Lupus guidelines cover nephritis, serositis, arthritis and the major neuropsychiatric syndromes. They do not cover the patient in front of you with lupus podocytopathy, catatonia or protein-losing enteropathy, and for those the literature is case reports. An international taskforce drawn from the European Reference Network on Connective Tissue and Musculoskeletal Diseases, the Systemic Lupus International Collaborating Clinics and the European Lupus Society, having previously published consensus strategies for 24 rare manifestations, has now added 22 more with 77 participants contributing.
The list is worth knowing for what it contains: diffuse pulmonary haemorrhage; bullous lupus, chilblain lupus, lupus tumidus, erythema multiforme and toxic epidermal necrolysis-like lupus among the cutaneous forms; interstitial nephritis and lupus podocytopathy among the renal; chorea, small fibre neuropathy, catatonia and intracranial hypertension among the neurological; protein-losing enteropathy, lupus hepatitis, intestinal pseudo-obstruction and peritonitis among the gastrointestinal; myositis and Jaccoud's arthropathy; and uveitis, angioedema from anti-C1 esterase inhibitor antibodies, interstitial cystitis and lupus mastitis.
The practical function of a document like this is to stop the clock. When a lupus patient develops intestinal pseudo-obstruction at two in the morning, the alternative to an agreed starting strategy is a literature search under pressure, and the treatment that gets given is whatever the on-call registrar last read about. Having a consensus position means the first move is defensible even though the evidence behind it is thin.
What it is not is evidence. These are expert-based strategies for situations where trials do not and will not exist, and the taskforce says so. Read them as a floor for decision-making, not as a standard of care to be audited against.
- Keep the list to hand for the out-of-hours presentation you have not seen before
- Note that lupus podocytopathy and interstitial nephritis are separately addressed — they are not treated as class V nephritis
- Recognise intestinal pseudo-obstruction and protein-losing enteropathy as lupus manifestations rather than incidental gastroenterology
- Consider small fibre neuropathy in the lupus patient with burning pain and normal conduction studies
- Document that a strategy is consensus-based when you use it, so later reviewers understand the evidence level
Why it matters
The manifestations nobody has trials for are the ones where practice varies most and where a first decision is made under pressure.
Don't overread it
These are expert consensus strategies for situations without trial evidence, not recommendations derived from data.
Read the rest in the app
You have read your two free briefings this month. The app carries all 27 specialties, every morning, free — and this finding is waiting in it.
Scan to keep reading on your phone. No account needed to start.
Tomorrow morning, before your first patient
One edition a day for rheumatology, written by the desk, every claim tied to its paper. Six minutes.
Get the app — free