Systemic Lupus Erythematosus (SLE) and other Vasculitides

Weird Rash/Rheum W/u

ana, c3, c4, hepatitis panel, UA, CRP, ESR

SLE

Joint pain, fever, and rash

Proteinuria, pericardititis, pleural effusions, GI, excess clotting due to lupus anticoag

Need 4 of 11 criteria:

1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6.  Serositis

   Pleuritis–convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion

   Pericarditis–documented by electrocardiogram or rub or by evidence of pericardial effusion

7. Renal disorder

   Persistent proteinuria >0.5 g/day or >3+ if quantitation not performed

   Cellular casts–may be red cell, hemoglobin, granular, tubular, or mixed

8. Neurologic disorder

   Seizures–in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance

   Psychosis in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance

9.  Hematologic disorder

   Hemolytic anemia with reticulocytosis

   Leukopenia <4000/mm3 total on two or more occasions

   Lymphopenia <1500/mm3 on two or more occasions

   Thrombocytopenia <100,000/mm3 in the absence of offending drugs

10. Immunologic disorder
11. Positive tests for antiphospholipid antibodies

    Anti-DNA: antibody to native DNA in abnormal titer

    Anti-Sm: presence of antibody to Sm nuclear antigen

    False-positive serologic test for syphilis known to be positive for at least 6 mo and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test

    Antinuclear antibody

Pt with SLE, fever and urinary retention = Myelitis, treat with high dose steroids

Wegener’s:

necrotizing granulomatous disease effecting lungs and kidney.  C-Anca

DISCUSSION Wegener’s granulomatosis (described by the German pathologist Friedrich Wegener in 1939)1 is a multisystem granulomatous conditionof unknown aetiology. It is defined by the American Collegeof Rheumatology as the presence of two of four clinopathologicalcriteria2(box).

ENT problems are found in 80% of patients and give the mainclue to the diagnosis.3 Those that may present in the emergencydepartment include stridor from subglottic stenosis, oral ulceration,sinusitis, otitis media and sudden conductive or sensorineuraldeafness.4 Necrosis of vessels in the cartilaginous septum leadsto epistaxis, septal perforation and eventually a saddle-nosedeformity.

Depending on the history, an ENT examination and chest radiograph or urine analysis should be performed. If characteristic abnormalitiesare found, the next step is either nasal or renal biopsy (the gold standard) or PR3 ANCA testing. In Wegener’s granulomatosis, autoantibodies are directed at proteinase 3, a proteinase foundin the granules of neutrophils. Meta-analysis has showen thesensitivity of PR3 ANCA for active Wegener’s is 91% andspecificity 99%.5 However, a small number of patients with vasculitidessuch as microscopic polyangiitis and Churg–Strauss syndromemay test positive for PR3 ANCA.6 In addition, a positive PR3ANCA may only be expressed late on in the disease. Hence, we suggest that in patients with active, severe disease who presentto an emergency department, autoantibody testing should be usedearly, as it is more likely to show an abnormality. Biopsy canbe performed once the patient is stable.

Fulminant disease may require dialysis, plasma exchange and, unlike other vasculitides, cyclophosphamide, making an accurate diagnosis important. Before its introduction, the median timefrom presentation to death was 5 months, typically from necrotising glomerulonephritis.7 This highlights the need to perform urineanalysis in patients with unusual ENT symptoms.

Classification criteria for Wegener’s granulomatosis (sensitivity88%, specificity 92%)

1. Development of oral ulcers, epistaxis orpurulent nasal discharge
2. Chest radiograph showing nodules,fixed infiltrates or cavities
3. Urine analysis showing microscopichaematuria or red cell casts
4. Histological examination showinggranulomatous inflammation in the wall of an artery or in the perivascular area (characteristically necrotising)

Go to source: Fulminant Wegener’s granulomatosis presenting as epistaxis — Thapar et al. 24 (3): e14 — Emergency Medicine Journal

Henoch-Schonlein Purpura:

rash, arthritis

Goodpastures:

Giant Cell Arteritis

Catastrophic Antiphospholipid Antibody Syndrome (CAPS)

Multiorgan failure in people with antiphospholipid syndrome.  Effects kidneys, CNS< Respiratory, CV.  Increased antibodies and signs of multi-organ.  Anticoagulation, steroids, plasmapheresis.

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