Henoch-Schönlein nephritis and salmonella typhi septicaemia

T. J. Youmbissi, T. Q. Malik, S. Ajit Kumar, A. Rafi, A. I. Al Khursanny and A. Karkar

Department of Nephrology, Damman Central Hospital, Saudi Arabia

Sir,

Henoch-Schönlein purpura (HSP) has been reported in association with a heterogenous number of events and diseases. The clinical presentation and laboratory features of this disease suggest that the syndrome has an immunological basis. The onset of the disease may be triggered by a non-self substance, usually a pathogen, a drug, or a food, which leads to the cascade of immune response. The details of HSP pathophysiology is not yet fully elucidated [1]. We report here an interesting and unusual association of a full blown Henoch-Schönlein nephritis and salmonella typhii septicaemia.

Case.

A 50-year-old Filipina lady was admitted to our hospital with a 2-day history of fever-chills, nausea, vomiting, and severe abdominal pains. She had been ill for 2 weeks with intermittent fever, anorexia, general malaise, abdominal pains, a sore throat, and mild diarrhoea, bloody at times. She received amoxicillin for a few days and her condition seemed to have improved. One week prior to her admission, she noticed a painful purple rash on her feet and, progressively, this rash extended to her buttocks and the fever recurred. There were no joint pains and she had no significant past medical history.

Examination upon admission showed temperature: 38.6°C; pulse: 72 b.p.m.; blood pressure: 170/100 mmHg, a purpuric rash over the lower limbs, the buttocks and sparsely over the extensor surfaces of the upper limbs. There was some abdominal tenderness. The rest of the physical examination was normal. The patient was admitted with a provisional diagnosis of Henoch-Schönlein purpura with sepsis.

Initial laboratory investigations showed (normal values in brackets) WBC: 11 000 (3.5–10 000/µl); Hb: 14.1 (12–16 g/dl); Platelets: 337 000 (150–400 000/µl); IgG: 600 (50–1400 mg/l); IgA: 350 (100–400 mg/l); IgM: 150 (50–200 mg/l); C3: 1.6 (0.77–1.87 mg/l); C4: 0.42 (0.12–0.45 g/l); ESR: 75 (6–12 mm); CRP: 2.6 mg/dl(negative); ANA, pANCA, cANCA, autoantibodies, rheumatoid factors, cryoglobulins, all negative. HCV, HIV antibodies and HBsAg all also negative. Serum creatinine: 1.2 (0.5–1.45 mg/dl); BUN: 32 (15–49 mg/dl). Twenty four hour urine excretion of albumin: 9.8 g.

A skin biopsy showed an inflammatory infiltrate (mostly neutrophils) surrounding the dermoid vessels, with extravasation of red blood cells. On immunofluorescence (IF) there were prominent deposits of IgA and C3. The patient became clinically nephrotic with a 24-h urine albumin excretion exceeding 18 g and a serum albumin of 2.2 g/dl. She also had macroscopic haematuria and her fever persisted. A renal biopsy showed a marked mesangial hypercellularity and increased mesangial matrix. IF revealed lumpy mesangial deposits of IgA in all glomeruli. Staining with IgG and IgM were minimal and there were coarse granular deposits of C3. Urine, blood and stool cultures yielded Salmonella typhi. The patient quickly deteriorated and went into non-oliguric acute renal failure (serum creatinine: 7.6 mg/dl; BUN: 320 mg/dl; serum K: 6.4 mEq/l). She also developed a deep vein thrombosis in the left femoral vein, but this was managed only with elastic stockings to avoid the risk of fatal haemorrhage. She was treated with pulse steroids for 3 days, was given i.v. ceftriaxone and antihypertensives. Her treatment was continued with oral steroids and she did not require dialysis. She recovered fully within 6 weeks and 2 months after discharge, her renal function was normal and her urine albumin-free.

Comment.

This patient displays all the classical features of HSP [1]. Her age, gender, and ethnic origin are also significant as the disease is rare in adults, and less common in women and in tropical countries [1,2]. HSP is believed to be an immune complex mediated disease and many exogenous antigens have been regularly implicated in its precipitation. Among these, infections figure prominently. In a recent review [1], a wide range of micro-organisms have been linked to the onset of the disease in a temporal association. These included viruses, mycoplasma, mycobacteria, Gram-positive and negative bacteria, parasites, and even vaccinations. Among Gram-negative bacilli, non-typhii salmonella have been reported earlier in two cases from Italy, a 19-year-old soldier [3] and in a paediatric case [4]. Both reporting groups hypothesized that the HSP–salmonella association could have been due to an overall anomalous regulation of the lymphoid system of the mucosa, possibly modulated by a genetic predisposition.

The case presented here is to our knowledge one of the first to describe an association between HSP and typhoid fever (Salmonella typhi). The clinical features of typhoid fever do overlap with those of HSP. Typhoid fever incubation period varies from 1 week to 2 months, and the overall syndrome shown by our patient may have actually translated the common expression of the two conditions. Typhoid fever should be added to the list of infections that may trigger HSP.

References

  1. Rai A, Nast C, Adler S. Henoch-Schönlein purpura nephritis. J Am Soc Nephrol1999; 10: 2637–2644[Free Full Text]
  2. Tay CH, da Costa JL. Henoch-Schönlein purpura in adult Singaporeans. A 10 year study of 107 patients. Ann Acad Med Singapore1972; 5: 11–20
  3. Fiocchi O, Stabellini G, Squerzanti R, et al. Henoch-Schönlein purpura after Salmonella hirschfeldii infection. Nephron1990; 55: 316–320[ISI][Medline]
  4. Zucchini A, Manfredi R. Schönlein-Henoch syndrome and Salmonella infection: a new association? Minerva Pediatr1992; 44: 559–563[Medline]




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