Table 1.
Clinical features of NF in systemic lupus erythematosus
| n | Race/age/sex | Organ involvement | Medications | Comorbidities | Reported anaemia | Alb (gr/dl) | WBC (×103/mm3) (Lym) | Classification of NF | Isolated micro-organism | Presentation | Outcome | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | A 26F | Lung and cutaneous vasculitis, myositis, Raynaud, thrombopenia, ART, hepatitis | CS, HCQ, CYC, RTX, Doxycycline | Detrusor overactivity, AVN of elbow, digital ischaemia. Escherichia coli urinary tract infections | Yes | 1.7 | 15.4 (1.9%) | 1 | Pseudomonas | Leg pain, purpuraic patches | S | Current report |
| 2 | NA 39F | N, ART, thrombopenia | CS, HCQ | DM | No | NA | 16.7 (NA) | 1 | Aeromonas | Leg pain, blisters | S | 1 |
| 3 | NA 28F | Cerebral lupus, N, ART | CS | Mental retardation, AVN hips | No | NA | 10.5 (9.5%) | 1 | Streptococcus, Serratia marcescens, Aeromonas, Morganella | Leg pain, fever | S | 1 |
| 4 | Aboriginal 34F | N, APS | CS | Renal failure | No | 3.3 | 7.0 (4.3%) | 2 | Streptococcus | Thigh skin sore | S | 4 |
| 5 | H 48F | Photosensitivity, oral ulcers, leucopenia, pleural effusion, haemolytic anaemia. | CS, anticoagulation. | PE, APS, DM. | Yes | NA | Normal | 1 | SA | Thigh swelling, pain | S | 5 |
| 6 | A 58F | NA | CS | Hepatitis C, cirrhosis | No | NA | 21.6 (NA) | 1 | SA, Serratia | Swelling leg | D | 6 |
| 7 | A 28F | Anaemia | None | None | Yes | NA | 17 (NA) | 1 | Pseudomonas | Facial swellling, pain, fever | S | 8 |
| 8 | H 34F | ART, seizures | CS | None | No | 3.3 | 15.6 (2.6%) | 2 | Streptococcus | Leg pain, swelling | S | 9 |
| 9 | H 43F | ART, N | CS, CYC | None | No | 2.2 | 1.6 (23%) | 1 | Escherichia | Leg pain and oedema | D | 9 |
| 10 | AA 53F | ART, N | CS | Hypertension | No | 1.9 | 1.3 (23%) | 1 | Pseudomonas, Enterococcus | Thigh ecchymosis, bullous lesion | S | 9 |
| 11 | AA 12M | ART, N | CS | None | No | 2.9 | 4.3 (28%) | 1 | Streptococcus pneumoniae | Neck pain, swelling | S | 9 |
| 12 | AA 20F | ART, vasculitis, haemolytic anaemia | CS, AZA, HCQ, napro-xen | None | Yes | NA | NA (NA) | 2 | Streptococcus | NA | S | 9 |
| 13 | H 30F | Vasculitis, neph | CS, AZA, HCQ | None | No | 3.3 | NA (0.5 ×103/mm3) | NA | NA | NA | S | 9 |
| 14 | H 38M | ART, rash | CS, ubupro-fen | None | No | 2.9 | 7.6 (9.2%) | — | Negative | NA | S | 9 |
| 15 | H 57F | Neph, ART, cerebritis | CS | CAD, hypertension | No | 2.2 | 25.6 (3.9%) | 2 | Streptococcus | NA | D | 9 |
| 16 | Canadian 30F | Serositis, purupura, ART, malignant hypertensio, N | CS, CYC | None | Yes | NA | NA (NA) | 1 | Streptococcus pneumoniae | Face and neck swelling, erythema | S | 10 |
| 17 | NA 18F | NA | NA | Renal insufficiency. Respiratory tract infection | No | NA | NA (NA) | 1 | Streptococcus pneumoniae | Dyspnoea, dysphagia, neck stiffness | S | 11 |
| 18 | NA 38F | ART | CS, CYC | Transverse myelitis | No | NA | 4 (8%) | 1 | Salmonella | Fever, lumbar pain | D | 12 |
| 19 | NA 46F | Alopecia, oral ulcers, photosensitivity, ART, rash, thrombopenia | Etodolac | NA | Yes | NA | 3.7 (NA) | 1 | Streptococcus pneumoniae | Thigh swelling, tenderness, pain | D | 13 |
| 20 | A 35F | N, thrombopenia | CS | Diarrhoea | Yes | NA | 15 (3%) | 1 | Salmonella | Fever, thigh pain, swelling and haemorrhagic bleb | S | 14 |
| 21 | French 66F | Anaemia | AZA | DM | Yes | NA | 1.8 (NA) | 2 | Streptococcus | Abdominal pain, leg swelling, haemorrhagic bleb | D | 15 |
| 22 | NA 40M | N | CS | NA | No | NA | NA (NA) | 1 | Serratia | Leg cellulitis | S | 16 |
| 23 | NA 46F | Pleuritis, ART | CS | None | Yes | NA | 2.1 (20%) | 1–2 | Gram-positive cocci in chains | Thigh ecchymosis, oedema, bullae | S | 17 |
| 24 | A 21F | N | CS | Chronic renal failure | No | 2.3 | 16 (40%) | 1 | Bacteroides, Morganella | Bartholin abcess | S | 18 |
| 25 | A 31F | N, thrombopenia | CS, CYC | DM, chronic watery diarrhoea | Yes | 1.9 | 6.1 (9%) | 1 | Salmonella | Arm pain, swelling | S | 19 |
| 26 | H 23F | NA | CS | Deeply carious tooth | No | NA | 16.5 (NA) | 2 | Streptococcus, Staphylococcus | Dysphagia, dyspnoea, trismus neck pain, oedema | S | 20 |
| 27 | NA 21M | N | CS, RTX, plasma-pheresis. | TTP, mesenteric vasculitis, C. difficile-associated colitis | No | NA | NA | 1 | Acinetobacter | Flank and thigh pain | D | 21 |
| 28 | NA 36F | Raynaud, APS, heart block, aseptic meningitis | CS, HCQ | Hip AVN | No | NA | 8.2 (8.5%) | 2 | Streptococcus | Back, abdominal pain, fever | S | 22 |
| 29 | NA 14F | NA | CS, AZA | NA | No | NA | NA (NA) | 1 | Streptococcus pneumoniae | Face and neck swelling, pain | S | 23 |
| 30 | A 40F | Photosensitivity, Raynaud, oral ulcer | CS, HCQ, AZA | Systemic sclerosis, cutaneous ulcers | Yes | NA | 2.3 (NA) | 1 | Enterobacter, Enterococcus | Fever, pain on ischial áreas | S | 24 |
| 31 | AA 17F | N, ART, palatal ulcer | CYC, CS, MMF, HCQ, aspirin. | Breast abscess | Yes | 1.4 | 2.1 (12%) | 1 | Haemophilus | Arm and thigh swelling, pain | D | 25 |
| 32 | NA 58M | Non-healing digital ulcers | CS and MTX | Diverticulitis | No | NA | 33 (NA) | 1 | Non-haemolytic Streptococcus, Bacteroides | Swelling scrotum pain | S | 26 |
A, Asian; AA, African–American; APS, antiphospholipid syndrome; ART, arthritis; AVN, avascular necrosis; AZA, azathioprine; CAD, coronary artery disease; CS, corticosteroid therapy; CYC, cyclophosphamide; D, died; DM, diabetes mellitus; F, female; FG, Fournier's gangrene; H, hispanic; HCQ, hydroxychloroquine; Lym, lymphocytes; M, male; MMF, mycophenolate; MTX, methotrexate; N, nephritis; NA, not available; NF, necrotising fasciitis; PE, pulmonary embolism; RTX, rituximab; S, survived; SA, Staphylococcus aureus; TTP, thrombotic thrombocytopenic purpura; WBC, white blood cells.