Restrictive vs Liberal approach to transfusion in Sepsis

Hello everyone, 

Here are some studies on approach to blood transfusion during sepsis:

One multicenter randomized study of 998 patients with septic shock reported no difference in 28-day mortality between patients who were transfused when the hemoglobin was ≤7 g/dL (restrictive strategy) and patients who were transfused when the hemoglobin was ≤9 g/dL (liberal strategy) . The restrictive strategy resulted in 50 percent fewer red blood cell transfusions (1545 versus 3088 transfusions) and did not have any adverse effect on the rate of ischemic events (7 versus 8 percent).

One randomized trial initially reported a mortality benefit from a protocol that included transfusing patients to a goal hematocrit >30 (hemoglobin level 10 g/dL) . However, similarly designed studies published since then reported no benefit to this strategy. 

Bhopalwala. H

Source: UpToDate 

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

qSOFA Score for Sepsis

The qSOFA (quick Sequential Organ Failure Assessment) score is easy to calculate since it only has three components, each of which are readily identifiable at the bedside and are allocated one point:

●Respiratory rate ≥22/minute

●Altered mentation

●Systolic blood pressure ≤100 mmHg

Bhopalwala. H

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

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

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

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

Low Alkaline Phosphatase

Subnormal values — Extremely low serum alkaline phosphatase concentrations can be seen in patients with fulminant Wilson disease complicated by hemolysis .
Low values can also occur in patients with hypothyroidism, pernicious anemia, zinc deficiency, congenital hypophosphatemia, and certain types of progressive familial intrahepatic cholestasis in children.

Bhopalwala. H

Beighton Score for Joint Hypermobility

Beighton score for joint hypermobility —

JHM should be evaluated in all patients suspected of JHS. JHM is ascertained by determination of their Beighton score, which depends on the presence of JHM in the hands, elbows, lumbar spine, and knees using specific examination techniques . One point is awarded for the ability to perform each of nine maneuvers (including four maneuvers tested bilaterally and evaluation of the spine). A score of 4 or more points represents generalized hypermobility. The specific maneuvers include:

●Passive apposition of the thumb to the volar aspect of the ipsilateral forearm

●Passive hyperextension of fingers, demonstrated by passive dorsiflexion of the fifth metacarpophalangeal joint to at least 90 degrees

●Hyperextension of the elbow to at least 10 degrees

●Hyperextension of the knee to at least 10 degrees

●Flexion of the spine with placement of the palms flat on the floor without bending the knees

The presence of JHM can be documented by an examination limited to those areas required for calculating the Beighton score, but an examination for JHM and joint stability that is adequate for fuller assessment of the patient and the formulation of treatment plans should also encompass the other joints, including the temporomandibular joints, shoulders, hips, cervical and thoracic spine, ankles, and feet.

In addition to determination of the Beighton score based upon the examination, the presence of generalized JHM, including its presence historically, may also be suspected in patients who answer ‘yes’ to two or more questions in a simple five-part questionnaire :

●Can you now (or could you ever) place your hands flat on the floor without bending your knees?

●Can you now (or could you ever) bend your thumb to touch your forearm?

●As a child did you amuse your friends by contorting your body into strange shapes OR could you do splits?

●As a child or teenager did your shoulder or kneecap dislocate on more than one occasion?

●Do you consider yourself double-jointed?

Bhopalwala. H

Difference Between Solitary and Singular Brain Metastasis.

Hello Guys!

So we were operating on a metastatic brain lesion the other day when My Consultant happened to ask me the question-  " What is the difference between Solitary and Singular Brain Metastasis?" Well I happened to have a vague idea and managed to blabber something, the actual definition goes as-

• A solitary brain metastasis is defined as the only known metastasis of a tumour in the whole body which happens to be
localised in the central nervous system.

• A singular brain metastasis is defined as a single cerebral metastasis with additional metastases in other organ systems.

Well it's a small nugget, may save you some embarrassing moments.

Let's Learn Together!
-Medha Vyas.

Bohan and Peter Criteria for diagnosing DM-PM

Bohan and Peter in 1975 defined DM and PM based upon the following features :

●Symmetric proximal muscle weakness

●Typical cutaneous eruption of DM (the only feature distinguishing DM from PM)

●Elevated serum muscle enzymes

●Myopathic changes on EMG

●Characteristic muscle biopsy abnormalities and the absence of histopathologic signs of other myopathies

Patients with the cutaneous eruption and at least three of the other four criteria met the requirements for definite DM according to these criteria, while requirements for definite PM were met by those with all four criteria other than the cutaneous features . Patients with findings indicating the presence of other disorders that may present similarly were excluded. Patients who did not meet these criteria but who lacked any of the exclusions could potentially have been diagnosed with possible or probable DM or PM, depending upon the number of criteria met.

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

Antisynthetase Syndrome

Antisynthetase syndrome — Up to 30 percent of patients with DM or PM have a constellation of clinical findings termed the "antisynthetase syndrome" . These findings include relatively acute disease onset, constitutional symptoms (eg, fever and weight loss), myositis, the Raynaud phenomenon, mechanic's hands, arthritis that is generally nonerosive, and ILD . Affected patients have antibodies to aminoacyl-transfer ribonucleic acid (tRNA) synthetase enzymes; the presence of one of these antibodies is highly specific for DM, PM, or ILD .

This syndrome can be further characterized as follows:

●Not all patients with antisynthetase antibodies or even those classified as having the antisynthetase syndrome have all manifestations of this syndrome. The syndrome is generally considered present in patients with an antisynthetase antibody plus two of the following features, which are elements of the syndrome: ILD, inflammatory myopathy, and inflammatory polyarthritis.

●This group of clinical findings or this general clinical picture is not specific for antisynthetase antibodies. Patients with other types of autoantibodies (eg, anti-PM-Scl or anti-U1 ribonucleoprotein [RNP] antibodies) can also present with these types of features. However, patients with antisynthetase antibodies generally have more prominent or severe myositis and ILD, and they usually lack some of the other clinical features seen in patients with these other autoantibodies.

●Some patients with antisynthetase antibodies have relatively little or no myositis, while ILD or other features are more prominent. The absence of myositis is seen more often with some antisynthetase antibodies than with others.

Bhopalwala. H

Classification Criteria for Rheumatoid Arthritis

2010 ACR/EULAR criteria —

Using the 2010 ACR/EULAR classification criteria for RA, classification as definite RA is based upon the presence of synovitis in at least one joint, the absence of an alternative diagnosis that better explains the synovitis, and the achievement of a total score of at least 6 (of a possible 10) from the individual scores in four domains . The highest score achieved in a given domain is used for this calculation. These domains and their values are:

●Number and site of involved joints

•2 to 10 large joints (from among shoulders, elbows, hips, knees, and ankles) = 1 point

•1 to 3 small joints (from among the metacarpophalangeal joints, proximal interphalangeal joints, second through fifth metatarsophalangeal joints, thumb interphalangeal joints, and wrists) = 2 points

•4 to 10 small joints = 3 points

•Greater than 10 joints (including at least 1 small joint) = 5 points

●Serological abnormality (rheumatoid factor or anti-citrullinated peptide/protein antibody)

•Low positive (above the upper limit of normal [ULN]) = 2 points

•High positive (greater than three times the ULN) = 3 points

●Elevated acute phase response (erythrocyte sedimentation rate [ESR] or C-reactive protein [CRP]) above the ULN = 1 point

●Symptom duration at least six weeks = 1 point

In addition to those with the criteria above, which are best suited to patients with newly presenting disease, the following patients are classified as having RA:

●Patients with erosive disease typical of RA with a history compatible with prior fulfillment of the criteria above

●Patients with longstanding disease, including those whose disease is inactive (with or without treatment) who have previously fulfilled the criteria above based upon retrospectively available data

Fun fact : Hand ultrasound is done to evaluate for inflammation in RA, if it's not clinically evident.

Bhopalwala. H

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