Rheumatology PDF

ABD & AMR AMR & ABD Abd & amr Amr & abd Rheumatic disorders Ola Hijjawi Consultant Rheumatologist Rheumatism is an umbrella term encompassing many diseases the first of which is: Rheumatoid arthritis  A 42 YO lady presents with 6/12 Hx of progressively increasing pa...

ABD & AMR AMR & ABD Abd & amr Amr & abd Rheumatic disorders Ola Hijjawi Consultant Rheumatologist Rheumatism is an umbrella term encompassing many diseases the first of which is: Rheumatoid arthritis  A 42 YO lady presents with 6/12 Hx of progressively increasing pain, stiffness and swelling of small joints of hands & feet, elbows and knees. She denied any fever, hair loss, dry eyes, dry mouth, mouth ulcers, rash, Raynaud's or pleurisy. She wakes at night with tingling in her R thumb, index & middle fingers.  extra reading Raynauds: Raynaud's (ray-NOSE) disease causes some areas of the body — such as fingers and toes — to feel numb and cold in response to cold temperatures or stress. In Raynaud's disease, smaller blood vessels that supply blood to the skin narrow. This limits blood flow to affected areas, which is called vasospasm. pleurisy: Pleurisy (PLOOR-ih-see) is a condition in which the pleura — two large, thin layers of tissue that separate your lungs from your chest wall — becomes inflamed. Also called pleuritis, pleurisy causes sharp chest pain (pleuritic pain) that worsens during breathing.  O/E: – Antalgic gait – Shoulders: restricted abduction & internal rotation – Elbows: limited flexion to 85 – Wrists: swelling, warmth, tenderness, limited ROM – Positive Tinel’s & phalan’s – Hands: swelling, warmth, tenderness in MCPs & PIPs proximal interphalangial joint metacarpophalangeal – Knees: effusions joint metatarsophalangial joint – Feet: positive MTP squeeze – Spine: normal ROM, SIJs normal sacroiliac joint  extra reading Carpal tunnel syndrome is one of the most common hand conditions. It is caused by pressure on the median nerve in the carpal tunnel of the wrist. The carpal tunnel is a narrow passageway surrounded by bones and ligaments on the palm side of the hand. When the median nerve is compressed, symptoms can include numbness, tingling and weakness in the thumb and fingers. Investigations  Investigations Blood Tests: normal MCV and low hg levels indicate anemia caused by – Haemoglobin 10.5 chronic disease – MCV 80 (normocytic anemia) Slight elevation due to immune – Platelets 650 system response (acute phase reactant) – ESR 85. CRP 50 Erythrocyte Sedimentation Rate C-Reactive Protein – RF strongly positive Inflammatory markers Rheumatoid factor – ANA negative Anti-nuclear AB  Erosion on X-Ray Some erosion at the LVL of 2nd metacarpophalangeal joint MRI of the LVL of the joint at the LVL of the wrist shows some erosion  Rheumatoid arthritis history : morning stiffness, small joint inflammation and swelling, examination : synovitis in joints investgations : high inflammatory markers, positive rheumatoid factor not every positive rheumatoid factor test means that the pt has rheumatoid arthritis, it could be present in other diseases or even in normal population  RA Chronic progressive inflammatory autoimmune disease Incidence : Male = 0.2/1000, female = 0.4/1000 most connective tissue diseases are more in females than males Prevalence = 0.5-1% 2-3 x more common in women than in men  genetic involvement is important in pt with RA RA Siblings of RA patients have 2-4 fold increased risk compared to general population Peak age of onset = 4th -5th decade Unknown aetiology HLA–DR4 is positive in 60–70% of patients with RA & In 20–25% of general population HLA genes only account for 37% of the genetic factors involved  RA Symptoms develop gradually over weeks to months 15% present acutely over days Generally presents as a symmetrical polyarthritis of small joints Approximately 1/3 of patients have single joint disease as the initial manifestation. Leads to cartilage damage and bone erosions and subsequent joint damage  long standing arthritis gone untreated causing irreversable flexion deformity nowadays due to the aggressive nature of therapy we rarely see these types of deformities swan neck deformity hyper extension of PIP flexion in the DIP ( Distal interphalangeal joints) x-ray of pt with RA showing severe erosion in the 4th and 5th metacarpal bones MRI of feet showing erosion in the metatarsal bones Risk factors  Risk factors multifactorial 1. Environment: smoking can cause RA and even make it worse in people a) Smoking (in CCP-positive patients) with RA and can make the pt b) low level of formal education less responsive to medication c) Urban environment 2. Hormones: a) Oestrogen b) Breast-feeding after 1st pregnancy c) Pregnancy has a favourable effect on RA pt with RA who get pregnant often get better 3. Genes: a) HLA–DR4, DR1 most important b) PTPN22 c) STAT4 Autoantibodies  Autoantibodies RF & CCP have same sensitivity ~70% CCP has higher specificity than RF 95% VS 85% Most patients with anti-CCP antibodies are also RF positive, and vice versa we can have a pt that has RA and is RF negative and anti-CCP negative, and these pts are called seronegative Rheumatoid factor (RF) Disease course Extra-articular manifestations connective tissue diseases are systematic diseases that effect all parts of the body  Extra-articular manifestations Skin Ocular Haematologic Pulmonary Cardiac Neurologic Renal Treatment after using NSAIDs and/or steroids to control inflammation we use Disease Modifying Drugs (DMARDs)  Disease-modifying antirheumatic drugs (DMARDs). These agents are characterised by their capacity to reduce or reverse: – Signs and symptoms – Disability – Impairment of quality of life – Inability to work – Progression of joint damage it is important to use them early to prevent joint damage  Disease-modifying antirheumatic drugs (DMARDs). Over the last 15 years, care of patients with RA has improved considerably. New drugs with novel modes of action have led to improvements in signs and symptoms, as well as in long-term outcomes, including joint destruction and disability.  Two major classes DMARDs Synthetic chemical compounds (sDMARDs) Biological agents (bDMARDs)  sDMARDs: Conventional (csDMARDs): – methotrexate (MTX) – sulfasalazine – Leflunomide – HCQ Targeted (tsDMARD): – tofacitinib  bDMARDs 1) TNF inhibitors: 1) (adalimumab, certolizumab pegol, etanercept, golimumab and infliximab) 2) T cell costimulation inhibitor: 1) abatacept 3) anti-B cell agent: 1) rituximab 4) interleukin (IL)-6 receptor (IL-6R)-blocking monoclonal antibody: 1) tocilizumab 5) biosimilars (bs) 1) bs-infliximab  Why important for a dentist to know about RA Relevant to the dentist The TMJ is affected in one-fourth of patients, usually symmetrically, and causing no major disability. In severe destructive cases, attrition of joint cartilage and bone causes mal- alignment of the teeth with malocclusion  2nd disease under the umbrella: Sjögren syndrome Too important to know coz it mostly affect the oral cavity  Inflammatory autoimmune disease affecting primarily the exocrine glands. Lymphocytic infiltrates replace functional epithelium, leading to decreased exocrine secretions. Tears, saliva Xerostomia, xerophthalmia Mucosal dryness is manifested as xerophthalmia (keratoconjunctivitis sicca) and xerostomia There is salivary gland enlargement. bilateral parotid gland enlargement Relevant to the dentist bad oral hygiene causing wear and cavitation of the teath biopsy of the gland showing lymphocytic infiltrate  sjögren can preset as primary disease or secondary to other diseases Characteristic autoantibodies (anti-Ro/SS- A and anti-La/SS-B) these ab help in diagnosis of sjögren There is non-erosive polyarthritis & Raynaud’s phenomenon can be both primary or secondary to a disease like in sjögren The extra-glandular manifestations: – vasculitis – peripheral neuropathy – glomerulonephritis  There is increased risk for lymphoma. Associated with other autoimmune diseases such as RA, SLE, SSc & PM  Treatment no treatment for the disease itself but we can control the symptoms Stimulation of salivary flow by sugar-free flavored lozenges dry food, smoking, and drugs with anticholinergic side effects, which further decrease salivary flow, should be avoided Adequate oral hygiene after meals to prevent dental disease Pilocarpine to increase salivary secretion Artificial tears Hydroxychloroquine for joint pain no evidence to suggest it has an effect on dryness in sjögren syndrome anti malaria drug that has a suppressive effect on the immune system Systemic Lupus Erythematosus  Systemic Lupus Erythematosus Chronic inflammatory multi-system immune disease. Associated with characteristic Autoantibodies. Clinical course :-Highly variable ,Polymorphic Chronic Relapsing Exacerbations and remissions Variable outcome and prognosis. Significant constitutional symptoms Fever, Malaise, Arthralgias, Myalgias, headache, and anorexia Mild disease in the absence of organ dysfunction. Ethnicity and idiopathogenesis Peak incidence between the ages of 20 -40. Female: Male ratio 8:1. Children 3:1 Prevalence worldwide :-about 50-150 per 100,000 people Twins concordance rate :- monozygotic 25% -50%. :- Dizygotic 5%  Etiology of SL E most connective tissue diseases are multifactorial Host Factors Environmental Factors Sex female hormones Infection Epstein–Barr virus Race Ultraviolet radiation Genetic factors HLA-A1, B8, DR2 and DR3 Drugs and chemicals procainamide, hydralazine, and quinidine Immunologic abnormalities :- loss of immunologic tolerance to nuclear autoantigen, B-cell hyperactivity, and T-cell dysregulation, Geographic factors Diet CLINICAL MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS MANIFESTATION APPROXIMATE FREQUENCY (%) Cutaneous 88 Arthritis/arthralgias 76 Neuropsychiatric 66 Pleurisy/pericarditis 63 Anemia 57 Raynaud’s phenomenon 44 Vasculitis 43 Atherosclerosis 37 Nephritis 31 Thrombocytopenia 30 Sensorimotor neuropathy 28 Cardiac valvar disease 18 Pulmonary alveolar hemorrhage 12 Pancreatitis 10 Myositis 5 Myocarditis 5 Clinical features  Dermatological Acute Subacute Chronic Acute Malar Rash AKA butterfly rash non-scarring alopecia Subacute annular euptions seen of the trunk and the lower limbs Chronic hyper pigmented lesion due to discoid LE discoid chronic discoid lupus can cause scarring alopecia Musculoskeletal jaccoud arthropathy this deformity is caused by laxity of the joints the difference between this deformity and arthritis caused deformities is the lack of erosion Pulmonary obliteration of the costophrenic angle due to pleural effusion infiltration of the lung could be bleeding or fibrosis Cardiovascular pallor in the hands due to raynauds phenomenon lupus inreases the risk of coronary artery disease Renal there are 4 classes of lupus nephritis and they are differentiated histologically, so a biopsy is necessary to determine the class and the suitable treatment Neuropsychiatric  Haematologic Anemia : normocytic (chronic disease) or microcytic (iron deficiency) Thrombocytopenia Leukopenia Lymphopenia  Relevant to the dentists Consider SLE in a patient with recurrent oral ulcers must recur at least 3 times in 12 must be inside the mouth not on the months lips like HSV  Behcet’s disease – Recurrent oral ulcers observed by physician or patient recurring at least 3 times in a 12 month period , plus 2 of the following: – Recurrent genital ulcers – Eye lesions – Skin lesions – Pathergy subcutaneous needle prick to check for hypersensitivity reactions ulcers are painful in bahçet disease EYE INVOLVEMENT: uveitis: inflammation in the uveal tract  Folliculitis/Pseudofolliculitis recurrent information of the hair follicles Erythema nodosum painful red raised round lesion on the shins can resolve spontaneously or with medication like NSAIDs severe acne Any Questions?