This is a 28-year-old female patient, with no history of previous illnesses, with a clinical picture characterised by complete infectious toxic syndrome and poly-arthralgias in major joints, of seven days' evolution, treated with oral amoxicillin without remission, associated with productive cough with muco-purulent expectoration, progressive dyspnoea on medium to light exertion, liquid stools and urinary symptoms.
Physical examination: poor general condition, B.P. 110/60 mmHg, C.F. 100 beats/minute, R.F. 24 /minute, with dry, pale skin and mucous membranes. On the skull, parietal region with areas of alopecia, centre-facial erythema of greater intensity on the cheeks, lungs with incomplete right subscapular condensation and contra-lateral substitution. Abdomen soft, painful in the right hypochondrium and flank, Blumberg positive. Genitalia with yellowish-white genital discharge. In the upper extremities, livedo reticularis was observed on the dorsum of the hands, distal cyanosis in the fingers of both hands, with a decrease in local temperature and "mouse bite" lesions in the third phalanges of the 2nd and 3rd fingers of the right hand, and in the third phalanges of the 1st, 3rd and 5th fingers of the left hand, painful on palpation, as well as subungual haemorrhages in splinters. Outlined oedema in the lower extremities, petechiae on the dorsum of both feet, confluent. Conscious, oriented, with no evidence of meningeal irritation or neurological focality.
Laboratory: haemoglobin 10.3 g/dl, haematocrit 31%, ESR 105 mm. leukocytes 10,000 mm3, segmented 8300/mm3, lymphocytes 1500/mm3, monocytes 100/mm3, platelets 200. 000 mm3, pre-prandial glycaemia 159 mg/dl, serum Na l43 mmol/l, serum K 4.2 mmol/l, serum Cl 118 mmol/l, serum urea nitrogen 46.9 mg/dl, creatinine 2.9 mg/dl, prothrombin time 12 seconds, with 100% activity. Urine examination: protein ++, blood ++, red blood cells 50 to 60, pyocytes 40 to 50, presence of haematic and granular casts.
CRP 2.6 mg/dl (< 0.8), rheumatoid factor (+) 1.3 IU/ml (less than 8), ASTO 50 (< 200), serology for hepatitis B, C, HIV negative, TSH 5.54 uU/ml (0.63-4.19), T3 0, 07 nmol/L (1.3-2.5) and T4 29 nmol/L (69-141), prolactin 36.20 ng/ml (3.6-18.9), impaired glucose tolerance curve, normal liver function tests. Blood culture, stool culture and urine culture were negative. Proteinuria in 24-hour urine: 899 mg. Complement 3, 12 mg/dl (70-170) and complement 4, 0 mg/dl (20-40). Diffuse and peripheral pattern ANA (+) 1:320 (< 1/40) and antiDNA ds (+) 64 IU/ml (< 27) core-extractable antigen profile (ENA) with doubtful JO-1 0.92 (< 0.90) Sm/RNP positive 1.2 (< 0.37). Antiphospholipid antibodies: anti-cardiolipin IgM 24 MPL/ml (< 12 ) and IgG 21 GPL/ml (< 13).
Viral serology: toxoplasma IgG ELISA positive 2.1 (> 1.1), cytomegalovirus IgG ELISA positive 4.4 (> 1.1) and IgM ELISA positive 2.8 (> 1.1).
During hospitalisation, the patient had deep vein thrombosis in the left lower extremity verified by venous Doppler ultrasound, severe pericardial effusion corroborated by chest X-ray, electrocardiogram, Doppler echocardiography and acrocyanosis in the distal phalanges of the fingers of both hands. Methylprednisolone 500 mg pulses were administered for 3 consecutive days, followed by prednisone at a dose of 1 mg/kg/day and azathioprine 100 mg/day, in addition to anticoagulation with sodium heparin and warfarin.
The evolution was unfavourable, with digital necrosis (dry gangrene), associated signs of psychosis, lupus nephropathy and cerebral ischaemia (left hemiparesis), documented with brain CT and electroencephalogram. She was treated with 3 sessions of plasmapheresis weekly for two weeks, pulses of 1 g of cyclophosphamide weekly for 3 consecutive weeks and immunoglobulin 20 g per day (400 mg/kg/day) for 7 days, pentoxifylline, flunarizine, levothyroxine, antivirals and ACE inhibitors, with a favourable evolution. She was discharged after 6 weeks of hospitalisation with neurological sequelae and complete necrosis of the 1st and 2nd distal phalanges of the aforementioned fingers on both hands, which were amputated naturally 9 months after her discharge. She is currently undergoing periodic medical check-ups and treatment with mycophenolate mofetil 1 g/day with favourable evolution.