BIRMINGHAM, ENGLAND — An examination of the systemically unwell patient should include screening for generalized vasculitis, a rare but sometimes life-threatening condition, according to a presentation at the annual meeting of the British Society of Rheumatology.
“Systemic vasculitis is a challenging diagnosis. It may require rapid action, so exclude other diagnoses as quickly as possible,” Dr. Richard A. Watts said. “It may require treatment without a complete data set,” he added. Vasculitis with renal involvement, in particular, can progress very quickly.
Assess organ involvement and classify the type of vasculitis as accurately as possible, said Dr. Watts, of the Ipswich (England) Hospital. The spectrum of vasculitis is very broad, from a minor rash with no organ involvement to a fulminating, life-threatening disease. Although vasculitis is rare, “it must be considered in severe multisystem disease.” Dr. Watts cited a 45-year-old male teacher, unwell for 3 months, who presented to the emergency department with low-grade fever, myalgia, arthralgia, and recent weight loss. Physical exam revealed he was pyrexial with a temperature of 38° C (100° F), and an inconsistent faint systolic murmur. He was diagnosed with infective endocarditis and treated with antibiotics. Echocardiography was normal, indicating no significant structural abnormality. Blood cultures also were normal. The patient continued to worsen, so his antibiotics were changed. After 1 week in the hospital, the patient developed foot drop and red eye, and rheumatology was consulted.
The patient had nasal stuffiness, epistaxis, a lower-extremity rash, and poor vision in his left eye. A diagnosis of vasculitis was considered. The rheumatologist ordered a renal biopsy and a consultation with ophthalmology and neurology. The patient had a rapid response to prednisolone and cyclophosphamide, and a renal biopsy showed focal segmental necrotizing glomerulonephritis. He tested positive for antineutrophil cytoplasmic antibody (ANCA), ANCA with a cytoplasmic staining pattern (c-ANCA), and proteinase 3. Wegener's granulomatosis was the ultimate diagnosis. “He died last year after a relapse,” Dr. Watts said.
In a second case, a 38-year-old steel erector presented reporting 4 days of epigastric pain, shortness of breath, and vomiting. He also described recent weight loss and flulike symptoms that persisted for 3 weeks, including headache, malaise, and myalgia. The patient was admitted under the care of surgeons and given medications including amoxicillin and tramadol. He had an elevated alanine aminotransferase (ALT) level of 45 U/L. Abdominal ultrasound did not yield any specific findings. On days 4 and 5, he developed a macular and erythematous rash on the left palm and right buttock, as well as diarrhea.
Rheumatology was consulted on day 6. The rheumatologist noted a pruritic rash, malaise, sore throat, and conjunctivitis. Mouth ulcers, some joint pain, and headache were other symptoms. Renal function was normal. A CT scan of the patient's abdomen revealed a thickened bowel wall indicative of vasculitis, Dr. Watts said. A renal biopsy revealed acute interstitial nephritis. He developed hemoptysis after about 1 week. Vasculitis was seen in only one artery, however. The patient continued IV methylprednisolone and was transferred for plasmapheresis. His respiratory function then deteriorated. “He had a massive pulmonary hemorrhage and died. This case illustrates the need for rapid diagnosis,” Dr. Watts said.
“The key [to such cases] is to take a good history, asking for suggestive symptoms,” Dr. Watts said. Malaise, fever, weight loss, arthralgia, and myalgia are general symptoms. Dermatologic symptoms include purpura, ulcers, and infarctions of the skin. Otolaryngology signs include epistaxis, crusting, sinusitis, and deafness. Gastrointestinal symptoms include mouth ulcers, abdominal pain, and diarrhea. Sensory and/or motor impairment can also occur, he added.
The next step is to assess the level of inflammation, Dr. Watts said. Order a complete blood count and differential white cell count. Look beyond the neutrophil count for eosinophilia or lymphopenia, he suggested. An acute phase response will feature elevated erythrocyte sedimentation rate (ESR) and C-reactive protein levels. Also order a viral screen and a cryoglobulins assay, Dr. Watts said. “We are always worried about infection” in the differential diagnosis, and urinalysis is critical, Dr. Watts said. Hematuria or proteinuria can signal renal involvement, and renal dysfunction, as noted earlier, can progress very rapidly.