Rheumatology

Question 1

Glucocorticoids associated osteoporosis

Answer 1

Current guidelines recommend that all men age >50 and postmenopausal women taking >7.5 mg/day of prednisone for an anticipated course >3 months should be started on bisphosphonates as initial therapy.

Alendronate and risedronate are preferred.

Parathyroid hormone (eg, teriparatide) is a second-line agent used in patients who cannot tolerate bisphosphonates.
Bisphosphonates are also recommended for patients with a 10-year fracture risk ≥20%, regardless of the anticipated dose or duration of glucocorticoid use.

Evidence to guide management of men age <50 and premenopausal women is limited, and care should be individualized.

Question 2

Polyarteritis nodosa
Clinical presentation

Answer 2

• Systemic: Fever, weight loss
• Skin: Nodules, livedo reticularis, ulcers, purpura
• Renal: Hypertension, renal insufficiency, arterial aneurysms
• Nervous: Headache, seizures, mononeuritis multiplex
• Gastrointestinal: Mesenteric ischemia/infarction
• Musculoskeletal: Myalgias, arthritis
• Other: Myocardial ischemia, retinal ischemia, orchitis

Question 3

Polyarteritis nodosa diagnosis

Answer 3

• Negative ANCA, ANA
• Association with hepatitis B, hepatitis C
• Microaneurysms on arteriography
• Tissue biopsy confirms vasculitis (nongranulomatous)

Question 4

Polyarteritis nodosa treatment

Answer 4

• Prednisone & cyclophosphamide
• ACE inhibitors for hypertension

Question 5

Psoriasis and HIV

Answer 5

Psoriasis can be a presenting feature of HIV infection. Psoriatic arthritis is more aggressive when associated with HIV infection and can include palmoplantar involvement and nail disease. Treatment involves methotrexate, sulfasalazine, or TNF inhibitors in some cases.

Question 6

Rotator cuff impingement or tendinopathy

Answer 6

• Pain with abduction, external rotation
• Subacromial tenderness
• Normal range of motion with positive
impingement tests (eg, Neer, Hawkins)

Question 7

Rotator cuff tear

Answer 7

• Pain with abduction, external rotation
• Subacromial tenderness
• Normal range of motion with positive impingement tests (eg, Neer, Hawkins)
• Weakness with abduction & external rotation
• Age >40

Question 8

Adhesive capsulitis

Answer 8

• Decreased passive & active range of motion
• Initial painful phase followed by stiffness > pain

Question 9

Parvovirus B19

Answer 9

-Most patients are asymptomatic or have flulike symptoms
-Erythema infectiosum (fifth disease): Fever, nausea & “slapped cheek” rash (more common in children)
-Acute, symmetric arthralgia/arthritis: Hands, wrists, knees & feet (resembles RA)
-Transient pure red cell aplasia; aplastic crisis in patients with underlying hematologic disease (eg, sickle cell)

Question 10

Joint disease in hereditary hemochromatosis
Presentation

Answer 10

• Pain in the small hand joints (especially 2nd & 3rd MCP)
• Can involve wrists, hips, knees, & shoulders
• Mild nocturnal arthralgias
• Can resemble osteoarthritis or rheumatoid arthritis

Question 11

Joint disease in hereditary hemochromatosis
Radiology

Answer 11

• Narrowed joint space
• Subchondral sclerosis
• Chondrocalcinosis
• Hooklike osteophytes on the metacarpal heads

Question 12

Raynaud’s phenomenon
Investigation

Answer 12

Nailfold capillary examination can help differentiate between primary and secondary RP. It involves placing oil on the nailfold/cuticle and observing the capillaries with an ophthalmoscope.

Normal capillaries are fine loops, and abnormal ones are enlarged with disorganized architecture and microhemorrhages. Normal nailfold capillaroscopy suggests primary RP, which has a low risk for subsequent development of connective tissue disease.
Abnormal nailfold capillary structure suggests secondary RP, and the patient should be evaluated for an underlying disorder.

Question 13

Hypertrophic osteoarthropathy

Answer 13

Hypertrophic osteoarthropathy usually involves distal parts of the extremities and is characterized by abnormal proliferation of the skin and osseous tissue. It presents with clubbing of the fingers and synovitis of knees, ankles, and fingers. The most common cause is an intrathoracic neoplasm.

Question 14

Cutaneous small vessel vasculitis

Answer 14

skin-isolated leukocytoclastic vasculitis that usually occurs 7-10 days after drug exposure (eg, penicillin, cephalosporin, phenytoin, allopurinol) or infection (eg, hepatitis B or C, HIV).

-Common findings include palpable purpura and a petechial rash.
-Systemic organ involvement should prompt evaluation for an alternate diagnosis.
-Spontaneous resolution typically occurs within days or weeks after the offending drug is discontinued (or the infection is treated).

Question 15

Acute gout management

Answer 15

Acute gout is usually treated with NSAIDs as first-line therapy and colchicine as second- line therapy. Allopurinol is usually given for prophylaxis along with colchicine to patients with recurrent gout, tophaceous gout, nephrolithiasis, radiographic evidence of gouty arthritis, or urinary uric acid excretion > 1100 mg/day. Patients who develop a gouty attack while taking allopurinol should not undergo dose adjustments or drug discontinuation until 3-4 weeks after the acute inflammation subsides.

Question 16

Acute osteoporotic vertebral fracture management

Answer 16

Initial management of mild to moderate pain from osteoporotic vertebral compression fracture includes nonsteroidal anti-inflammatory drugs and acetaminophen. In addition, intranasal calcitonin is associated with improvement of acute fracture-related pain when given shortly after fracture.

Question 17

Rheumatoid arthritis poor prognosis factors

Answer 17

• Positive anti-CCP antibody with high titers
• Positive anti-CCP antibody and rheumatoid factor
• Early development of multiple-joint inflammation
• Radiographic erosions
• Severe functional limitations
• Lower socioeconomic status & less education
• Elevated ESR & CRP
• Persistent joint inflammation >12 weeks

Question 18

Colchicine toxicity in CKD

Answer 18

Chronic accumulation of colchicine, often seen in patients with chronic kidney disease, can result in significant neuromyopathy. The cytoplasmic vacuolization seen on muscle biopsy is characteristic. This condition resolves on discontinuation of colchicine.

Question 19

Parvovirus B19 infection

Answer 19

Manifestations in adults are often nonspecific, although arthralgias are typical.

Symptoms usually resolve quickly and rarely last for more than 6 weeks. Most adults in the general population have evidence of prior infection as indicated by a positive IgG titer, but IgM antibodies are more specific for acute infection.

Question 20

Morphea

Answer 20

Morphea is a category of cutaneous sclerosis that involves only the skin in the absence of other systemic manifestations of systemic sclerosis.

Question 21

Bechet syndrome treatment

Answer 21

Topical glucocorticoids are indicated for the treatment of Behçet syndrome−related oral and genital ulcers; colchicine is the drug of choice for the prevention of recurrent mucocutaneous lesions.

azathioprine, interferon-alfa, or tumor necrosis factor (TNF)-α inhibitors should be considered in recurrent and chronic arthritis

Question 22

Gout prevention

Answer 22

For most patients, including those with stage 3 chronic kidney disease, allopurinol can be titrated to a maximum of 800 mg/d in 100-mg increments.

Question 23

Drug induced ANCA vasculitis

Answer 23

Drug-induced ANCA-associated vasculitis has been reported with a number of agents, with the strongest associations found in patients using hydralazine, minocycline, and antithyroid drugs (propylthiouracil, methimazole, and carbimazole).

Of these, hydralazine is the most commonly reported agent to induce ANCA-associated vasculitis. Patients with a drug-induced ANCA-associated vasculitis usually manifest very high titers of antimyeloperoxidase antibodies, and can also display “double positive” ANCAs with concomitantly positive, low-titer antiproteinase 3 antibodies.

Question 24

Long term complications of Sjogren’s Syndrome

Answer 24

Non-Hodgkin lymphoma is a long-term (> 6 years) complication of Sjögren’s syndrome. Risk factors include anti-Ro/SSA or anti-La/SSB antibody positivity, rheumatoid factor positivity, cryoglobulinemia, hypocomplementemia, and lymphocytopenia.

The primary NHL associated with SS usually presents extranodally (commonly in the parotid gland) and is a low-grade, marginal zone B-cell lymphoma. Diagnosis is made similarly to other NHLs with excisional biopsy of an intact node. Chemotherapy is usually efficient in treating these lymphomas.

Question 25

Milwaukee Shoulder

Answer 25

Basic calcium phosphate (BCP) deposition disease of the shoulder - Milwaukee shoulder - is characterized by aggressive destruction of the glenohumeral joint and rotator cuff. It often presents with a large hemorrhagic effusion, but BCP crystals may not be visible on conventional joint fluid microscopy.

Question 27

Behcet disease management

Answer 27

Apremilast (PDE 4 inhibitor) is a reasonable alternative to colchicine as a glucocorticoid-sparing agent for recurrent oral ulcers in Behçet syndrome.

Apremilast must be started at a low dose and uptitrated over a few days to achieve a maintenance dosage of 30 mg twice daily. It is associated with adverse events, such as diarrhea, nausea, and headache. It should be used cautiously in patients with depression.

Systemic glucocorticoids in tapering doses can be used in patients who are refractory to colchicine and apremilast. However, if the patient requires continued systemic glucocorticoid therapy, azathioprine, thalidomide, or tumor necrosis factor inhibitors are most appropriate.

Question 28

Fibromyalgia treatment

Answer 28

• FDA-approved medications for fibromyalgia include pregabalin, duloxetine, and milnacipran.

Patients with fibromyalgia do not respond to anti- inflammatory drugs, including NSAIDs and glucocorticoids, and do not respond to opioids, with the exception of tramadol.

Question 29

Giant cell arteritis treatment
Glucocorticoids sparing

Answer 29

• Generally accepted indications for a glucocorticoid- sparing agent in patients with giant cell arteritis include the presence of comorbid diseases that are negatively affected by glucocorticoids, development of glucocorticoid-related adverse effects, and prolonged need for glucocorticoids.

• Tocilizumab, an interleukin-6 blocker, is an effective glucocorticoid-sparing agent in the treatment of giant cell arteritis.

Question 30

Gout prophylaxis

Answer 30

• Febuxostat is recommended for patients with indications for urate-lowering therapy who have contraindications to allopurinol therapy.

• Allopurinol and febuxostat are both strongly recommended over probenecid as urate-lowering therapies in patients with moderate-to-severe chronic kidney disease.

The ACR strongly recommends against pegloticase as a first-line urate-lowering therapy. Pegloticase is indicated in patients in whom allopurinol and febuxostat have failed or cannot be tolerated

Question 31

Refractory gout treatment

Answer 31

In patients with severe refractory gout that has not responded fully to other agents or in whom other agents are contraindicated, anakinra may be used as off-label treatment. It has a short half-life and is given by subcutaneous injection, 100 mg/d, usually for 3 to 5 days.
The dose is decreased in patients with stage 4 or 5 chronic kidney disease (CKD).

Canakinumab, a monoclonal antibody to IL-1B, which blocks binding to the IL-1 receptor, is a longer-acting IL-1 inhibitor that may be used in refractory gout flares but is more expensive than anakinra.

Question 32

Dermatomyositis glucocorticoids sparing agents

Answer 32

• In patients with dermatomyositis, initiation of methotrexate or azathioprine at diagnosis or after an early initial response to glucocorticoids is the most appropriate next step to achieve long-term disease control and to allow effective tapering of the prednisone dose.

Question 33

Abatacept and Tocilizumab adverse effects

Answer 33

Abatacept has been associated with exacerbations of COPD in clinical trials but not in real-world practice. However, it must be given cautiously in patients with COPD.

Tocilizumab increases the risk for bowel perforation in patients with a history of diverticulitis.

Question 34

Leflunomide and pregnancy

Answer 34

• Leflunomide is highly teratogenic and absolutely contraindicated during any stage of pregnancy; during the preconception phase, leflunomide should be dis- continued and cholestyramine used to remove its metabolite.

• Hydroxychloroquine can be continued throughout pregnancy.

Question 35

Chikungunya infection

Answer 35

Acute infection with chikungunya virus is heralded by the abrupt onset of severe, polyarticular arthralgia, high temperature, rash, and conjunctivitis and often with bilaterally symmetric polyarticular arthritis.

Question 36

SLE flare during pregnancy

Answer 36

• In pregnant women, differentiating a flare of lupus nephritis from preeclampsia can be difficult; a lupus flare may be identified by rising anti-double-stranded DNA antibody titers and falling complement levels.

• In a pregnant patient with a life- or organ-threatening flare of systemic lupus erythematosus, first-line treat- ment is the addition of glucocorticoid therapy