Computer generated transcript

Warning!
The following transcript was generated automatically from the content and has not been checked or corrected manually.

Hello. Uh Can anyone hear me? Hi. Yeah. Ok, good. Uh I hope your parents can hear me. So let's uh move on the topic. So today's topic, uh Dermatomyositis and uh pois. So it's a type of inflammatory myopathy. So we're talking about these two conditions. Now, this uh uh dermatomyositis and polym that a group they fall into this group of inflammatory myo with uh which is in which is involved in progressive weakness of these muscles, acute chronic skeletal muscle inflammation. So when you talk about uh myositis, itis means inflammation. So this uh dermatomyositis are clean now. Hello. Ok. Bye bye. Is it clear now? Uh is the audio now first. Uh So today's topic is on uh polym and t. So both of these conditions, they fall into a group called this inflammatory myopathy. So this inflammatory myopathy, it is uh a group of these systemic diseases which has, which is involved in skeletal muscle problems, skeletal muscle inflammation, which causes weakness, the uh weakness of uh these muscles. So this uh this whole group that is this inflammatory myopathy. They can be classified into dermatomyositis, overlap, myositis, immune, uh immune media necrotizing myopathy and inclusion, polymyositis and polymyositis. So this is like a broad classification of all these myositis in this. Uh what we're gonna focus today is on dermatomyositis and polymyositis. Uh Talking about these other two other myositis. This inclusion by uh inclusion body just sentences. It's a small description means this inclusion body myositis. It's a myopathy which affects both proximal and distal ske muscles. So both the uh example into the upper limb, both the arm and forearm is affected or the uh lower limb, the thigh region and also lower leg region. Both affected this immune mediated necrotizing myopathy. It's a fairly new kind of a new kind of classification. It affects uh the proximal skeletal muscles and it is uh associated with a severe rise in uh creating kindness. That's uh marker with a marker of skeletal muscle damage which we see which we uh talk about more slides more and it's overlap. My is over myositis is when there's inflammatory myopathy, one of these conditions and it is associated with features of another uh autoimmune connective tissue disease. That is, it can be sly or it can be Sjogren Syndrome or it can be rheumatoid arthritis. If it is associated with that, uh we call overlap myositis or classic form of this over. So overlap myositis, this anti synthetase syndrome and is characterized by this muscle weakness, rayna's phenomenon. Arthritis in intestinal lung, uh lung disease and so on. So those is that like a brief introduction about what the other forms are. But what we're gonna focus on is about this dermatomyositis and this poly polymyositis. Now, uh moving on this bo bo they classify this uh in uh myopathies into 676 or seven classifications. So we have a primary idiopathic polymyositis. Are you primary oath dermatomyositis. And the, we have this chi uh poly poly associated malignancies. Then we have childhood dermatis uh my of polymyositis. So it can be associated with other tissue diseases or it can be miscellaneous. These are very uh features which are found along with this kind of myo that is, is my of my my. Now, moving on. Now, when we talk about the etiology of this condition, that is uh how this pathophysiology. Now this uh myositis, it is myositis immune media syndrome. That is our own immunity. That is our B cell scent cell antibodies. They uh affect our own cells. So this is a defective cell immunity. Here, there are multiple risk factors. So we all uh have heard about this HLA types there, HLA A one B and D three. So these conditions uh when the patient has this kind of uh uh genetic makeup, they have increased risk of getting this condition and it can be other infections such as this coa virus HIV hepatitis eco virus, adenovirus. These uh uh infected conditions also increases the risk of this patient. There is also uh some drugs which are also involved in increasing the incidence of this uh derma derma uh dermatomyositis. That is uh we have here drugs such as penicillAMINE, hydrALAZINE or you can even uh angiotensin converting enzyme inhibitors which are commonly used in uh hypertension. They also have associated with this uh inflammatory myopathies. And also there have been cases of this myopathy, uh this dermatomyositis involved after COVID-19 infections, COVID-19 infection and also vaccination for COVID. After that they have there, there have been cases reported for the dermatomyositis. Uh and I would like to ask you a question here. Mhm. Uh Can you name me another drug? Which we, which is commonly used, which causes uh muscle inflammation, a very common used drug. Not one of the ones I mentioned, but another drug is commonly used which causes muscle inflammation. The drug is used for obesity or even the have this aosis conditions like that. This drug is commonly used. Can someone mentioned that Trump? Ok. Uh I will accept it. So this is a drug that causes inflammation we had uh that is another drug that is statins. The statins uh are used uh you know, obesity. This is uh this is a drug that decreases cholesterol formation and that is so it can be used in obesity and also uh it stabilizes the plaques and atherosclerosis. So it prevents rupture of it which can block vessels. So these statins are used in ischemic heart diseases or even strokes, it can be used. So it stabilizes the plaque, preventing from rupturing and causing ate blockage of vessels. So, statins is a drug that causes uh say muscle inflammation. It also causes proximal myopathy, also affects the proximal muscles and also causes rhabdomyolysis. So, that's breakdown of the muscles and release of all the enzymes. So it uh statins cause proximal myopathy and also rhabdomyolysis. Uh moving on, on spec especially you're talking about polymyositis and dermatomyositis. So, the polymyositis, it is a cell media cytotoxic. So it's a uh that is the T cells and cytotoxic cells that mean cytotoxic against the skeletal muscle antigens. Uh mainly the Endomysium then with dermas. So, dermatomyositis, it is an antibody mediated, it's an antibody mediated uh autoimmune condition here which which also associated vasculopathy. That is, that means the small and medium size vessels they also affected in this uh uh dermatomyositis. And uh also we know there's a 25% risk the derma uh dermatomyositis there, a 25% risk with associational malignancies as mentioned here. So, these malignancies are like gastric cancer, ovarian cancers or it can be non hodgkin's lymphoma. So these uh sometimes this uh ovarian cancer that can be the first presentation of this uh dermatomyositis condition. So, this ovarian cancer can be a first presentation for dermatomal. So we should always, we we are assessing the ovarian cancer. Sometimes we should also be on lookout for dermatomyositis. Also, we should ask some few questions to rule out this dermatomal is now moving on. Uh when talking about the condition and the clinical features. Uh most common uh this this condition, it uh causes muscle weakness because it's muscle inflammation. So what we find here, the asymmetrical proximal muscle weakness and atrophy. So all the arms and upper thighs. So the symmetrical uh weakness of both arms, proximal weakness. So what uh and it commonly affects the pelvic can hold the girdle muscles. The pill we can, should the girdle muscles are affected. So, what we can find in the patient is we can uh uh the patient will have difficulty kneeling down, climbing or climbing or coming down stairs or if they kneel down and uh if they kneel down to the floor for them to come back up again, to be difficult, combing their have, which is very common that will be difficult for them and they raising their arms, lifting objects. It's all a little bit difficult for the patient to, to do. And also there is also the neck muscles are affected where the flexors are affected more than the extensor. This can be elicited during the physical examination where the patient will have the difficulty holding the neck up for a long time. Patients flexor are more com more are more stronger in this condition because the flexes, uh sorry, sorry, the flexes are affected more here. The patient can't keep them uh neck in fixed position for a long time, right? And moving on the uh there's muscle, uh muscle pain as a also can be found here and there's oropharyngeal muscle. We can study the muscles of swallowing or with a patient. We present our patient. We have that is difficult and also sometimes in the respiratory muscles, these intercostal muscles or this uh diaphragm muscles or even the smooth muscles of the bronchial or the bronchus, they also can be affected, it can lead to restrictive lung disease. So, this muscle weakness is common for both polymyositis and dermatomyositis. There is all these uh my, I mentioned this derma over or for all these my my this muscle weakness uh clinical features is present in all but these uh cutaneous features, these cutaneous features which I'm gonna mention uh for in the future. Now, this is very characteristic for dermatomyositis. So, dermatomyositis will have cutaneous manifestations as well as muscle, at least muscle weakness manifestations. Whereas polymyositis, it will only have these muscle weakness manifestations. So we talk about this uh cutaneous manifestations. We have this uh there's a symmetric erythro rash on the extensor surface of the hand joints, elbows, knees. So when there's a rash on this knees, we call it the Ron sign. And also we can see scaly papules, scaly papules, which can be found on these uh extended surfaces. These are called Ron papules. So you can see the Ron sign, the knee areas and these uh elbows and you can see the scaly papules, which is Ron paes on these uh extend surface of these uh hand joints and around the upper eyelids, around the upper eyelids. We can see uh this rash that is called heliotrope rash. And also it's associated per edema, which is a rash can be found in the midface. It can be found in the midface area, this uh rash in the midface, it can mimic the mala rash which is found. So we can mimic this butterfly shaped rash. But here the nasal labial also the nasal la also affected. Whereas in l the nasal ab also not affected. So that's one thing which can differentiate between a a silly uh rash and this uh rash. And uh there will be a rash from the upper back, posterior neck and also the shoulders that is called a sign where behind posterior neck, commonly, posterior neck and shoulder. We can see this rash that's called a sign and that can be a rash in the upper just that's a W sign. The sign is in the lateral thighs. Then we can see this uh poikiloderma which is the condition is a chronic condition. I mean course for a long time, we can see poikiloderma. So this polio derma is when there's a hyperpigmented or hypopigmented, macular ectasia, the di skin vessels or there can be epidermal atrophy. All these conditions can be found that is this poikiloderma. Then the patient can present also present with mechanic hands. So this mechanic hands are when there uh when a pa when a person, uh how do you say when a person uses the fingers? Long time, we get this uh cracked and thickened skin in the fingers, right? Uh I hope you can get an idea of it. So we can see the mechanic hands also in these patients with dermatomyositis, then they can per ectasis that is dilation of vessels around the uh nail areas. And that can be calcinosis cutis with calcinosis. Uh cutis is when there's increased calcium deposition, which can be uh increased calcium deposition in the skin areas should be fine. Uh I'd have to ask another question also uh at least uh and please in a book. So if someone can please answer these questions, it'll be good. Uh OK, then uh that the other condition that present calcium, there are multiple conditions that can present this calcium disease. What I was looking for here is a scleroderma. Also another tissue disorder which affects uh uh tissue disorder which involves the skin area that is skin tightening. So they can be limited or diffuse. So in that there are limited symptoms, we have the crest uh pic the crest things, calcinosis, Raynaud's phenomenon, eso facial dysfunction, sclerodactyly disease. I hope you to know this crest in which is found in scleroderma. So in that the C stands for this calcinosis condition, then uh moving on. I'd like to show you a picture here. See, we hear what we see is prox muscle weakness. As you can see the thigh muscles and the shoulder girdle muscles, they are affected. And we can see this heliotrope rash that is a rash in the up eyelids, got tru papules which are found in the proximal interphalangeal joint and distal interphalangeal joints. We can see these got Crum papules which are erythematous papules on the dorsum of the hand. And the patient can also have dy I show the picture. They can have Raynaud's phenomena. So you mentioned there but it pictures. So you can have Raynaud's phenomena also. And also there can be this uh calcification in the calcium, as I mentioned. And there can be some uh systemic manifestations of interstitial lung disease or myocarditis or conduction defects. Uh So here are the pictures, live pictures which I hope everyone can see these live pictures. So what we see here is this here, we can see these kind of papules which can form the distal inter joint and also this proximal joint here. So we can see those papules, these are drawn papules which I mentioned earlier. So it's a clear picture here. I hope you all can see this uh appreciate this pap and erythematous rash here. And what y'all can see here is this, this is a heliotrope rash mostly in the upper eyelids or it can be around the eyelids. This is a heliotrope rash I can see. So these two are kind of pathos are very commonly found in dermatomyositis patients and moving on. Uh This is the SW sign. Uh not the uh this sorry, this is the V sign, this one N TV chest area. It uh that is this a sign and this is the calcinosis that is a deposition of calcium. This is the calcinosis which can be found in patients and uh moving on. This is the shall sign that is on the posterior ne area and the shoulder. This is a sign which I hope you can appreciate this a show. It's like this. And this is the uh other sign which I mentioned earlier, which can be found uh in the lateral side of the thigh and also in near the pelvic uh pelvis area. Now, this is the mechanic's hand. Uh we can see they appreciate this kind of skin uh thickening areas in the side of the thigh fingers. And like, I hope you all have seen this in mechanics and our people who use their hand a lot for doing so much work. It thickens, the skin starts be a little. So this can be appreciated in the derma is also then another thing I I could, I didn't mention it is uh this uh this thing. So you can see this scar, this alopecia, which is also can be found in these patients with dermatomyositis. And also we can see that in this is uh after photosensitive after the hand, this uh dermatomyositis rash is photosensitive. So when exposed to sun, it can exacerbate this condition. What you see in this pictures of hand which has been kept outside the sun that is it's uh it has reacted to sunlight. So what uh we should appreciate here is there there is uh the these areas, these inter digital spaces, these areas have not been affected by this uh dermatomal, this should be appreciated in this picture. So in patients, most dermatosis patients that uh that area is not affected this interdigital space area. So I hope you all can appreciate that that the interdigital space area is not affected in this dermatomal condition. Moving on. We also can see these tees which I mentioned uh which here here, I hope you can appreciate these are the dilation of the superficial blood vessels. And also we can uh find ulcers in patients. Uh in the hand, we here we can see ulcers. These can be found in patients of this condition. I hope I can hear this. These are ulcers which can be found in Dermatomyositis patients. We can appreciate that and they still inject us. Yes. Now moving on, we're talking about the systemic complications. Uh systemic complications of these are systemic manifestations of this disease. So that can be gestation, lung disease, aspiration, pneumonia can occur because patient like dysphagia or the difficult swallowing, sometimes they can sweb because of weakness of these over muscles. Sometimes they can swallow, you know, uh anything can go inside the, the fria into the windpipe. Heart blocks can occur with arrhythmias or congestive heart failures. Pericarditis, dysphagia. I mentioned earlier, malabsorption can occur, pneumonias, myocardial infarctions. Carcinoma, carcinoma mentioned earlier. It's very common with dermatomyositis. With patients can present with ovarian carcinoma the first itself without this uh dermatomyositis. Now, in dermatomyositis, a patient can present with uh this is the skin, the cutaneous manifestations I mentioned now. So the patient can present these cute manifestations before they have this proximal muscle weakness or with the proximal muscle weakness or after the proximal muscle weakness. So they can present it with in any of these three stages that should also be known. I hope everything is clear for now. Uh Please do ask any doubts if you'll have now. Moving on now, moving on. Uh let's talk about the exam. Uh this uh diagnosis, there is a lab in a routine laboratory examination. What uh what changes we will be seeing. So here, the complete blood count that the full blood uh the full blood count, it can show leukocytosis increased WBC levels. The ESR and CRP which are inflammatory markers, it can be normal and mildly elevated. Here should be noted. The A and CRP can be normal and mildly elevated and the muscle enzymes. This is a very, this is very important is muscle enzymes. So, creating kinase is an enzyme which is uh released when the muscles are are damaged, creating kinase. It is elevated uh 2 to 200 times the normal value, but the levels do not correlate the symptoms. Sometimes it can be very severe and very slight elevation of. So it cannot correlate that we are other liver chemistries. A TL D can be elevated. These are LDH L Dola and myoglobin is also found in the muscles. So this uh lactate dehydrates. LDH can be elevated. LSE can be elevated. Myoglobin can be elevated. It should be for and also we should exclude uh serum electrolytes and TSH. Now, I would like to ask another question, right? I hope you have got the question. Uh The question is which electrolyte is involved in prox muscle weakness. I hope uh someone can answer this question. OK. OK. Uh that uh electrode is potassium, potassium is electrolyte involved here. So, hypokalemia uh when there's a decrease in uh potassium, that's uh that condition also presents with proximal muscle weakness. So we should exclude that. And also magnesium also can cause this uh muscle weakness. We should exclude magnesium muscle. So TSH also we checked because hypothyroidism, hypothyroidism also presents hypo all this hypothyroidism, it also presents with muscle weakness. So we should exclude that also. So we should do those tests. Also, we should check for basic, these basic metabolic profile. So potassium sodium, all those electrolytes and also magnesium should be checked. Also TSH should be, we should exclude this in the diagnosis. Next thing we should check for the antibodies. Now, this antinuclear antibody assays are done, but it's uh only positive in around one third of patients polymyositis. So uh anti antibodies can be done in most patients. But we uh uh not very specifically for polymyositis. But for dermatomyositis, we have the NTI two antibodies. These are highly specific for dermatomyositis but not highly sensitive, which is only highly uh specific for this. So if it is positive means we can go with the dermatomyositis other antibodies which can be checked. We have anti MD five anti TIF one Y anti NP two NTSA. These antibodies also can be checked but this is a very important antibodies. We should, we should be checked and also remember this anti M two antibodies which is found in Omy. No, we go we have muscle biopsy. Now this muscle biopsy is the gold standard investigation for this myositis. There is inflammation of muscle myositis. So what we find is we can find nec necrosis, degeneration, regeneration muscles. So what we can find specifically in dermatomyositis, we can find this, as I mentioned, it is an antibody mediated condition which involves CD four positive T cells, dendritic cells and B lymphocytes in the perimysium means it is a connective tissue that surrounds the fascicle. So I uh I hope you all have, we all know the fascicle, these and perimysium is a covering of this fascicle which shows the peri fascicle atrophy. Then poly polym is a, it is a cell mediated, as I mentioned, it's a cell media which involves a cytotoxic ct eight positive T cells which uh involves endomysium iny covers the muscle fibers. So multiple, multiple muscle fibers form the fascicle. But you can see is a biopsy of dermatomyositis. I think you all can see this atrophy mark is a muscle atrophy here with the infiltration of this areas with lymphocytes. And so that's perivascular infiltrates. So I hope you are gonna appreciate this as perivascular infiltrates here with uh muscle atrophy and also that infiltrates infiltrates here. Moving on. Now, an MRI also can be used to show the presence of the inflammatory myopathies in patients. Some patients do not present with uh severe weakness but MRI can show this inflam myopathy. What's shown here in this MRI T two image. We can see this hypertense image. This is my uh the quadriceps which we can see there's a myopathy as inflammation in these areas. Just the mark here. That's inflammation here. Electromyography, electromyography is not done anymore cause that's the introduction, ra the big use of these biopsies, electrogram is not use anymore, but it can help in help to distinguish myopathic and neuropathic uh processes and includes them. Uh this myograph checks the motor unit action potentials of the muscles. So it can show any changes in that which decreases myograph, it decreases in the myositis. So that can be checked there. But if not you not used much anymore. Now, finally, when talking about the management of these conditions, not all patients, they should be start on supportive therapy. That is uh physical occupation, speech therapy, uh regular aerobic exercise, the schedule should be made for them, for them to keep uh increasing their muscle strength. Now, the sun avoidance and sun protective measure, this is for dermatomyositis should be done uh done with use of with the use of broad spectrum sunscreens and photo cept photo protective clothing. So they should cover the whole body or cover the whole area. Use uh sunscreen, all kinds uh to protect themselves from sun and a high protein dash should be given for these patients. Now, moving on to specific management, we are the pharma culture management. That is the main management, the pharma culture in this Pharma glucocorticoid that is prednisoLONE is the main first line drug used for these conditions. If severe or multisystem in involved, we can go for IV this mostly oral predniSONE is given. But if it's a severe condition of multis involvement, we can give IV methyl prednisoLONE can be given. And uh uh if uh a steroid sparing, immunosuppressive like methotrexate, erythro can be used. Uh after predniSONE, we can use methotrexate and methotrexate should be looked out for because it is also immunosuppressant that can be increase the risk of infection for this patient. Now cause liver toxic. One is for the skin. There is dermatomyositis. Hydrochloro has been shown to improve the symptoms of the skin cute manifestations. So that also can be used. Now, we should monitor the response to therapy, ba we should monitor the response to therapy based on the improvements in the muscle strength. The uh, muscle strength scores are there. We can check using that muscle endurance, how long they can keep using that muscle and also that levels of, uh, creatinine level decrease and, uh, moving on, we can give on additional medications. That is, uh if the patient is having severe refractory disease, we can give IV immunoglobulins or riTUXimab dysphagia. If the patients have dysphagia, we can give immunoglobulins. And also the patient is having rapidly progressing interstitial lung disease. That means that the lung disease is rapidly progressing. We can do to remove the immunoglobulins and the uh cytotoxic CD eight cells. Ok. Uh So that's it for today. I hope uh the session was good. I would like to ask one more question also. So, you know, if a patient I wanna uh uh answer this question, I hope your session will help your answer your clinical questions too. Uh My opinion, the patient presents with the same symptoms of worsening of these symptoms. Uh What do you think the cost will be for that condition? Ok. Yeah. So, uh we should think about two things here that is whether there is a relapse or whether there's worsening of this. Uh my or as I mentioned, the first line drug for this is steroids So we should know that steroid itself can cause muscle damage. So, one of the side effects are the steroids that is prednisoLONE or met prednisoLONE. One of the side effect side effects of that uh drug is muscle damage. So that uh is the cause of this uh worsening of the muscle worsening of this muscle damage. It can be the steroid myopathy itself or it can be the relapsing of worsening of this uh myo. So these are the two things we should think about. So how to uh differentiate these two means. Now, if the patient has steroid induced myopathy, we can stop the steroid therapy for a short period of time and check whether muscle where the patient's symptoms get better. So if that is the cause, then the steroid myopathy is the cause for that uh myopathy, the patient has. But if you stop the steroids and the the condition of the patient worsens even further, that means it is not the steroids, the cause it is worsening of this oil as a uh worsening of the po polym derma mass. So we should go for a higher dose or change the drug to this immune suppressant such as ane or this uh methotrexate. So that's one thing we should look out for. Ok. So I guess that's the session was good. You learned something I hope uh that's good. Can you please uh fill this feedback form to get your certificates