Showing posts with label Rheumatology. Show all posts
Showing posts with label Rheumatology. Show all posts

Saturday, May 8, 2021

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

Friday, September 4, 2020

Lover's heel

Lover's heel is a term used for Gonococcal tenosynovitis of the Achilles tendon given the sexually transmitted nature of gonorrhea.

I just wanted to share this interesting fact with you.

-IkaN

Sunday, December 29, 2019

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 )

Monday, November 11, 2019

Extra - articular manifestations of RA ( Notes and mnemonic )

Hi!

Extra- articular manifestations of Rheumatoid arthritis :

( mnemonic - NOVELA is FrickiN' Hot! )

Friday, October 25, 2019

Route of bisphosphonate administration mnemonic

Mini post!

IVZ: Intravenous zoledronic acid (once a year)

oRAl: Oral bisphosphonates are Risedronate and Alendronate

- IkaN

Denosumab

Hi! Long time no see :)

This post is on Denosumab!

MOA:
- Monoclonal antibody against the receptor activator of nuclear factor κB ligand (RANKL)
- Reduces bone resorption by inhibiting the development of osteoclasts

Route: SC
Dosing: Administered twice yearly

Friday, March 8, 2019

Classification Criteria for Adult Still Disease

●Yamaguchi criteria – The Yamaguchi criteria require the presence of five features, with at least two being major diagnostic criteria . In addition, the presence of any infection, malignancy, or other rheumatic disorder known to mimic ASD in its clinical features precludes the diagnosis of ASD, at least for the purpose of research.

The four major Yamaguchi criteria are:

•Fever of at least 39ºC (102.2ºF) lasting at least one week

•Arthralgias or arthritis lasting two weeks or longer

•A nonpruritic macular or maculopapular skin rash that is salmon-colored in appearance and usually found over the trunk or extremities during febrile episodes

•Leukocytosis (10,000/microL or greater), with at least 80 percent granulocytes

The minor Yamaguchi criteria include:

•Sore throat

•Lymphadenopathy

•Hepatomegaly or splenomegaly

•Abnormal liver function studies, particularly elevations in aspartate and alanine aminotransferase and lactate dehydrogenase concentrations

•Negative tests for antinuclear antibody (ANA) and rheumatoid factor (RF)

Bhopalwala. H

Thursday, February 28, 2019

Cogan's Syndrome

●Cogan's syndrome (CS) is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis (IK) and vestibuloauditory dysfunction, and associations between CS and systemic vasculitis, as well as aortitis, also exist. There are a range of pathologic findings, most of which reflect immune-mediated injury of the affected tissues; however, despite an association with systemic vasculitis, eye and inner ear specimens of those with CS do not reveal any evidence of vasculitis. The underlying mechanisms responsible for the eye and inner ear disease in CS are unknown.

●The predominant ocular feature of CS is IK, which typically causes eye redness, pain, photophobia, and blurred vision. Slit-lamp examination commonly demonstrates a patchy, deep, granular corneal infiltrate. IK is not essential for the diagnosis; ocular inflammation may involve other parts of the eye and may lead to iridocyclitis, conjunctivitis, episcleritis, anterior or posterior scleritis, or retinal vasculitis.

●The inner ear manifestations of CS are Ménière-like attacks consisting of vertigo, ataxia, nausea, vomiting, tinnitus, and hearing loss. Vestibular dysfunction may also cause oscillopsia, and caloric testing often reveals absent vestibular function. Recurrent episodes of inner ear disease frequently result in profound sensorineural hearing loss. Noninflammatory down-fluctuations in hearing may be difficult to distinguish from those of inflammatory origin. If hearing loss is associated with eye inflammation or other features of active CS or does not resolve within three to five days, an inflammatory origin is more likely.

●When present, the systemic vasculitis associated with CS is a large- or medium- to small-sized vessel vasculitis or an aortitis. The pattern of vessel involvement may be overlapping. Other systemic manifestations of CS include fever, fatigue, weight loss, lymphadenopathy, hepatomegaly, hepatitis, splenomegaly, pulmonary nodules, pericarditis, abdominal pain, arthralgia, arthritis, myalgia, and urticaria. An association with inflammatory bowel disease has also been observed.

●Evaluation of the patient with possible CS requires ophthalmologic examination to establish the presence of IK, scleritis, or episcleritis and to exclude other diseases and ocular pathology; neurologic and otologic examination to establish the presence of vestibuloauditory abnormalities; and rheumatologic examination to seek evidence of systemic vasculitis. We diagnose CS based upon the presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. The eye and inner ear are nearly equally likely to be the cause of presenting symptoms, while less than 5 percent of patients initially present with systemic manifestations

Bhopalwala. H

Wednesday, February 27, 2019

GPA vs MPA Flares

The distinction between GPA ( Granulomatosis with Polyangiitis) and MPA (Microscopic Polyangiitis) is important chiefly because of differential tendencies to flare. Although both diseases may flare after the achievement of remission, GPA is substantially more likely to relapse.

That's all.

Bhopalwala. H

ANCA titers and Disease flare.

Does a rise in ANCA titers predict a disease flare? — This has been a controversial area in the literature almost since ANCA were first identified in the 1980s. However, several rigorous studies have demonstrated that elevations in the titers of ANCA do not predict disease flares in a timely manner . The largest of these studies was performed on a clinical trial cohort of 180 patients (Wegener's Granulomatosis Etanercept Trial [WGET] Research Group, 2005), with serum samples drawn at three-month intervals and ANCA assays performed at the Mayo Clinic . The following findings were observed:
●Among patients who were PR3-ANCA positive compared with negative at baseline, there were no differences in the median time to relapse, disease activity score , or organ involved at relapse. Decreases in PR3-ANCA levels were not associated with a shorter time to remission, and increases were not associated with relapse.
●Relapses occurred among 46 of 101 patients (46 percent) who were mature-PR3-ANCA positive at baseline and achieved remissions of at least six months' duration. However, the proportion of patients who experienced a disease flare within one year of an elevation in ANCA titer was only 40 percent.
Other studies have come to slightly different conclusions, indicating that persistently high or rising titers of ANCA are associated with an increased risk of disease relapse . However, even in those studies, the temporal relationship between a rise in ANCA titer and the occurrence of a disease flare was poor. As an example, in a prospective study of 100 ANCA-positive patients observed over a two-year period, relapse did not occur in 43 and 29 percent of those with a rise in ANCA titers by immunofluorescence and in PR3-ANCA titers by ELISA, respectively .
In addition, a meta-analysis of 18 studies found that neither a rise in ANCA titer nor a persistently elevated ANCA titer were strong predictors of a subsequent disease flare . Therapies for relapsed ANCA-associated vasculitis (often, high doses of glucocorticoids and cytotoxic agents) carry substantial risk, including severe infections, cystitis, bladder cancer, lung fibrosis (rarely), and death. Treating all patients with increases in ANCA titers would result in unnecessary risks of toxicity in a substantial percentage of patients, nearly 30 percent in the study mentioned above. Because of these concerns, using a rise in ANCA titer as the sole parameter to justify altering immunosuppressive therapy cannot be endorsed.
A reasonable recommendation is to closely follow patients with rising ANCA titers but not to alter their therapy unless there are clear clinical signs of active disease.

Bhopalwala. H

POTS Syndrome

●The postural tachycardia syndrome (POTS) is defined as a form of orthostatic intolerance characterized by an excessive increase in heart rate that occurs on standing without arterial hypotension.

●The etiology of POTS is not clear, but the disorder may be heterogeneous. Abnormalities in autonomic regulation that may either be genetic or acquired are described. Proposed mechanisms include partial sympathetic denervation leading to discordant cardiac and vascular sympathetic control, hypovolemia and impairment of the renin-angiotensin-aldosterone system, venous abnormalities and baroreflex dysfunction.

●The clinical symptoms of POTS are varied and nonspecific, and include dizziness, lightheadedness, weakness, blurred vision, and fatigue upon standing. The orthostatic nature of the symptoms is the primary clue to the diagnosis.

●The diagnosis of POTS is established from the history and head-up tilt testing which demonstrates a heart rate increase of >30 bpm over baseline or to >120 bpm. Dehydration, prolonged bedrest, medications, and other dysautonomias should be excluded as etiologies.

●The optimal therapy of POTS is not established. Patients should avoid precipitating factors, and physical activity should be encouraged. We suggest volume repletion and fludrocortisone (0.05 to 0.2 mg per day) as the first line of therapy . Some patients may benefit from midodrine or beta blocking agents. Other therapies remain under investigation, and further confirmation of benefit is needed before they can be recommended.

Bhopalwala. H

Tuesday, February 26, 2019

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

Eye Findings in GCA

●Anterior ischemic optic neuropathy – At least 80 percent of cases of vision loss in patients with GCA are caused by AION . The ischemic insult in arteritic AION is typically the consequence of occlusion of the posterior ciliary artery, a branch of the ophthalmic artery from the internal carotid artery, and the main arterial supply to the optic nerve.

Only about five percent of the total occurrences of AION are due to GCA, the majority being nonarteritic and secondary to atherosclerotic disease . About 40 percent of patients who suffer nonarteritic AION regain some amount of visual acuity, in contrast to visual loss due to GCA, which is more often massive and irreversible .

●Central retinal artery occlusion – CRAO is responsible for approximately 10 percent of the cases of visual loss in GCA . On the other hand, approximately two percent of older patients with CRAO have underlying GCA . Bilateral CRAOs in an older adult should prompt evaluation for GCA.

●Posterior ischemic optic neuropathy – PION occurs in less than five percent of patients with GCA . It results from the interruption of blood flow to the retrobulbar portion of the optic nerve. Histopathologic examination typically reveals inflammatory occlusion of the short nutrient posterior ciliary arteries .

●Branch retinal artery occlusion – BRAO is distinctly uncommon in GCA, though it has been described.

●Cerebral ischemia — Homonymous hemianopia is a visual field defect involving either the two right or the two left halves of the visual fields of both eyes. The most common cause in GCA is an occipital lobe infarction resulting from a lesion in the vertebrobasilar circulation. In rare cases, bilateral occipital lobe involvement leads to bilateral homonymous field defects and to the development of cortical blindness.

Bhopalwala. H

Imaging Findings in PMR

Imaging —

As discussed above, there are characteristic features of periarticular structures (eg, bursitis and tenosynovitis) that can be seen on ultrasonography, magnetic resonance imaging (MRI), and positron emission tomography (PET) . Routine radiographs do not show abnormalities in patients with PMR.

Ultrasound (US) and MRI can demonstrate synovitis of the glenohumeral and hip joints and frequent involvement of extraarticular structures, especially the subacromial/subdeltoid bursa, long head of the biceps, and trochanteric bursa. While subdeltoid/subacromial bursitis is a characteristic imaging feature of PMR, it is not specific and is seen in patients with rheumatoid arthritis (RA) and other shoulder pathology .

Bhopalwala. H

How to Diagnose Polymyalgia Rheumatica?

General approach — There is no pathognomonic test or established diagnostic criteria for polymyalgia rheumatica (PMR). We use the presence of all of the following empirically formulated criteria for the clinical diagnosis of PMR in whom another disease to explain the findings is not present :

●Age 50 years or older at disease onset.

●Proximally and bilaterally distributed aching and morning stiffness (lasting at least 30 minutes or more) persisting for at least two weeks. The stiffness should involve at least two of the following three areas: neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs.

●Erythrocyte sedimentation rate (ESR) ≥40 mm/hour.

●Rapid resolution of symptoms with low-dose glucocorticoids. Symptoms are generally 50 to 70 percent better within three days in patients with PMR started on prednisone at a dose of 10 to 20 mg/day, and almost all patients respond completely within three weeks of beginning treatment. The lack of response to initial therapy strongly suggests an alternative diagnosis. Symptomatic improvement with low-dose glucocorticoid treatment can also be seen in patients with rheumatoid disease, psoriatic arthritis, and other inflammatory arthritides.

Bhopalwala. H

Adalimumab (Humira)

Use

Ankylosing spondylitis: Treatment (to reduce signs/symptoms) of active ankylosing spondylitis in adults

Crohn disease: Treatment (to reduce signs/symptoms and to induce and maintain clinical remission) of active Crohn disease (moderate to severe) in adults and pediatric patients ≥6 years of age (Humira only) with an inadequate response to conventional therapy or who have lost response to or are intolerant to infliximab.

Hidradenitis suppurativa (Humira only): Treatment of moderate to severe hidradenitis suppurativa in adults and children ≥12 years of age

Juvenile idiopathic arthritis: Treatment (to reduce signs/symptoms) of active polyarticular juvenile idiopathic arthritis (moderate to severe) in pediatric patients ≥2 years of age (Humira) or ≥4 years of age (Amjevita; Cyltezo); may be used alone or in combination with methotrexate

Plaque psoriasis: Treatment of chronic plaque psoriasis (moderate to severe) in adults who are candidates for systemic therapy or phototherapy, and when other systemic therapies are less appropriate (with close monitoring and regular follow-up)

Psoriatic arthritis: Treatment (to reduce signs/symptoms, inhibit progression of structural damage, and improve physical function) of active psoriatic arthritis in adults; may be used alone or in combination with nonbiologic disease-modifying antirheumatic drugs (DMARDs)

Rheumatoid arthritis: Treatment (to reduce signs/symptoms, induce major clinical response, inhibit progression of structural damage, and improve physical function) of active rheumatoid arthritis (moderate to severe) in adults; may be used alone or in combination with methotrexate or other nonbiologic DMARDs

Ulcerative colitis: Treatment (to induce and sustain clinical remission) of active ulcerative colitis (moderate to severe) in adults who have had an inadequate response to immunosuppressants such as corticosteroids, azathioprine, or 6-mercaptopurine. (Note: Efficacy in patients that are intolerant to or no longer responsive to other TNF blockers has not been established.)

Uveitis (Humira only): Treatment of non-infectious intermediate, posterior, and panuveitis in adults and children ≥2 years of age

Mechanism of Action

Adalimumab is a recombinant monoclonal antibody that binds to human tumor necrosis factor alpha (TNF-alpha), thereby interfering with binding to TNFα receptor sites and subsequent cytokine-driven inflammatory processes. Elevated TNF levels in the synovial fluid are involved in the pathologic pain and joint destruction in immune-mediated arthritis. Adalimumab decreases signs and symptoms of psoriatic arthritis, rheumatoid arthritis, and ankylosing spondylitis. It inhibits progression of structural damage of rheumatoid and psoriatic arthritis. Reduces signs and symptoms and maintains clinical remission in Crohn disease and ulcerative colitis; reduces epidermal thickness and inflammatory cell infiltration in plaque psoriasis.

Bhopalwala. H

Monday, February 25, 2019

Detection of Cryoglobulins

Detection of cryoglobulins — To detect cryoglobulin, 10 to 20 mL of blood are drawn into syringes and/or collection tubes that have been prewarmed to 37ºC without anticoagulants. These precautions are required because failure to prewarm may lead to false-negative results, due to loss of the cryoglobulin in the clotted blood (eg, if there is cooling below 37°C during collection, clotting, or centrifugation) and because the presence of anticoagulants may produce false-positive results due to the formation of cryofibrinogen or heparin-precipitable complexes.

After clotting at 37°C for one-half to one hour, the serum is separated by centrifugation at 37°C, placed in a graduated (Wintrobe) tube, and refrigerated (4°C) to allow the precipitation of cryoglobulin. In type I cryoglobulinemia, precipitates are often seen within 24 hours (sometimes in less than 90 minutes). However, three to five days are usually allowed for complete precipitation, especially for the mixed cryoglobulins, and some type II and type III cryoglobulins require up to seven days for precipitation . Most laboratories will determine a cryocrit, which is a measure of the packed (centrifuged) volume of the precipitate as a percentage of the original serum volume at 4°C.

Further confidence that the precipitate is a true cryoglobulin is obtained by washing the precipitate three to six times in cold saline solution to reduce the possibility of precipitated salts or other proteins. In addition, the precipitate can then be redissolved in saline at 37°C to confirm the warm solubility of the cryoglobulins. At this time, cryoglobulin protein concentration can be determined by spectrophotometry. Further characterization can be accomplished by immunofixation, enzyme-linked immunosorbent assay (ELISA), or another specific immunologic assay.

Some laboratories perform further testing consisting of a measurement of absolute cryoglobulin concentration, along with a description of the components of the immune complexes, including mono- or polyclonality of IgM, IgG, IgA, IgE, kappa, and/or lambda light chains. In type II cryoglobulinemia, the monoclonal component is typically IgM kappa with rheumatoid factor (RF) activity.

The cryocrit in individuals without cryoglobulinemia is close to zero; generally, a cryocrit over 0.5 to 1 percent or cryoglobulin concentration over 50 mcg/mL is considered clinically significant . The cryocrit in affected patients may approach 50 percent or may encompass the entire serum volume in type I cryoglobulinemia under conditions in which the monoclonal protein forms a gel.

The cryocrit is generally between 2 and 7 percent in type II and between 1 and 3 percent in type III disease, but there is a poor correlation between the cryocrit and clinical symptoms and features.

When cryoglobulinemia is suspected clinically, a negative result from routine laboratory testing for cryoglobulins does not exclude cryoglobulin-mediated disease . The clinician may need to draw a new specimen after consulting with the laboratory staff or clinical pathologist to assure that procedures are in place for the appropriate handling of the patient's blood when the sample is obtained and transported and to be certain that the laboratory has the necessary equipment (particularly a temperature-controlled centrifuge) to prevent premature cooling of the sample.

Bhopalwala. H

Vision Loss in Giant Cell Arteritis

Causes of vision loss —

Permanent loss of vision in GCA results from arteritic anterior ischemic optic neuropathy (AION), central or branch retinal arterial occlusion (CRAO/BRAO), posterior ischemic optic neuropathy (PION), or, rarely, cerebral ischemia

●Anterior ischemic optic neuropathy – At least 80 percent of cases of vision loss in patients with GCA are caused by AION . The ischemic insult in arteritic AION is typically the consequence of occlusion of the posterior ciliary artery, a branch of the ophthalmic artery from the internal carotid artery, and the main arterial supply to the optic nerve.

Only about five percent of the total occurrences of AION are due to GCA, the majority being nonarteritic and secondary to atherosclerotic disease . About 40 percent of patients who suffer nonarteritic AION regain some amount of visual acuity, in contrast to visual loss due to GCA, which is more often massive and irreversible .

●Central retinal artery occlusion – CRAO is responsible for approximately 10 percent of the cases of visual loss in GCA . On the other hand, approximately two percent of older patients with CRAO have underlying GCA . Bilateral CRAOs in an older adult should prompt evaluation for GCA.

●Posterior ischemic optic neuropathy – PION occurs in less than five percent of patients with GCA . It results from the interruption of blood flow to the retrobulbar portion of the optic nerve. Histopathologic examination typically reveals inflammatory occlusion of the short nutrient posterior ciliary arteries .

●Branch retinal artery occlusion – BRAO is distinctly uncommon in GCA, though it has been described.

●Cerebral ischemia — Homonymous hemianopia is a visual field defect involving either the two right or the two left halves of the visual fields of both eyes. The most common cause in GCA is an occipital lobe infarction resulting from a lesion in the vertebrobasilar circulation. In rare cases, bilateral occipital lobe involvement leads to bilateral homonymous field defects and to the development of cortical blindness.

Bhopalwala. H