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Contributor: Peter Valen, MD, FACP
Rheum2Learn cases contain links to images in the Rheumatology Image Library. Access to the Library is an ACR/ARP member benefit. See information about membership types and how to become a member.
A 50 year old female presents with a six-month history of " painful red spots " on her lower legs. They occur in crops over a period of time and resolve with residual hyperpigmentation. She has no systemic features such as fever or weight loss, and no circulatory, pulmonary, gastrointestinal, or other symptoms. Her past medical history is unremarkable and she is on no medications. There is no family history of bleeding disorders.
Physical examination is unremarkable except for palpable purpura on both lower extremities. Initial laboratory tests include a normal complete blood count, ESR and urinalysis. A biopsy of a skin lesion reveals leukocytoclastic vasculitis (LCV).
What is the differential diagnosis?
LCV resulting in palpable purpura is secondary to another underlying condition in more than 70% of cases.
What testing is indicated in this patient's work up?
The following tests can be done to look for systemic causes of LCV:
All tests were negative or normal.
This patient had LCV of unknown cause, with no evidence of systemic vasculitis, underlying connective tissue disease, or other systemic illness. Treatment in this case often consists of low dose prednisone with a gradual taper. Other medications such as colchicine, anti-histamines and dapsone may be helpful in resistant cases.
A 43 year old man presents with a 2-3 week history of fever, cough and migratory arthralgias. This was preceded by persistent nasal stuffiness and a mildly productive cough that on one occasion included bright red blood. He reports a 5-10 year history of recurrent sinus and ear infections that seem unresponsive to antibiotics and decongestants. Additionally, he reports recent malaise and fatigue, with mild dyspnea on exertion. For the past 4-5 days, he has had a painful, red left eye. He is a non-smoker, with no recent travel or exposures, and he denies cocaine or other drug use.
Physical examination reveals an ill-appearing male, with a blood pressure of 140/90 and a pulse of 102, temperature is 101 degrees F. There is no skin rash or adenopathy. He is tender over the maxillary sinuses, and the left eye is injected and slightly swollen. Chest exam reveals decreased breath sounds with rales in the upper lung fields. A chest X-ray reveals multiple, large pulmonary nodules in both upper lobes with cavitation in several areas. Laboratory studies include a mild anemia and WBC of 12,200 with a slight left shift and no significant eosinophilia. A urinalysis reveals 2+ protein and 20-30 RBCs. Serum creatinine is 1.5. Sinus films show opacification of the left maxillary sinus.
What is the likely diagnosis and what additional tests do you want to obtain to help confirm your diagnosis?
This patient presents with multi-organ system disease, with prominent findings in the upper and lower respiratory tract and kidney. Systemic vasculitis, as well as infection, malignancy, and vasculitis mimics, should be considered.
A PPD skin test was negative; sputum for AFB and fungal cultures were obtained; AFB smears and sputum cytology were negative. Serum antibody tests for ANA, RF, and HIV were negative. Anti-GBM antibodies were negative. ANCA was positive at a titer of 1:1280 with cytoplasmic staining (c-ANCA), and anti-proteinase 3 (PR3) antibodies by ELISA were also positive. Serum C3 and C4 complement levels were normal. Bronchoscopy revealed no obstructing lesions or bloody secretions. An open lung biopsy was performed, and tissue sections revealed granulomatous inflammation, necrotizing vasculitis, and multi-nucleated giant cells.
What is the most likely diagnosis in this patient?
This patient's presentation suggests a pulmonary-renal syndrome, the most common causes of which include Goodpasture's (anti-GBM disease), post-infectious, lupus and other auto-immune diseases, and ANCA-associated vasculitis. Several additional clinical features in this case suggest Granulomatosis with Polyangiitis - Wegener's (GPA) as a likely diagnosis. This condition should be suspected in patients with chronic epistaxis, septal perforation, nasal bridge collapse, chronic sinus and/or ear infections, hearing loss, hoarseness, necrotizing scleritis of the eye, proptosis, hemoptysis and glomerulonephritis. Several conditions may mimic GPA, including cocaine-induced midline destructive lesion (CIMDL), sino-nasal sarcoid, relapsing polychondritis, fungal infections (esp. mucormycosis) and NK/T cell lymphomas. Although c-ANCA and anti-PR3 positivity are highly suggestive, tissue biopsy is recommended in most cases to confirm the diagnosis, and rule out malignancy and infections.
In this case, a diagnosis of Granulomatosis with Polyangiitis-Wegener's was made, based on clinical presentation, test and biopsy results. He was treated with high-dose corticosteroids and oral cyclophosphamide, with trimethoprim-sulfa for pneumocystis prophylaxis Plasmapheresis may be indicated in certain patients with alveolar hemorrhage, rapidly progressive renal failure, or concomitant anti-GBM antibodies. Rituximab has recently been approved to treat this condition and may be an alternative to cyclophosphamide. The treatment plan was to continue cyclophosphamide and tapering doses of steroids for 3-6 months and clinical remission, then switch to methotrexate or azathioprine for maintenance therapy.
(Answer questions 1 - 5 on a piece a paper. Find Answer Key at the bottom on the page.)
The laboratory testing which would be the most appropriate in this patient's evaluation is:
The next best step in management of this patient is:
Hemoglobin 12.9 g/dL
Leukocyte count 12,400 /uL (46% neutrophils, 29% eosinophils, 16% lymphocytes, 9% monocytes)
Serum creatinine 1.0 mg/dL
Creatinine kinase normal
Urinalysis trace protein; 0-3 RBC's/hpf
Chest radiograph reveals scattered bilateral nodular infiltrates, cardiac enlargement and vascular congestion
Echocardiogram reveals global hypokinesis with ejection fraction of 4
The most likely diagnosis is:
Leukocyte count 11,500 /uL with 18% eosinophils
Erthrocyte sedimentation rate 68 mm/h
Rheumatoid factor negative
Anti-nuclear antibodies Titer of 1:80
Creatinine 1.8 mg/dL
Urinalysis trace protein, 0-3 RBCs/hpf
Anti-cardiolipin antibodies negative
Hepatitis serologies negative
Complete blood count normal
Erythrocyte sedimentation rate normal
Urine drug screen positive for cocaine
Spinal fluid analysis normal
Chest X-ray normal
Non-contrast head CT normal
Contrast-enhanced head MRI normal
Cerebral angiogram suggestive of "vasculitis", with diffuse areas of vascular ectasia and stenosis ("beading")
Repeat cerebral angiogram 3 weeks later normal
Last updated February 2015.