Rheumatology Case: Spring Break Souvenir

nobody here?

the answers are: 1,2,4,6,7
Fever occasionally occurs in inflammatory arthritis, but its presence in this case would raise the suspicion of infection (gonococcus, nongonococcal bacterial septic arthritis, subacute bacterial endocarditis, or rheumatic fever).

The typical rash of reactive arthritis (Reiter’s syndrome) is keratoderma blennorrhagica. Other rashes of diagnostic significance include erythema chronicum migrans (Lyme disease); erythema marginatum (rheumatic fever); malar rash (SLE); erythema nodosum (sarcoidosis, inflammatory bowel disease),skin pustules, particularly over the palms (gonococcus); and scaly plaques over extensor surfaces and the scalp (psoriasis).

Previous diarrheal episodes may indicate inflammatory bowel disease. Patients with IBD may have intermittent or mild symptoms for many years before consulting a physician.

Difficulty getting to an early class could be the result of many things, including either psychological factors (e.g., poor quality of sleep due to depression) or physical problems (e.g., morning stiffness). If his problem is morning stiffness lasting longer than one hour, this would be very suggestive of inflammatory arthritis.

A positive family history of spinal arthritis, psoriasis, or inflammatory bowel disease would point toward the diagnosis of spondyloarthropathy.

Childhood vaccinations are not be related to the current problem.

A history of tick bite would raise suspicion for Lyme disease, but lack of recollection of tick bites is very common in patients with Lyme disease. A description of the characteristic rash is much more useful. However, this patient’s description of his rash does not fit that of Lyme disease.

He reports that he has felt feverish but has not taken his temperature. He has noticed some blisters on his toes and bottoms of his feet, which do not itch. He remembers no tick bites and has not been to the beach other than in Florida. The episode of diarrhea during spring break was distinctly unusual for him.
When asked to elaborate on the problem of getting to his chemistry class, he states that his joints and muscles feel stiff and sore all over for one to two hours after awakening and his feet are quite sore when he first gets out of bed. He reports that all the men on his father’s side of the family have back problems of some kind, but he does not know their medical diagnoses.
Physical examination reveals a well-developed, well-nourished young man with normal vital signs. Mucous membranes are abnormal, with slight erythema and exudate on bulbar and palpebral conjunctivae. There are two painless superficial erosions of his tongue and a sharply demarcated erosion of the hard palate . He has no lymphadenopathy.
Several skin abnormalities are noted, including separation of the distal portion of the nails from the nail bed on several of his fingers (onycholysis) with accumulation of subungual hyperkeratotic material. Several small, red, scaly plaques are present on the plantar surface of the toes of his left foot, along with grouped pustules and vesicles on the sole of this foot.
Lungs are clear, and his heart has a regular rate and rhythm, without murmurs or gallops. The abdomen is flat, without hepatosplenomegaly, masses, or tenderness. His external genitalia are without lesions, and there is no penile discharge. Rectal examination showed no prostate enlargement or tenderness.
His left knee is swollen and slightly warm, and he experiences discomfort as you take it through a passive arc of motion. Test for an anterior “drawer sign” is negative. He is tender around the Achilles tendon insertion into the calcaneus at both heels, but it is worse on the left, where there is also visible swelling. The third and fourth toes on his left foot are diffusely swollen and tender. The small joints of the hands do not have any swelling, tenderness, or loss of motion. Axial skeleton examination discloses normal neck motion and chest expansion, but lumbar flexion is painful. There is tenderness of the buttocks over the region of the sacroiliac joints. Schober’s test is normal.

Question # 2: Which six of the following tests would be useful in further evaluating the patient?
A. Repeat urinalysis .
B. CBC with differential .
C. Erythrocyte sedimentation rate .
D. Test of stool for occult blood .
E. HLA-B27 .
F. Urethral swab for Gram stain and culture .
G. Arthrocentesis of knee for cell count with differential, Gram stain, and crystals .

the answers are:1,2,3,4,6,7
Repeat urinalysis after treatment with antibiotics will distinguish a urinary tract infection from sterile or inflammatory urethritis, which is associated with the spondyloarthropathies.

CBC and ESR are inexpensive indicators of chronic inflammation or acute infection.

A test of stool for occult blood should be done to evaluate the possibility of inflammatory bowel disease.

A urethral swab for culture is effective for diagnosing gonococcal infection.

Arthrocentesis of a swollen joint is necessary to obtain fluid for culture and crystal examination.

HLA-B27 testing is neither specific nor sensitive for diagnosis of the spondyloarthropathies. From 60% to 90% of patients with spondyloarthropathy have the HLA-B27 antigen, indicating it is an important factor in the pathogenesis of those diseases. However, 6-12% of the normal population (depending on genetic background) also carry HLA-B27, so there is a high rate of false-positive results. In addition, testing for HLA-B27 does not help differentiate among the various types of spondyloarthropathies.

next:
Urinalysis demonstrates slightly cloudy urine with a few mucus threads; examination of sediment from a spun specimen reveals many WBCs, but no bacteria or casts. Results of the CBC show a WBC of 13,100/mm3 with normal differential, hemoglobin, hematocrit, and platelets. ESR is 30 mm/hr. Stool for occult blood is negative. Urethral swab Gram stain demonstrates polys and much amorphous pink material, but no organisms are seen and the culture is negative.

Joint fluid is cloudy, with reduced viscosity. The synovial fluid WBC is reported as 7,500/mm3 with 60% polymorphonuclear cells. No crystals are seen under the polarizing microscope, and no organisms are found on Gram stain. HLA-B27 is pending.

Question # 3: Which three additional tests would be most helpful?
A. HIV test .
B. Arthroscopy with synovial biopsy of knee .
C. Serum uric acid .
D. Test of urethral smear for Chlamydia antigen .
E. MRI of knee .
F. Radiographs of knees, feet, and sacroiliac joints .

answers are :1,4,6
Because of the patient’s urethritis and sexual history, both HIV and Chlamydia need to be excluded as potential concurrent infections. Chlamydia is a recognized “trigger” of reactive arthritis, and evidence is mounting that treatment directed at Chlamydia may modify the course of disease. However, a sterile urethritis can complicate reactive arthritis even when the inciting infection is acquired via an enteric route.

We do not necessarily expect the radiographs to detect abnormalities this early in the disease, but evidence of erosion at tendon insertions or sacroiliitis would support the diagnosis of spondyloarthropathy.

The appropriate test for suspected gout (polarized light microscopy of synovial fluid for crystals) has already been done in this patient. Furthermore, a serum uric acid level would neither confirm nor eliminate gout.

Arthroscopically directed biopsy is indicated only if an infection of synovial tissue was suspected (e.g., fungus, TB).

MRI of the knee is indicated if the history and examination suggest internal derangement or avascular necrosis of bone, which was not the case in this patient.

next:
Urethral smear for Chlamydia is negative. The radiographs show a left knee effusion, a small calcaneal spur, and an erosion at the insertion of the Achilles tendon. Although the radiograph of his sacroiliac joints was read as normal, your review of the films show suspicious widening and irregularity of the joint spaces. HIV test is negative. Serum uric acid is normal at 6.0 mg/dl

The radiologist indicates that the MRI would not be performed until you can explain how the results might alter management of the case. Arthroscopy is tentatively scheduled.

Question # 4: What do you think is the most likely diagnosis in this case?
A. Psoriatic arthritis .
B. Gonococcal arthritis .
C. Reactive arthritis (Reiter’s syndrome) .
D. Ankylosing spondylitis .
E. Arthritis associated with inflammatory bowel disease

answer is 3
This previously healthy young man developed a syndrome characterized by abnormalities in three systems: musculoskeletal (acute asymmetrical pauciarticular synovitis of lower extremity joints, along with evidence of enthesopathy in the form of insertional tendonitis of both Achilles tendons and symptoms and signs suggestive of sacroiliitis); mucocutaneous (conjunctivitis, painless ulceration of palate and tongue, onycholysis with subungual hyperkeratosis, and a psoriasiform rash involving the feet, which bears the tongue-twisting name keratoderma blennorrhagica, literally, “weeping pus from horny skin”); and urogenital (sterile urethritis characterized by dysuria and sterile pyuria). All symptoms seemed to develop about two weeks after a brief but severe bout of gastroenteritis contracted after eating improperly maintained food and probably due to Salmonella.

While each component of this patient’s illness could have alternate explanations, the concurrence of these features defines a subset of reactive arthritis or Reiter’s syndrome. Other infectious agents associated with reactive arthritis include Chlamydia, Yersinia enterocolitica, and Shigella flexneri among others. These organisms can all invade mammalian cells, and small numbers of organisms or bacterial particles have been shown to persist in synovial and other cells of patients with reactive arthritis. The diagnosis is reached by pattern recognition: there is no single finding or test that “makes” the diagnosis.

The musculoskeletal features in this case could occur in psoriatic arthritis.However, the cutaneous manifestations would be expected in more typical locations (extensor surfaces of elbows, knees, etc.), and enteric infection, urethritis, and conjunctivitis would not be present.

Gonococcal arthritis would not explain the mucocutaneous or enthesopathic features. A concurrent infection with GC should always be considered in a patient with reactive arthritis and an active sexual history, but it is excluded here by appropriate cultures.

The diagnosis of ankylosing spondylitis requires evidence of inflammatory spine disease and sacroiliitis, and it usually does not include peripheral arthritis and mucocutaneous lesions, as seen in this case. Patients with reactive arthritis and psoriatic arthritis may develop a spondylitis indistinguishable from that seen in ankylosing spondylitis, but usually it is less severe and tends to be more asymmetrical.

The arthritis associated with inflammatory bowel disease ( (also called enteropathic arthritis) may manifest as peripheral arthritis preceding the development of colitis, but the diagnosis cannot reliably be made in the absence of evidence of bowel involvement.

There is considerable overlap among the various spondyloarthropathies, and patients may develop features of one or another type during the course of their illness, but the constellation of findings in this patient most closely fits the diagnosis of reactive arthritis or Reiter’s syndrome.

Question # 5: What two treatments do you recommend as initial therapy?
A. High-dose aspirin .
B. Indomethacin .
C. Celecoxib .
D. Tetracycline .
E. Prednisone .
F. Methotrexate .
G. Sulfasalazine .
H. Physical therapy .

answers are:2,8
While high-dose aspirin may be effective, toxicity and short dosing intervals limit its use. Indomethacin is generally considered to be more effective in reactive arthritis (and other spondyloarthropathies) than other NSAIDs. The drug does, however, frequently cause GI and CNS side effects.

Celecoxib is a new COX-2 selective NSAID that offers the advantage of a lower incidence of serious gastrointestinal side effects. However, it is currently more expensive than generic indomethacin. Moreover, this patient has not had difficulty tolerating non-selective NSAIDs thus far and does not currently have significant risk factors for NSAID gastropathy.

The utility of antibiotic therapy in reactive arthritis continues to be a matter of some debate. Patients with documented Chlamydia infection should receive a course of tetracycline or doxycycline, but there is no proven efficacy of antibiotic therapy in the absence of infection.

Prednisone is less effective in reactive arthritis than in other chronic arthropathies such as RA. Nevertheless, patients who are severely disabled by constitutional and musculoskeletal features of reactive arthritis may need moderate doses of steroids to permit daily functioning if NSAIDs are not effective. Disease-modifying agents such as methotrexate and sulfasalazine are indicated only if symptoms cannot be controlled with adequate doses of NSAIDs.

Physical therapy is very important to maintain range of motion and prevent contractures. This patient should be given a structured daily home exercise program for this purpose, which should include general conditioning and spinal flexibility exercises. It is particularly important for patients with spondylitis to do spinal extension exercises to maintain an upright posture.

that's all......