Collapsing glomerulopathy presenting as the initial manifestation of systemic lupus erythematosus.

A 48-year-old African American female with a history of essential hypertension presented with fatigue of several weeks’ duration. Her only home medication was amlodipine. The patient denied illicit drug use. Apart from a purplish malar rash, physical examination was noncontributory. The patient did give a history of a photosensitive skin eruption on both arms in the past. Laboratory data included blood urea nitrogen 90 mg/dl, creatinine 8.6 mg/dl (baseline 0.9 mg/dl), platelet count 80 000, positive ANA with titer of 1:180, positive anti-double stranded DNA antibodies with titer of 140, C3: 80 mg/dl (normal 86–184), C4: 15 mg/dl (normal 20–59), C-reactive protein 1.7 mg/dl and urine protein excretion 8.6 g over 24 h. Systemic lupus erythematosus (SLE) was diagnosed taking into consideration the patient's thrombocytopenia, proteinuria, significantly elevated antinuclear and anti-double stranded DNA antibody titers along with the malar rash and history of photosensitive eruption (hence meeting the minimum of four American College of Rheumatology criteria). A renal biopsy (Figure 1) revealed tubuloreticular inclusions and podocyte foot process effacement. No immune deposits were seen by immunostaining. There were a total of 19 glomeruli seen, 10 of which revealed obsolescence with global sclerosis, retraction of the tuft and Bowman's space occupied by collagenous material. One glomerulus showed collapsing features including wrinkling and collapse of the basement membrane, confirming the diagnosis of collapsing glomerulopathy. The remaining eight glomeruli were normal. Tests for viral hepatitis, cytomegalovirus infection (CMV), parvovirus B19, human T-cell lymphotrophic virus infection 1 (HTLV-1) and human immunodeficiency viruses (HIV) were negative. The patient's condition improved with hemodialysis, intravenous corticosteroids and cyclophosphamide therapy and her creatinine at the time of discharge was 2.4 mg/dl.

Fig. 1

Renal biopsy revealing collapsing glomerulopathy and absence of immune deposits.

Collapsing glomerulopathy is a form of glomerular injury that is characterized by segmental or global collapse of glomerular capillaries with obliteration of capillary lumens. There is no significant deposition of immune-complexes on immunoflorescence. The presence of a minimum of one glomerulus with global and/or segmental collapse is required for the diagnosis. Collapsing glomerulopathy in the setting of SLE has been previously described in three cases, ours being the fourth report of this association [1-3]. Most cases of collapsing glomerulopathy are associated with HIV infection. Other associations include pamidronate therapy, interferon-alfa therapy, multiple myeloma, post-renal transplantation, Adult Still's disease and infection with CMV, hepatitis C, HTLV-1 and parvovirus B19. Early diagnosis with renal biopsy and appropriate therapy may be crucial as prognosis is poor with rapid progression to end-stage renal disease.

Conflict of interest statement. None declared.