The cases you never forget

There are times when a something unusual comes along. Maybe a rare disease, or an unusual presentation of a common disease. Something that you find challenging, and it doesn’t happen ever again. My first rare bird was farmer’s lung, allergic hypersensitivity pneumonitis. My patient, Sarah Elmes, 64, regularly visited her barn to rake straw, and look after the cattle. She had presented with a dry cough, fever, and malaise. Her exam was normal except for tachypnea and fine crackles in the mid-zones posteriorly—more or less where the patchy infiltrates were on her chest x-ray. Her eosinophil count was mildly elevated. Bronchoscopy was done to rule out the usual pathogens, and cancer. The lavage fluid was lymphocyte rich. Her disease was caused by inhalation of mouldy hay or grain dust containing thermophilic actinomycetes (e.g., micropolyspora faeni). She was treated with complete avoidance of farm work, and oral Prednisone. Like many patients I saw in residency, I didn’t see her beyond the acute phase. Read: What do patients need from their caregivers? Douglas MacPherson was referred to my IM clinic in rural B.C. I was the third internist to see him. He was 67, generally well, but frustrated and frightened by his “Fever of Unknown Origin.” I was flattered that colleagues in neighbouring communities had poured over his story, done a myriad of tests and scans, but disappointed that he was without a diagnosis. His history was completely bland. I went through his chart, and at the end of the day only one fact stood out: his CBC was abnormal. Hemoglobin 116, WC 11.0 with a normal differential, and platelets 98,000. Everything else—from liver profile to serum immunoglobulins and from pan-cultures to x-rays, ultrasounds and CT scans—was normal. Doug was fed up with temperatures of 101F every other day, accompanied by sweats and exhaustion, but he wasn’t prepared to give up. So, I ordered a bone marrow aspirate, because his hemogram said “that’s where the money is.” The result came back ‘non-caseating granuloma present.’ Great, I thought, this is sarcoidosis! Funny the chest x-ray is bland, but I guess this is non-pulmonary sarcoidosis. Read: Medicine and radiology: What do we need to know? I told Doug, and he was excited that he might be cured with a blast of steroids. I realised that TB should be considered, and I sent him in to an ID specialist in Vancouver. “Hold off on Prednisone until TB cultures are negative” was the disappointing response. Six weeks seemed like an eternity. I started Prednisone 50mg /day, with a slow taper, and his fever disappeared. His hemogram normalised. I chalked this up to experience. Of course, beating out two other internists to the diagnosis made me feel good too. Needless to say, Doug was a very happy man. Marla Despines taught me something too. I was working as a solo internist in a small community hospital and Marla came to the ER with a headache. She looked ill. I took a full history, examined her (including fundoscopy), and decided she should be admitted. Her cardiac and neurologic examination was fairly normal otherwise; but her blood pressure was 160/90. We’ve all seen patients with BP 160/90. Lab work, EKG and chest x-rays were normal. Our small hospital didn’t have a CT scanner, but she had no physical findings of CNS disease. I sent her out to tertiary care for a CT anyway, where a neurologist diagnosed tension headache. On her repatriation, I started her on a betablocker, which seemed the least I could do whilst the analgesics took effect. Then she had a seizure. Oh, shit. It didn’t last long, but it came completely out of left field. I sent her back to tertiary care, where the accepting ER doctor remarked “Look at those fingers, classic sausage-fingers of scleroderma. She’s had a hypertensive stroke or TIA, and then seized.” He was right. This was a new presentation of scleroderma. And I looked like an idiot, working in a small rural hospital, who had missed the obvious. Marla’s condition taught me two things. One, I can get it wrong, and two, it’s easy to make a colleague look stupid—when he’s done his best. Will I see these conditions again? No. Will I be a little smarter as a result of my experience? I hope so. As the saying goes, “Good judgment comes from experience, and a lot of that comes from bad judgment.” Some further reading suggestions: Farmer’s lung. Zergham AS (2002) NLM Management of extrapulmonary sarcoidosis: challenges and solutions. Al-Kofahi K et al: (2016) Ther Clin Risk Manag 12:1623-34 Increased risk of ischemic stroke in systemic sclerosis. Ying, D et al (2020): J Rheumatol, 47(1): 82-88

The cases you never forget

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Hector Baillie

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