Abstract
IgA vasculitis in adult HIV patients is rare and not well understood. Data on treatment of IgA vasculitis associated with HIV is sparse and limited to case reports. Highly active antiretroviral therapy (HAART) is the mainstay therapy for inducing remission. We report a case of a 41-year old HIV patient who presented with palpable purpura, melaena and oedema and was diagnosed with IgA vasculitis with severe renal (crescentic glomerulonephritis), gastrointestinal and skin involvement. He was treated with immunosuppressive drugs and HAART which led to remission of disease. This case is unusual due to the presence of crescentic glomerulopathy and the requirement of immunosuppression for remission induction.
Case report
A 41-year old male was diagnosed with HIV three years previously. He was on highly active antiretroviral therapy (tenofovir/lamivudine/dolutegravir) and had a CD4 count of 155/mm3 and an undetectable viral load. He presented with complaints of lower extremity rashes and oedema. The rash started as a non-blanching, non-pruritic palpable purpura over both lower extremities which gradually increased in size and coalesced to develop into multiple maculopapular lesions. He also developed swelling of the feet that started four weeks ago. He also complained of pain abdomen, along with the passage of black-coloured tarry stool for three days.
Physical examination revealed a normal blood pressure of 150/90 mmHg, bipedal pitting oedema and diffuse non-blanching multiple palpable purpuric rashes on the flexor and extensor surface of both upper and lower extremities, as well as the anterior aspect of the abdomen and lower back. Both ankle and elbow joints were tender without any swelling.
Laboratory investigations revealed anaemia (Hb 100 g/L, a total leukocyte count of 7.32 x 109/L (70% neutrophils, 20% lymphocytes, and 3% eosinophils) and a platelet count of 3,23 x 109/L. Serum creatinine was 44 umol/L and urea was 10.3 mmol/L. Total serum protein was 63 g/L and albumin was 30 g/L. Urine examination revealed 3 + proteinuria and 5–7 red blood cells (RBCs)/hpf). A 24h urine protein excretion was 3.9 g/day. Complement levels (C3 and C4) were within normal limits. Hepatitis B, hepatitis C, rheumatoid arthritis factor, antinuclear antibodies and antineutrophilic cytoplasmic antibodies were negative.
Light microscopy of the kidney biopsy core (Fig. 1) showed 16 glomeruli (none sclerosed), mild increase in mesangial cellularity and matrix, endocapillary hypercellularity, cellular crescents in two glomeruli and RBC cast in tubular lumina. Immunofluorescence studies showed mesangial IgA (3+) deposits. MEST-C score was M1E1S0T0C1. Skin biopsy demonstrated RBC extravasation with no significant perivascular lymphocyte infiltration suggestive of leukocytoclastic vasculitis and immunofluorescence studies reported granular staining of the vessel wall with IgA, C3 and fibrinogen.
Light microscopy of kidney biopsy. The black arrow shows mesangial hypercellularity and the blue arrow shows endocapillary hypercellularity.
Contrast-enhanced computed tomography abdomen revealed a paucity of haustration along with increased pericolic vascularity at the sigmoid colon and descending colon.
He was treated with prednisolone (1 mg/kg) and telmisartan (80 mg/day). Highly active antiretroviral therapy was continued. He reported worsening proteinuria after 1 month of treatment and therefore mycophenolate mofetil (2 g/day) was added. Complete remission was achieved after one month. Steroids were tapered to 2.5 mg/day at 12 weeks of treatment and mycophenolate mofetil tapered to 1 g/day after 12 months of treatment.
At 12 months of follow-up, he was in complete remission (S. creatinine 35.3umol/L and 24 h urine protein excretion – 160 mg/day).
Discussion
IgA vasculitis is rare in adult. The paediatric form (incidence of 22/105 person-years) is more common than the adult form (4/105 person-years). 1 The pathogenesis of Henoch-Schönlein purpura in patients with HIV remains unknown. Circulating immune complexes in blood are increased. There is polyclonal hypergammaglobulinaemia with a particular elevation of IgG and IgA. 2 3 There is a deposition of immune complexes3, composed of HIV peptides such as p24, gp41, and gp120 and antibodies (IgG or IgA) directed against these antigens, leading to the development of glomerulonephritis.
There is no clear guideline on the treatment of HIV-associated IgA vasculitis. Studies show that treatment with antiretroviral drugs helps in inducing remission. The role of steroids and immunosuppression is controversial. Despite limited evidence, treatment should include conservative medical therapy with angiotensin-converting-enzyme inhibitors or angiotensin receptor blockers. If the disease is progressive despite conservative management, immunosuppressive agents may be considered. 4
Our choice was of prednisolone and mycophenolate mofetil because of worsening proteinuria despite one month of conservative management, gastrointestinal involvement and cellular crescents on kidney biopsy.
Footnotes
Declaration of conflicting interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Ethical Approval
Ethical clearance and written informed consent for publication of their clinical details and/or clinical images were obtained from the patient/parent/guardian/relative of the patient. A copy of the consent form is available with the corresponding author for review by the Editor of this journal.
Funding
The authors received no financial support for the research, authorship, and/or publication of this article.
