Rheumatology Flashcards

Question

How are the wrist, elbow, shoulder Lower extremities: feet, knees Cervical spine Others involved in RA

Answer 1

Three ‘A’s and two ‘6’s 1. Arthritis 2. Antibodies (anti-CCP, RF) 3. Acute phase reactants (ESR, CRP) 4. 6 weeks duation <6 weeks duration: 0 >6 weeks duration: 1 5. >6/10 points = Definite RA

Answer 2

Mnemonic: ELISA Please Give Her Methotrexate E-Etanercept L-Leflunomide I-Infliximib S-Sulfasalazine A-Anakinra P-Penicillamine G-Glucocorticoid + Gold salts H-Hydroxychloroquine M-Methotrexate

Answer 3

Most likely dx is Rheumatoid arthritis ddx  Polyarticular gouty arthritis and elbow gout tophi ± Bursitis  Nodular osteoarthritis  Psoriatic arthritis  Jaccoud’s arthritis of SLE More likely RA reasons  Presence of ulnar deviation, Z-thumb and swan neck deformities makes gout arthritis less likely  Absence of MCP and wrist involvement makes nodular osteoarthritis less like  Absence of associated skin psoriatic lesions and presence of subcutaneous nodules makes psoriatic arthritis less likely  Presence of rheumatoid nodules makes Jaccoud’s arthritis of SLE less likely

Answer 4

Knee affected most

Answer 5

Mnemonic for ankylosing spondylitis The six “A”s of Ank spond: Atlanto-axial subluxation Anterior uveitis Apical fibrosis Aortic regurgitation Amyloidosis (renal) Achilles involvement (enthesitis)

Answer 6

 Evidence of skin psoriasis * Located in scalp, behind ears, peri-umbilical, natal cleft, extensor aspects of elbows, knees and trunk  Reiter’s syndrome * Presents with urethritis and painless urethral discharge  Eye involvement * Conjunctivitis and iritis * Common to all forms of seronegative spondylitis  CVS derangement * Aortic regurgitation

Answer 7

 Drug non-compliance (most common)  Infection  Stress * Physical stress including surgery * Psychological stress  Pregnancy  Drug-induced  UV light exposure

Answer 8

Less common (3): * Mixed connective tissue disease: overlapping features of SLE, systemic sclerosis and polymyositis (one disease that is mixed). Expresses anti-U1 ribonucleic protein (Anti-U1 RNP) * Overlap syndrome: distinct entity where patient is diagnosed to have 2 well defined rheumatological syndorme most commonly SLE and RA * Undifferentiated connective tissue disease (UCTD): interim label and the actual disease should be defined when patient develop more specific clinical manifestation on follow up.

Answer 9

SLICC diagnostic criteria 4 out of 17 criteria including at least 1 clinical criterion and 1 immunological criterion (OR) Biopsy proven lupus nephritis with +ve ANA or anti dsDNA Serositis Oral ulcer Arthritis Photosensitivity Blood (all bloods are low) Renal protein loss ANA Immunologic (DS-DNA) Neurologic (psych, seizure) Malar rash Discoid rash

Answer 10

Direct antiglobulin test (Coombs test): detect antibodies on the RBC --> use of anti human IgG (AHG reagent) and anticomplement antibodies if patients RBC is coated by Ig Indications: transfusion reaction, hemolytic disease of the newborn, autoimmune hemolysis, drug induced hamolysis. Positive DAT result: suggests presence of immune mediated haemolytic process Anti-Histone (drug-induced SLE) = hi STONED on drugs

Answer 11

I - No: Normal kidney II - Man: Mesangial GN III - Faces: Focal proliferative GN IV - Diffuse: Diffuse proliferative GN V - Menstrual: Membranous GN VI - Situation: Sclerosing GN

Answer 12

In about 42% of patients with SLE-related PAH there have been detected antibodies against the endothelin receptor type A [19,31]. Those antibodies promote endothelial dysfunction and inhibit prostacyclin production, one of the main vasodilators, which result in elevated pulmonary artery pressure [19].

Answer 13

 Acute nephritic syndrome secondary to lupus nephritis

Answer 14

 Corticosteroids * At moderate to high dose with gradual tapering  Cyclophosphamide (Oral/ IV) * Combined with corticosteroids * Given for 2 – 6 months  Azathioprine/ Mycophenolate mofetil * Maintenance therapy following cyclophosphamide

Answer 15

 Hypertension  Hyperlipidemia  Complications of treatment * Leukopenia * Infection * Alopecia (Cyclophosphamide) * Amenorrhea (Cyclophosphamide)

Answer 16

 Relapse of underlying SLE  Infection due to immunocompromised * SLE itself is immunocompromising * Patient is taking corticosteroids and azathioprine  Serositis (Inflammation of serous tissue: pleura/ pericardium/ peritoneum) * Manifestation of lupus or secondary to a chest infection  Pulmonary embolism ± infarction * Can give rise to pleuritic chest pain * Complication is rare

Answer 17

3.  Delineate underlying infection (Septic workup): CXR, Blood cultures, Sputum and urine smears and culture  Delineate possible SLE relapse: ANA, anti-dsDNA, serum C3/4, Coomb’s test, Hemolysis screen  Delineate possible renal involvement during relapse: Urine microscopy, 24-hour urinary protein and creatinine estimation, Renal biopsy  Increase dosage of immunosuppressant * Increased steroid dosage * Azathioprine is substituted by a more potent agent such as cyclophosphamide, cyclosporin A or mycophenolic acid  Bone necrosis of right femoral head * Related to chronic steroidal usage * Active SLE can also cause this condition due to underlying vasculitis  NSAIDs  Steroid dosage adjustment * Dosage should be kept to minimum to prevent necrosis of the other hip  Physiotherapy  Referral to orthopedic surgeon * For consideration of surgery

Answer 18

 Systemic sclerosis (Scleroderma)

Answer 19

* Gottrons papule: erythematosus or violaceous rash. Located symmetrically on dorsal surface of MCP, PIP and DIPJ * Heliotrope eruption: erythematous to violaceous eruption on upper eyelids --> accompanied with eyelid edema * Facial erythema * Photodistributed poikoloderma: poikiloderma refers to skin that demonstrates hyperpigmentation and hypopigmentation as well as telangiectasia and epidermal atrophy. Photodistributed sites: upper back (shawl sign), neck and upper chest (V sign) * Holster sign: poikiloderma in the lateral aspects of thigh which is a photoprotected site * Periungual abnormalities: capillary nail beds may be erythematous and show vascular changes observed in systemic sclerosis and SLE. Abnormal capillary nail bed loops may be evident with alternating areas of dilatation and dropout and periungual erythema * Psoriasiform changes in scalp: scalp changes in resembling psoriasis or seborrheic dermatitis * Calcionosis cutis: deposition of calcium within the skin

Answer 20

* Glucocorticoids: imrpoves strength and preserves muscle function High dose steroid or pulse steroid as initial function. High dose steroid = PO prednisolone 1mg/kg/day (max = 80mg/day). Pulse steroid = IV methylprednisolone 1g/day for 3 days. Steroid sparing agents should be initially together with glucocorticoid treatment to reduce cumulative dose of steroid and diminish steroid induced morbidity. * Azathioprine: antimetablite (purine antagonist): inhibits adenine and guanine ribonucleatide production and hence decreases intracellular purine synthesis. Incorporates into replicating DNA as false nucleotides to stop replication. AE: myelosuppression/hepatotoxicity/pancreatitis * Methotrexate: antimetabolite --> irreversibly binds and inhibits dihydrofolate reductase (DHFR), inhibits formation of reduced folates and thymidylate synthetase resulting in reduced purine and thymidylic acid synthesis. AE: pulmonary toxicity, hepatototixicty, nephrotoxicity, myelosuppression * Hydroxychloroquine: effective in 75% of patients in controlling skin diseae but without any benefit to muscle disease. AE: GI disturbance most common (nausea/vomiting), hyperpigmentation, myopathy and neuropathy, retinopathy (bulls eye maculopathy) * IVIG: reasonable 2nd line agent for patients with refractory disease. Indicated in selected patients with severe life threatening weakness or patients with severe dyspahgia at risk for aspiration. AE: headache, thromboembolism, anaphylaxis in patients with IgA deficiency, transfusion related acute lung injury, transfusion volume overload (TACO)

Answer 21

 MOST common fatal complications * Aspiration pneumonia due to dysphagia * Interstitial lung disease (ILD) * Myocarditis o Leads to conduction abnormalities and fatal arrhythmia

Answer 22

Takayasu Arteritis - Transmural fibrous thickening of aortic branches - Japanese women <40 y/o - ‘Pulseless disease’ (subclavian artery disease = decreased or uneven blood supply to upper limbs) - Uneven BP in upper extremities + Parasthesia - Elevated ESR Samurai getting their arm cut off = transmural fibrous thickening Giant Cell (Temporal) Arteritis: - Intimal thickening & fragmentation of lamina - Increased ESR - Jaw claudication & temporal headaches - Polymyalgia Rheumatica - Visual Problems (ophthalmic artery) Give high dose corticosteroids BEFORE BIOPSY (prevent blindness) Give Corticosteroids Ammediately Gas Claudication Always

Answer 23

Polyaarteritis Nodosa - Transmural Arteritis w/ fibrinoid necrosis - Renal, coronary, mesenteric ‘String of Pearls’ appearance on Biopsy - Fibroid => Necrosis => Fibroid => Necrosis - +ve HBsAg PlaN-B: Give pearls Kawasaki Disease - Necrotising vasculitis involving coronary arteries - Can cause **M**I in children = give IV immunoglobulin + aspirin (which may cause Reye’s syndrome) - Aden**O**pathy - **O**ropharyngeal signs - **D**esquamation The cow (Kaw) that Moo’d!

Answer 24

HSP: - AKA IgA vasculitis + Haematuria + Palpable purpura + Arthralgia **+ GI pain, + IgA deposition, follow recents URI (B19 & GaS)** HSP = has stomach pain Cryoglobulinemic Vasculitis: - AKA, HepC vasculitis + Haematuria + Palpable purpura + Arthralgia **no GI pain, + HepC & Cryoglobulin deposition, treat underlying HepC** CGV = C gets Viral Churg-Strauss Syndrome - AKA Eosinophilic Granulomatosis with Polyangiitis - Necrotising, eosinophilic, granulomatous infiltration - p-ANCA - Asthma/Allergic rhinitis - Polyps - Antibodies - Peripheral neuropathy (PAPAP) Wegner Granulomatosis - AKA Granulomatosis with polyangiitis - c-ANCA (draw C which overlaps NP, lung, kidney) - We-C-Ner = nasopharynx, lungs, kidneys Microscopic polyangiitis - p-ANCA - lungs & kidneys (NO nasopharynx) - Draw P over body, P crosses lungs and kidneys)

Answer 25

Churg-Strauss Syndrome - AKA Eosinophilic Granulomatosis with Polyangiitis - Necrotising, eosinophilic, granulomatous infiltration - p-ANCA - Asthma/Allergic rhinitis - Polyps - Antibodies - Peripheral neuropathy (PAPAP) Wegner Granulomatosis - AKA Granulomatosis with polyangiitis - c-ANCA (draw C which overlaps NP, lung, kidney) - We-C-Ner = nasopharynx, lungs, kidneys Microscopic polyangiitis - p-ANCA - lungs & kidneys (NO nasopharynx) - Draw P over body, P crosses lungs and kidneys)

Answer 26

Churg-Strauss Syndrome - AKA Eosinophilic Granulomatosis with Polyangiitis - Necrotising, eosinophilic, granulomatous infiltration - p-ANCA - Asthma/Allergic rhinitis - Polyps - Antibodies - Peripheral neuropathy (PAPAP)

Answer 27

Wegner Granulomatosis - AKA Granulomatosis with polyangiitis - c-ANCA (draw C which overlaps NP, lung, kidney) - We-C-Ner = nasopharynx, lungs, kidneys

Answer 28

Microscopic polyangiitis - p-ANCA - lungs & kidneys (NO nasopharynx) - Draw P over body, P crosses lungs and kidneys)

Answer 29

 Deposition of misfolded and insoluble fibrous proteins in normal organs and tissues * Extracellular tissue deposition of fibrils composed of subunits of a variety of proteins many of which circulate as plasma constituents  Type of precursor protein, tissue distribution and the amount of amyloid deposition largely determine the clinical manifestation

Answer 30

Cryoglobulinemic Vasculitis: - AKA, HepC vasculitis + Haematuria + Palpable purpura + Arthralgia **no GI pain, + HepC & Cryoglobulin deposition, treat underlying HepC** CGV = C gets Viral

Answer 31

 Cryoglobulinemic syndrome * Form of immune-complex mediated systemic vasculitis involving small and medium- sized arteries and veins  Essential mixed cryoglobulinemia * Traditional term for idiopathic (primary) vasculitis caused by circulating CGs that contains both a polyclonal IgG and an IgM with rheumatoid factor (RF) activity which are called mixed CGs