September 27, 2010

Interesting Case Challenge: Woman with Shortness of Breath, Hematuria and Lymphadenopathy

This is an interesting case challenge I read up on Medscape and decided to share with you guys here. It is about a young woman presenting with Shortness of Breath, Hematuria, and Lymphadenopathy. It is a really long read, but it tickled my fancy and I believe if you are patient enough to read to the end, it will also tickle your fancy. Happy reading folks!

Background

A 41-year-old black woman presents to the emergency department (ED) with a 5-day history of increasing shortness of breath along with a mild, nonproductive cough. She denies having any fever, chills, chest pain, or palpitations. No sick contacts are identified, and she denies any recent travel. On further questioning, the patient reports that she first experienced shortness of breath 1 year ago, and that it has been gradually progressive since then.

Her shortness of breath has particularly worsened over the last 5 days, to the point that it is limiting her daily activities; this has prompted her to come to the ED. She does not attribute the shortness of breath to any precipitating event. Her past medical history is significant for asthma and hypertension, as well as for several episodes of presumed bronchitis/pneumonia last year; she recalls being treated with antibiotics as an outpatient by her primary care provider. She also has a history of heavy menstrual periods, for which she has been on oral contraceptive pills (OCPs). Her family history is positive for diabetes mellitus and ischemic heart disease. Except for the OCPs, she is nonadherent to the rest of her regular medications, which include an angiotensin-converting enzyme (ACE) inhibitor, an inhaled corticosteroid, and a beta-2 agonist. She quit smoking 10 years ago, after a 20-pack-year history. She denies any alcohol or illicit drug use, and she has no known allergies (drug or otherwise).

On physical examination, she appears to be in no acute distress. She is morbidly obese, with a body mass index (BMI) of 45. Her blood pressure is 170/95 mm Hg, her heart rate is 106 bpm, her respiratory rate is 18 breaths/min, and her temperature is 98.6°F (37°C). She has an oxygen saturation of 97% while breathing room air. Pertinent findings on chest examination include fine crackles at the lung bases, with decreased vocal fremitus. Auscultation of the rest of the chest reveals no abnormalities. Her cardiovascular examination shows normal first and second heart sounds, with no jugular venous distention, murmurs, rubs, or gallops. There are, however, several enlarged, nontender cervical and axillary lymph nodes bilaterally. She has no rashes. The neurologic examination is nonfocal. Her peripheral pulses are palpable. Examination of her lower extremities elicits mild bilateral pitting pedal edema. The rest of her examination reveals no significant findings.

An electrocardiogram is performed that is remarkable for sinus tachycardia. The initial laboratory workup reveals a creatinine of 1.6 mg/dL (141.44 µmol/L), proteinuria (>300 g/L), and hematuria (50-100 red blood cells per high-power field). She is found to have anemia, with a hemoglobin and hematocrit of 8.1 g/dL (81 g/L) and 25.4% (0.254), respectively. Her mean corpuscular volume is 77 µm3 (77 fL), with iron levels of less than 10 g/dL, a total iron binding capacity of 197 μg/dL (35.26 µmol/L), and a ferritin level of 68 ng/mL (152.8 pmol/L). The D-dimer is positive at 4.73 μg/mL (4.73 mg/L), and the erythrocyte sedimentation rate (ESR) is elevated at 50 mm/hr. A chest x-ray obtained during the ED visit (see below) shows a right-sided pleural effusion and a patchy linear opacity at the base of the left lung that is consistent with atelectasis/scar tissue. Computed tomography (CT) scanning of the thorax shows bilateral small pleural effusions that are greater on the right than the left, significantly enlarged axillary and subpectoral lymph nodes bilaterally, and a small pericardial effusion. Ultrasonography of the kidneys, ureters, and bladder reveals no obstruction.

A chest x-ray showing right-sided pleural effusion and patchy linear opacity at the base of the left lung
Chest x-ray showing right-sided pleural effusion and patchy linear opacity at the base of the left lung consistent with atelectasis/scar tissue. Image credit: Medscape
Keeping the history in mind, what is the most likely diagnosis?
Hint: Asymptomatic azotemia and proteinuria are often a part of the picture in this disease.

A. COPD exacerbation
B. Pulmonary embolism
C. Systemic lupus erythematosus
D. Sarcoidosis
E. Congestive heart failure

Case challenge answer

So what did you make of this 41-Year-Old Woman With Shortness of Breath, Hematuria, and Lymphadenopathy? Any bright ideas yet? Read on...

The constellation of serositis, hypertension, diffuse lymphadenopathy, azotemia, and proteinuria in this patient was highly suspicious for a systemic autoimmune disease. Further laboratory workup revealed a positive result for antinuclear antibodies (ANA) 1:640, including those against double-stranded DNA (ds-DNA); IgG titer of 300 IU/mL, and an anti-Smith finding (156 EU/mL) which, when correlated with the clinical picture, are consistent with a diagnosis of systemic lupus erythematosus.

Other relevant findings included a decreased complement level, with C3 and C4 levels of 46.2 mg/dL (0.462 g/L) and 5.3 mg/dL (0.053 g/L), respectively; a positive Coombs test; hypoalbuminemia (3.1 g/dL [31 g/L]); and a negative lupus anticoagulant antibody finding. Biopsy of the kidney was performed, which showed diffuse proliferative lupus nephritis class IV, with moderate activity and no chronicity; moderate interstitial inflammation was also seen.

Very interesting read, would you not say? If you need to read more about systemic lupus erythematosus, please see the separate article, Systemic Lupus Erythematosus (SLE).

The patient in this case began treatment with high-dose oral corticosteroids and mycophenolate mofetil. After 1 week, her condition improved, with significantly decreased shortness of breath. She was discharged to home with renal, rheumatologic, and primary care outpatient follow-up.

Source:
The above article is reproduced from material entitled 'A 41-Year-Old Woman With Shortness of Breath, Hematuria, and Lymphadenopathy' by Medscape CME. Retrieved 27.09.2010 from here. Note: Materials may be edited for content and length.

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