pace your MRCP-PACES

ANNOUNCEMENT FOR ONLINE COURSE NO 42 ( 22 July 24—09 Aug 24 ) Hello and salam everyone. We are pleased to announce admissions for our July-Aug 2024 online course ( 15 days ) for PACES MRCP (UK) and CLINICAL EXAM MRCPI . We will start from 22th of July 24 and finish on 09th of Aug 24. Active slots available on first come--first served basis. Listener slot available too. At least 90 Important exam cases ( including recent diets cases ) will be practiced and discussed as we do in our weekly Sunday sessions. After the performance detailed feedback will be given to elaborate on the weak skills. It is equally beneficial for those who are beginners or have exams in coming diet. Interested candidates may send a personal message for details. WhatsApp No: +923346036496. Email: drtanzeelbukhari@gmail.com GOOD LUCK.

our next course may run from 12 Aug 24 to 30 Aug 24 ( tentative dates ). Regards

ANNOUNCEMENT FOR ONLINE COURSE NO 42 ( 22 July 24—09 Aug 24 ) Hello and salam everyone. We are pleased to announce admissions for our July-Aug 2024 online course ( 15 days ) for PACES MRCP (UK) and CLINICAL EXAM MRCPI . We will start from 22th of July 24 and finish on 09th of Aug 24. Active slots available on first come--first served basis. Listener slot available too. At least 90 Important exam cases ( including recent diets cases ) will be practiced and discussed as we do in our weekly Sunday sessions. After the performance detailed feedback will be given to elaborate on the weak skills. It is equally beneficial for those who are beginners or have exams in coming diet. Interested candidates may send a personal message for details. WhatsApp No: +923346036496. Email: drtanzeelbukhari@gmail.com GOOD LUCK.

👉 IMPORTANT 671 👈 Brief management summary for Ankylosing Spondylitis The following is partly based on the 2010 EULAR guidelines : encourage regular exercise such as swimming NSAIDs are the first-line treatment physiotherapy the disease-modifying drugs which are used to treat rheumatoid arthritis (such as sulphasalazine) are only really useful if there is peripheral joint involvement the 2010 EULAR guidelines suggest: 'Anti-TNF therapy should be given to patients with persistently high disease activity despite conventional treatments' research is ongoing to see whether anti-TNF therapies such as etanercept and adalimumab should be used earlier in the course of the disease paceUrMRCP.

👉 IMPORTANT 670 👈 Few Investigations for Ankylosing Spondylitis Inflammatory markers (ESR, CRP) are typically raised although normal levels do not exclude ankylosing spondylitis. HLA-B27 is of little use in making the diagnosis as it is positive in: 90% of patients with ankylosing spondylitis 10% of normal patients Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis. Radiographs may be normal early in disease, later changes include: sacroiliitis: subchondral erosions, sclerosis squaring of lumbar vertebrae 'bamboo spine' (late & uncommon) syndesmophytes: due to ossification of outer fibers of annulus fibrosus chest x-ray: apical fibrosis If the x-ray is negative for sacroiliac joint involvement in ankylosing spondylitis but suspicion for AS remains high, the next step in the evaluation should be obtaining an MRI. Signs of early inflammation involving sacroiliac joints (bone marrow oedema) confirm the diagnosis of AS and prompt further treatment. Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints. paceUrMRCP.

👉 IMPORTANT 669 👈 Referral criteria for ankylosong spondylitis NICE advise: all patients with suspected ankylosing spondylitis should be referred to a specialist 'Do not rule out the possibility that a person has spondyloarthritis solely on the presence or absence of any individual sign, symptom or test result.' paceUrMRCP.

👉 IMPORTANT 668 👈 Few clinical features of Ankylosing spondylitis Introduction Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathy. It typically presents in males (sex ratio 3:1) aged 20-30 years old. Clinical features Features typically a young man who presents with lower back pain and stiffness of insidious onset stiffness is usually worse in the morning and improves with exercise the patient may experience pain at night which improves on getting up Clinical examination reduced lateral flexion reduced forward flexion - Schober's test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible reduced chest expansion Other features - the 'A's Apical fibrosis Anterior uveitis Aortic regurgitation Achilles tendonitis AV node block Amyloidosis and cauda equina syndrome peripheral arthritis (25%, more common if female paceUrMRCP.

👉 IMPORTANT 667 👈 Few complications of ITP Complications of treatment are related to side effects of specific drugs used for treatment of ITP. A referral to local haematology department is desirable to weigh up the risks and benefits of choosing a particular modality of treatment against the risks of bleeding. I.e. if platelet counts are stable and above a certain acceptable threshold (depending on hospital guidelines, i.e. >50 and asymptomatic), it may be reasonable to continue monitoring. Patients who received splenectomy require long term prophylactic penicillin V and pneumococcal/meningococcal vaccines. Most patients with ITP do not have severe bleeding. In a review of adult ITP cases in Oklahoma over a 10 year period, 83% patients were managed on an outpatient bases. Indications for inpatient management of ITP include: Large bleeding; Haemodynamic compromise; Intracranial haemorrhage; Platelet count of less than < 30. Inadequate response to conventional therapy agents. paceUrMRCP.

👉 IMPORTANT 666 👈 Some treatment options for ITP ITP at first presentation should be referred to and managed by haematologists. The requirement for treatment varies between patient to patient; as such, clinical status is a key determinant for these considerations - i.e. asymptomatic, bleeding or planned surgical/dental procedures. Treatment is only given to those patients that absolutely require it, hence decreasing the risks of adverse effects such as infection as a result of immunosuppression. Below are the recommendations as according to British society of haematology. First line treatment for ITP is oral prednisone at 1mg/kg daily with proton pump inhibitors . This is given over a period of 2 - 4 weeks and weaned off a few weeks after. Most patients respond to this treatment within a week of commencing steroids. Pooled normal human immunoglobulin (IVIG) is also listed as first line treatment. Intravenous immunoglobulin is administered at 1g/kg. The mechanism of action is unknown but it was thought that one way is by blocking the Fc receptor of macrophages. Splenectomy had been used for many years, long before steroids were used. It is now second line for ITP in steroid refractory cases. It works by avoiding platelet destruction by removing the spleen. Other immunomodulatory drugs such as rituximab, cyclophosphamide and dapsone have also been used to treat ITP. Romiplostim works by activating the thrombopoietin receptor and stimulating JAK2, thus it stimulates platelet production. It has been approved by NICE as an option for ITP. Indications for usage include: ITP is refractory to standard active treatments and rescue therapies or Patients have severe disease and a high risk of bleeding that needs frequent courses of rescue therapies. Additionally it is important to avoid drugs which make bleeding worse. It is important to avoid antiplatelet drugs such as aspirin and clopidogrel. In cases of severe bleeding, it is important to treat promptly with transfusion of platelets, IVIG, tranexamic acids and initiate major haemorrhage protocol. Although transfusion of platelets may not improve platelet count, it has been shown to be effective at relieving the symptoms in acutely severe bleeding. paceUrMRCP.

👉 IMPORTANT 665 👈 Some investigations for ITP There are a number of investigations to be considered in patients presenting with bleeding and in those with incidental findings of thrombocytopenia. There is no single blood marker for ITP. Rather it is a diagnosis of exclusion. Minimum investigations to be done if thrombocytopenia is found include: Firstly, full blood count and blood film need to be requested. This would demonstrate isolated decrease in platelet counts, with normal counts in all other cell lineages. Additionally, there should be no evidence of fragments on the film. Secondly, a virology screen including HIV, EBV, hepatitis screen. Further investigations to be considered, as guided by history and examination findings: Clotting screen including PT and APTT, especially in those with severe bleeding. Thyroid function tests Malarial screen Myeloma screen. Immunological tests including ANA is considered especially if coexisting rheumatological features are present. Imaging including CT may be useful if there is a high index of suspicion for cancer. Bone marrow biopsy is no longer a standard investigation for ITP. If there are blood count abnormalities such as anaemia, leucopenia or other unexplained haematological findings, a bone marrow biopsy is warranted. In cases where ITP is not responsive to treatment and there is a high clinical suspicion for MDS, bone marrow biopsy should be carried out. paceUrMRCP.

👉 IMPORTANT 664 👈 Few differential diagnosis for ITP by courtesy ITP is a diagnosis of exclusion. Thrombocytopenia can be caused by increased destruction of platelets, and/or reduced production of platelets. Important differential diagnoses to consider include: Non-immunological causes of increased destruction of platelets include disseminated intravascular coagulation (DIC) i.e. in the setting of sepsis or malignancy. Pregnancy and its complications such as HELLP syndrome can also cause thrombocytopenia. Chronic liver disease and hypersplenism is associated with thrombocytopenia. Thrombotic thrombocytopenic purpura (TTP) is life-threatening disease which presents with thrombocytopenia but can result in multi-organ failure, cardiac complications, strokes and other thrombotic events. The hallmark for DIC and TTP is the presence of fragments on the blood film. Additionally, drugs such as heparin may result in thrombocytopenia, known as heparin-induced thrombocytopenia or HIT. Immunomodulatory drugs such as gold, alemtuzumab and more recently immunotherapy agents such as nivolumab and pembrolizumab have been associated with ITP. Thrombocytopenia may be the first sign in well patients with underlying viral infections such as HIV or hepatitis. Many haematological malignancies can result in thrombocytopenia such as leukaemia, lymphoma, myelofibrosis, myelodysplasia and myeloma. Therefore it is important to take a careful history, as patients with these diseases often present with constitutional symptoms. Finally, rheumatological diseases such as SLE and rheumatoid arthritis can present with thrombocytopenia. paceUrMRCP.

👉 IMPORTANT 663 👈 Some info about clinical presentation of idiopathic thrombocytopenic purpara ( ITP ) Approximately 1/3 of patients are asymptomatic and have incidental discovery on blood test. Approximately 2/3 of patients have bleeding. Commonly patients with ITP present to their doctors with petechiae , small red dots on the skin. Purpura , formed by petechiae joined together, can also occur. Mild epistaxis is common. Continuous epistaxis requiring nasal packing or cauterisation may pose greater risks of bleeding. In women, patients with ITP may have prolonged and heavy menstrual cycles. Very rarely, nonetheless the most importantly, ITP can present with intracranial bleeding; this occurs in 1.4% of the cases. Severe non-intracranial bleeds such as large gastrointestinal bleed occur in 9.6% of the cases. Patients with ITP can present as generally unwell, i.e. feeling lethargic. Occasionally, patients with ITP may present with strokes and TIA, often referred to as paradoxical thrombotic events in ITP. paceUrMRCP.

👉 IMPORTANT 662 👈 Some info about pathophysiology of idiopathic thrombocytopenic purpara ( ITP ) ITP is an autoimmune disorder. In ITP abnormal immune system leads to destruction of own platelets . Although the precise mechanism for ITP is not completely understood, there have been several widely accepted theories: In ITP, autoantibodies are produced by the patient's B cells, principally of IgG. These target against platelet membrane glycoproteins GPIIb/IIIa. These platelets with autoantibodies are then engulfed by macrophages and degraded in the spleen. The bone marrow compensates by producing megakaryocytes (precursor to platelets). Purpura occurs as a result of increased permeability in capillary due to low platelets. In some cases, it is thought that viral infections can precede development of ITP. Antibodies against viral antigens cross react with normal antigens on platelet surfaces thus producing molecular mimicry. paceUrMRCP.

👉 IMPORTANT 661 👈 Some info about etiology of idiopathic thrombocytopenic purpara ( ITP ) It is thought that ITP is an autoimmune disorder resulting in antibodies produced to target against individual’s own platelets, leading to thrombocytopenia. This in turn results in petechiae and in rarer cases more severe bleeding. Furthermore, ITP can be primary or secondary. Primary ITP occurs when there is isolated thrombocytopenia is found with no co-existing conditions. Secondary ITP is associated with co-existing conditions. These include: Rheumatological diseases CLL Lymphoma Viral infections Various pharmacological agents paceUrMRCP.

👉 IMPORTANT 660 👈 Important points in the management of sarcoidosis Indications for steroids patients with chest x-ray stage 2 or 3 disease who have moderate to severe or progressive symptoms. Patients with asymptomatic and stable stage 2 or 3 disease who have only mildly abnormal lung function do not require treatment hypercalcaemia eye, heart or neuro involvement Prognosis Factors associated with poor prognosis insidious onset, symptoms > 6 months absence of erythema nodosum extrapulmonary manifestations: e.g. lupus pernio, splenomegaly CXR: stage III-IV features black people paceUrMRCP.

👉 IMPORTANT 659 👈 Important clinical features of sarcoidosis acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia insidious: dyspnoea, non-productive cough, malaise, weight loss skin: lupus pernio hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol) the Kveim test (where part of the spleen from a patient with known sarcoidosis is injected under the skin) is no longer performed due to concerns about cross-infection paceUrMRCP.

👉 IMPORTANT 658 👈 Important investigations for sarcoidosis There is no one diagnostic test for sarcoidosis and hence diagnosis is still largely clinical. ACE levels have a sensitivity of 60% and specificity of 70% and are therefore not reliable in the diagnosis of sarcoidosis although they may have a role in monitoring disease activity. Routine bloods may show hypercalcaemia (seen in 10% if patients) and a raised ESR A chest x-ray may show the following changes: stage 0 = normal stage 1 = bilateral hilar lymphadenopathy (BHL) stage 2 = BHL + interstitial infiltrates stage 3 = diffuse interstitial infiltrates only stage 4 = diffuse fibrosis spirometry: may show a restrictive defect tissue biopsy: non-caseating granulomas gallium-67 scan - not used routinely paceUrMRCP.

👉 IMPORTANT 657 👈 Few syndromes associated with Sarcoidosis Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent Lofgren's syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis In Mikulicz syndrome there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma this term is now considered outdated and unhelpful by many as there is a confusing overlap with Sjogren's syndrome Heerfordt's syndrome (uveoparotid fever ) there is parotid enlargement, fever and uveitis secondary to sarcoidosis paceUrMRCP.

Dear colleagues May I have your attention please We are now having a regular 3 month *course for MRCP Part 1* for almost every diet and we are also conducting *free sessions to discuss part 1 stuff.* If any one of you is doing part 1 then I suggest you to join our part 1 group for future updates. Regards and good luck.

👉 IMPORTANT 656 👈 Important complications of Psoriasis Psoriatic arthropathy (around 10%) Increased incidence of metabolic syndrome Increased incidence of cardiovascular disease Increased incidence of venous thromboembolism Psychological distress paceUrMRCP.