Case 545 -- A 17 year-old girl with Raynaud's phenomenon

Contributed by Marian A Rollins-Raval, MD, MPH


The patient is a 17 year-old previously healthy girl who developed Raynaud's phenomenon in late 2006. In early 2007, she developed fatigue and general malaise with reportedly increased ESR and CPK. In February, she had progressing symptoms, with periods of chills followed by sweating thought to be fevers that were never measured. In early March, she had joint pain, specifically in her hands, wrists, knees and ankles, as well as myalgias in her back and lower legs. In late March, she was admitted for appendicitis and had an appendectomy at which time an abdominal CT scan noted interstitial changes at the base of her lungs. In April, she was being followed as an outpatient by Rheumatology and Pulmonary Medicine at an outside hospital. She was found to have a positive rheumatoid factor and diagnosed with rheumatoid arthritis with possible associated pulmonary fibrosis. She was started on NSAIDS. Later that month, she began to have more joint pain and weakness. In early May, she was started on prednisone which was increased to 60 mg bid when she noticed significant improvement. In mid May, she began to have shortness of breath, which progressively worsened.

In June, she complained of bubbling around her neck and was found to have a pneumothorax and progression of her lung disease (see Figures 1 and 2). She underwent a VATS procedure for a lung biopsy and a concurrent muscle biopsy. Her lung biopsy showed organizing pneumonitis, likely secondary to her underlying connective tissue disorder, but there was the possibility of an underlying or concomitant infection as well. Her muscle biopsy showed myopathy, which was more chronic than active, of an unspecified type. She was started on azathioprine.

Due to her worsening condition, she was transferred to UPMC for transplant evaluation. She had a family history significant for an aunt with polymyositis and a mother with psoriasis. Her review of systems on initial presentation to this hospital revealed a history of Raynaud's phenomenon, arthralgia/arthritis, history of proximal muscle weakness which had improved, shortness of breath and questionable fevers which had never been measured. Her physical examination showed periungual erythema, discoloration over PIPs and MCPs as well as cracking of skin over fingers and bilateral lung crackles.



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