doing well. Today I wanted to share with you the many faces of Celiac Disease. Although considered as the disease which chiefly causes gastrointestinal symptoms, the entire spectrum of possible manifestations it can cause is quite broad.

Some significant associations are as follows:

1.) GI- Enteropathy associated T-cell lymphoma (EATL), Microscopic colitis

2.) Liver- NASH

3.) Spleen- Functional Asplenia (SLE & Amyloidosis being other notable causes)

4.) CNS- Seizures with posterior cerebral calcification, Neuro-psychiatric symptoms, Ataxia

5.) Hematology- Evans syndrome

6.) Pulmonary- Diffuse alveolar hemorrhage

Here is the full spectrum. Hope you like it.

-Kirtan Patolia

Causes of Acute Pancreatitis

Hi guys,

I have an exam soon and I was struggling with this, so, I made this up!

To remember causes of Acute Pancreatitis, you need to think of none other than your nursery school days! :P

So, here we go- ABCDEFGHI, (oh wait did I just say "HI" :P)

Now going a little off track, but, when I was small I used to do a lot of gardening, so I remember rest of the causes as Me POT.

Alcohol ingestion  (acute or chronic)
Biliary calculus
Connective tissue diseases- SLE, TTP,  PAN
Drugs- diuretics- frusemide, thiazide. (Others are anti retroviral drugs, sulphomamides, tetracycline, tamoxifen, etc)
Endoscopic procedures
Familial/ Genetic
Hereditary Pancreatitis/ Hyperparathyroidism/ hypercalcemia/ Hypertrigylceridemia
Infections- viral

Metabolic conditions - renal failure,
Penetrating Peptic Ulcer
OPC poisoning
Trauma to abdomen

Phew, a long list. Isn't it?

Hope it helps!!
That's it!

-Rippie

Immunohemolytic anemias part-1

Hello awesomites!
This is my very first post, so am starting with my favorite subject Hematology.
Today's post is about Immunohemolytic anemias, commonly ignored type of anemia

Also referred as Autoimmune hemolytic anemias(AIHA)
Where antibodies are responsible for premature destruction of red blood cell.
Types- warm antibody type
            - cold agglutinin type
            - cold hemolysin type

Warm antibody type  -  It is the most common type of AIHA.
you can remember it by mnemonic
" WARM GRILLED "
W - Warm because, antibodies are active  
       at 37°C
A -  Associated with other Autoimmune        
       disorders ( secondary causes like 
       SLE)
R - Red cell hemolysis is mainly
      extravascular
M- Moderate spleenomegaly due to
       hyperplasia of splenic phagocytes      

G- Ig G class - most common causative
      antibodies ( IgA sometimes too)

R- Rh blood group antigens are the main
      target 
I- 50% primary cases are  Idiopathic
       Secondary causes can be

L- Lymphoid neoplasm

ED-  Exposure to Drugs.
Examples - penicillin, cephalosporins, quinidine, methyl dopa etc

Mechanism - A) Antigenic drugs-
Drugs such as penicillin binds to red cell membrane and they are recognized by the antidrug antibody. The antibody either recognizes the drug and bind to it or both drug and membrane protein,ultimately results in hemolysis.

B) Tolerance breaking drugs- In drugs such as methyl dopa, antibodies are formed against red cell antigens particularly Rh antigens.

Stay awesome✌️