Lymphoplasmacytic sclerosing pancreatitis ("autoimmune" pancreatitis) is the best-known manifestation of an unusual corticosteroid-sensitive systemic fibrosclerotic disease that is associated with high plasma immunoglobulin G4 (IgG4) and tissue infiltration with IgG4-secreting plasma cells. Pancreatic and biliary manifestations of this condition are well-reported, but reports of other systemic involvement are few. We report here a case of initially unrecognized autoimmune pancreatitis followed 5 years later by a focal sclerosing lymphoplasmacytic tubulointerstitial nephritis and concurrent membranous nephropathy. The patient presented with hypertension, a raised serum creatinine, proteinuria, elevated serum IgG4, and eosinophilia. Immunolabeling of renal tissue showed numerous IgG4 positive plasma cells with peritubular and glomerular subepithelial IgG4 deposition. On steroid therapy serum IgG4 levels normalized, the eosinophilia resolved, and there was improvement in symptomatic wheeze, dry eyes, serum creatinine, and liver function tests. This case highlights a distinctive and potentially treatable form of interstitial nephritis manifesting from a systemic immune disorder, and provides circumstantial evidence to support the notion that dysregulated IgG4 can precipitate the development of a form of membranous nephropathy.
A 56 year old aromatherapist presented with advanced renal failure following chronic coal tar creosote vapour inhalation, and a chronic tubulo-interstitial nephritis was identified on renal biopsy. Following dialysis dependence occult inhalation continued, resulting in seizures, ataxia, cognitive impairment and marked generalised cerebral atrophy. We describe for the first time a case of creosote abuse by chronic vapour inhalation, resulting in significant morbidity. Use of the polycyclic aromatic hydrocarbon-containing wood preservative coal tar creosote is restricted by many countries due to concerns over environmental contamination and carcinogenicity. This case demonstrates additional toxicities not previously reported with coal tar creosote, and emphasizes the health risks of polycyclic aromatic hydrocarbon exposure.
Immunoglobulin G4-related disease (IgG4-RD) is a multisystem, fibroinflammatory condition unrecognised in medical science until the last decade. It is characterised by progressive scarring and dysfunction of affected organs and tissues including the pancreas, hepatobiliary tree, kidneys, salivary glands, retroperitoneum and lungs. The diagnosis is made with the presence of numerous IgG4 positive plasma cells within a histologically-distinct chronic inflammatory process; most patients also have elevated serum IgG4. Though early cases were all identified in Japan, subsequent reports clearly demonstrate that IgG4-RD exists worldwide. There are no data confirming the prevalence of IgG4-RD in the West but it is thought to be very rare. Limited awareness of the condition and its heterogeneous presentation frequently results in misdiagnosis. Prompt and correct diagnosis is critical, as a rapid reversal of even advanced disease is often seen with corticosteroid therapy. We present three cases that illustrate some of the typical features of this condition. KeywoRdS CASe 1A 67-year-old man presented with a progressive decline in kidney function; serum creatinine rose from 73 µmol to 346 µmol (n=60-120 µmol) between 2010 and 2012 ( Figure 1A). Previous medical history included recent onset type 2 diabetes mellitus (HbA1c <7% -diet controlled), kidney stones and colorectal adenocarcinoma, treated with an anterior resection complicated by pseudomembranous colitis. Physical examination demonstrated evidence of previous colorectal surgery but no other medical condition of note. The clinical features are summarised in Table 1A. A urine dipstick test showed protein++ and blood+++. The urinary albumin:creatinine ratio was 3.7 mg/mmoL (n=0-2.5). Urinary Bence Jones protein, plasma electrophoresis, serum calcium and liver function test (LFTs) results were normal. Immunological investigations are summarised in Table 1B. An abdominal ultrasound scan (USS) showed a nonobstructive left renal calculus. A biopsy of the left kidney was performed; the histological findings were consistent with IgG4-RD associated nephropathy, with coincidental clinically-insignificant mesangial immunoglobulin A (IgA) disease ( Figure 1A). 1 This diagnosis was supported by significantly elevated serum IgG4 with otherwise normal IgG4 subclasses (Table 1B). Given normal LFTs and no significant pancreatic symptoms, the diagnosis of diabetes was felt to be unrelated to IgG4-RD.Prednisolone 40 mg once daily (0.5 mg/kg/day) was commenced and the serum creatinine fell from 408 µmol to 251 µmol within four weeks ( Figure 1A). Gradual steroid reduction in 5 mg increments per fortnight began after eight weeks; dialysis was not required. CASe 2A 55-year-old man presented with malaise, dyspepsia, weight loss (7 kg), dry eyes, intermittent cramps and pruritus. Previous medical history included chronic sialadenitis of the right submandibular gland and two pulmonary emboli (the patient was on life-long warfarin). He took no other long-term medications. His blood ...
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