Presentation of Rheumatologic Diseases and Vasculitides

Question 1

Fatigue, malar rash, inflammatory and symmetric joint pain, photosensitivity

Answer 1

SLE (may also see discoid lesions, Reynaud’s, myalgias, pericarditis/endocarditis, pleuritis, hemolytic anemia, proteinuria/GN, impaired immune response, nausea/vomiting/dyspepsia, seizures/psychosis) Treatment: NSAIDS for mild symptoms, corticosteroids for exacerbations and severe manifestations, hydroxychloroquine (constitutional/cutaneous/articular), and cyclophosphamide (glomerulonephritis)

Question 2

SLE syndrome without CNS or kidney involvement

Answer 2

Drug-induced lupusTreatment: remove offending drug (hydrazine, procainamide, isoniazid, chlorpromazine, methyldopa, quinidine)

Question 3

Raynaud’s, sclerodactyly, dysphagia, pulmonary HTN

Answer 3

Diffuse scleroderma (can have renal malignant HTN, pulmonary HTN/interstitial fibrosis, or pericardial/myocardial involvement)Treatment: symptomatic, treat complications

Question 4

Raynaud’s, sclerodactyly, dysphagia, calcium deposits, telangiectases under nails

Answer 4

CREST (cutaneous limited–no renal/pulmonary/cardiac findings)Treatment: symptomatic

Question 5

Dry eyes/mouth, arthalgias, interstitial nephritis, vasculitis, positive Schirmer test

Answer 5

Sjogren’s (Dx: Schirmer test for lacrimal gland output, salivary gland biopsy)Treatment: pilocarpine/cevimeline (increase secretions), artificial tears, NSAIDS

Question 6

Pulmonary manifestations, esophageal dysfunction, polyarthritis, sclerodactyly, cutaneous findings, myopathy, Raynaud’s

Answer 6

Mixed connective tissue disease (SLE, RA, systemic sclerosis, polymyositis)Treatment: varies depending on which disease predominates

Question 7

Fatigue and symmetrical joint pain in PIP/MCP/wrist/knees, morning stiffness which improves throughout day, subcutaneous nodules over extensor surfaces

Answer 7

RATreatment: NSAIDS/low dose corticosteroids and DMARDS (methotrexate with folate, leflunomide, or hydroxychloroquine)

Question 8

Anemia, neutropenia, splenomegaly, RA

Answer 8

Felty’s syndrome

Question 9

50 year old man with podagra and fever

Answer 9

Gouty arthritis (Dx: needle shaped negatively birefringent urate crystals in synovial fluid) Treatment: bed rest, indomethacin, colchicine, corticosteroids (oral or intra-articular injection) Prophylactic: allopurinol (xanthine oxidase inhibitor decreases uric acid synthesis), probenecid (uricosuric drugs increase renal excretion of uric acid)

Question 10

Acute onset of severe unilateral knee or wrist pain with erythema, swelling, and warmth

Answer 10

Pseudogout, also called calcium pyrophosphate deposition disease (Dx: weakly positively birefringent rod-shaped and rhomboidal crystals in synovial fluid)Treatment: treat underlying disease (hemochromatosis, hyperparathyroidism, hypothyroidism, Bartter’s syndrome) and manage symptoms with NSAIDS, colchicine, and intra-articular corticosteroid injections

Question 11

Subacute onset of weakness of neck flexors, shoulder girdle, and pelvic girdle muscles with myalgias and rash around eyes, bridge of nose, and cheeks Elevated CK, AST, ALT, and LDH

Answer 11

Dermatomyositis (can be associated with vasculitis)Types of rashes: heliotrope, Gottron’s papules (scaly lesions over knuckles), V sign (rash on face, neck, anterior chest), shawl sign (rash on shoulders, upper back, and elbows), periungual erythema with telangiectases Treatment: corticosteroids and immunosuppression (methotrexate, cyclophosphamide, chlorambucil)

Question 12

Weakness of shoulders and pelvic muscles, dysphagia, and myalgias

Answer 12

Polymyositis Treatment: corticosteroids and immunosuppression (methotrexate, cyclophosphamide, chlorambucil)

Question 13

Older man with progressive asymmetrical weakness of quadriceps, forearm flexors, tibialis anterior, and facial weakness with loss of deep tendon reflexes and dysphagia

Answer 13

Inclusion body myositis Treatment: poor response

Question 14

Weakness of shoulders and pelvic muscles, dysphagia, and myalgias

Answer 14

Polymyositis Treatment: corticosteroids and immunosuppression (methotrexate, cyclophosphamide, chlorambucil)

Question 15

Older man with progressive asymmetrical weakness of quadriceps, forearm flexors, tibialis anterior, and facial weakness with loss of deep tendon reflexes and dysphagia

Answer 15

Inclusion body myositis Treatment: poor response

Question 16

Older man with progressive asymmetrical weakness of quadriceps, forearm flexors, tibialis anterior, and facial weakness with loss of deep tendon reflexes and dysphagia

Answer 16

Inclusion body myositis Treatment: poor response

Question 17

Elderly patient with abrupt stiffness, especially in morning, in shoulder/hip after a period of inactivity. Pain with movement and muscle strength 5/5.

Answer 17

Polymyalgia rheumatica (10% develop temporal arteritis)Treatment: resolves within 1-2 years (self limited) but corticosteroids reduce inflammation until this happens

Question 18

Woman with anxiety/depression and aching pain in many joints exacerbated by weather changes and stress

Answer 18

Fibromyalgia Treatment: some effect with SSRIs and TCAs, otherwise cognitive/psych

Question 19

Man with low back pain/stiffness worse in morning, acute anterior uveitis, enthesitis

Answer 19

Ankylosing spondylitisTreatment: indomethacin, anti-TNF (etanercept, infliximab), PT

Question 20

Acute asymmetric arthritis and effusions of joints of lower extremities that progresses from one joint to another. History of recent GI infection

Answer 20

Reactive arthritis (preceded by salmonella, shigella, campylobacter, chlamydia, yersinia) Treatment: NSAIDS, if no response give sulfasalazine and immunosuppressive agent (azathioprine)

Question 21

Arthritis, uveitis, urethritis

Answer 21

Reiter’s syndromeTreatment: NSAIDS, if no response give sulfasalazine and immunosuppressive agent (azathioprine)

Question 22

Sausage digits, psoriasis, arthalgias of small joints

Answer 22

Psoriatic arthritis Treatment: NSAIDS, if no response methotrexate/leflunomide

Question 23

Severe headache with jaw claudication and visual impairment

Answer 23

Temporal arteritis (40% also have polymyalgia rheumatica)Treatment: high dose prednisone

Question 24

Constitutional symptoms, absent pulses in carotid/radial/ulnar arteries with tenderness and signs of ischemia

Answer 24

Takayasu’s arteritis (of aortic arch/branches)Treatment: steroids, anti-HTN, surgery/angioplasty

Question 25

Oral and genital ulcers, arthritis of knee/ankle, uveitis, intracranial HTN, fever

Answer 25

Behcet’s syndromeEye findings: uveitis, optic neuritis, iritis, conjunctivitisCNS findings: intracranial HTN, meningoencephalitis Treatment: steroids

Question 26

Glomerulonephritis, sinusitis, and hemoptysis

Answer 26

Wegener’s granulomatosis Treatment: cyclophosphamide and corticosteroids can induce remission but most die within 1 year of diagnosis

Question 27

Painful palpable purpura, with fever, weight loss, and fatigue after infection

Answer 27

Hypersensitivity vasculitis (reaction to penicillin, sulfa drug, infection, or other stimulus)Treatment: steroids and removal of offending agent

Question 28

Oral and genital ulcers, arthritis of knee/ankle, uveitis, intracranial HTN, fever

Answer 28

Behcet’s syndromeEye findings: uveitis, optic neuritis, iritis, conjunctivitisCNS findings: intracranial HTN, meningoencephalitis Treatment: steroids

Question 29

30 year old male smoker with leg pain on exertion, paresthesias, and ulcerations on digits

Answer 29

Buerger’s disease (thromboangiitis obliterans)Treatment: smoking cessation

Question 30

Painful palpable purpura, with fever, weight loss, and fatigue after infection

Answer 30

Hypersensitivity vasculitis (reaction to penicillin, sulfa drug, infection, or other stimulus)Treatment: steroids and removal of offending agent

Question 31

Glomerulonephritis, hemoptysis, and IgG anti-glomerular basement membrane antibody

Answer 31

Goodpasture’s syndrome